Bradyarrhythmias and Conduction Disorders: Clinical Guidelines

Applies toadults2025 editionI44, I44.0, I44.1, I44.2…

Clinical Guidelines of the Russian Ministry of Health, adults. 2025 edition, ICD-10 codes: I44, I44.0, I44.1, I44.2…. The page features a shortened version by WhiteCoat Pro and the full official text.

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Brief for Practice

WhiteCoat Pro editorial summary of the guideline text, 2025 edition. Each item links to the original section — verify against it before prescribing.

Bradyarrhythmias are disorders of impulse formation and conduction resulting in a slowed heart rate; the main clinical variants are sick sinus syndrome, atrioventricular, and intraventricular blocks. The recommendation focuses on confirming the link between symptoms and rhythm disturbances, searching for reversible causes, emergency care, and choosing temporary or permanent cardiac pacing.

Key points

Diagnostics

  1. Complaints and medical history are clarified; special attention is paid to syncopal and presyncopal states. → in text
  2. A physical examination is performed, including pulse assessment and heart auscultation, searching for reversible causes, including medication-induced ones. In iatrogenic bradycardia, it is determined whether the continuation of the causative therapy is mandatory (LoE A, GoE 3). → in text
  3. A 12-lead ECG is recorded to assess rhythm, rate, atrioventricular and intraventricular conduction, as well as signs of structural and systemic heart diseases (LoE A, GoE 3). → in text
  4. For intermittent symptoms, prolonged monitoring is performed, selecting the method based on symptom frequency: for daily episodes — 24-hour recording, for weekly episodes — recording up to 7 days, for monthly episodes — 14–30 days, for episodes less than once a month — an implantable loop recorder. → in text
  5. Initial evaluation includes complete blood count, basic metabolic panel, TSH, sodium, and potassium; there is no specific laboratory test for bradyarrhythmias (LoE C, GoE 4). → in text
  6. In left bundle branch block, second-degree Mobitz II atrioventricular block, high-grade atrioventricular block, or third-degree atrioventricular block, transthoracic echocardiography is performed when structural heart disease is suspected (LoE B, GoE 3). → in text
  7. To assess chronotropic function, exercise electrocardiography is performed, particularly in patients with sick sinus syndrome. → in text
  8. In unexplained syncopal states after ruling out cardiac pathology, a passive prolonged head-up tilt test is performed. → in text
  9. In bradycardia or syncopal states of unclear origin with no cardiac causes identified, carotid sinus massage is performed to diagnose carotid sinus syndrome. → in text
  10. If initial non-invasive methods are uninformative, an intracardiac electrophysiological study is used to diagnose and determine the mechanism of bradycardia. → in text

Treatment

When to refer and how to monitor

Frequently asked questions

When is a permanent pacemaker needed in sick sinus syndrome?

In symptomatic bradycardia, symptomatic tachycardia-bradycardia syndrome, or symptomatic chronotropic incompetence after ruling out reversible causes. If bradycardia is asymptomatic or caused by a correctable factor, pacing is not recommended. → in text

Is a pacemaker needed in complete or high-grade AV block without symptoms?

Yes, if acquired second-degree Mobitz II AV block, high-grade AV block, or third-degree AV block has no reversible or physiological cause: pacing is performed regardless of symptoms. → in text

How to choose the duration of ECG monitoring?

The method is chosen based on the frequency of symptoms: 24 hours for daily episodes, up to 7 days for weekly ones, and 14–30 days for monthly ones. If symptoms occur less than once a month and external monitoring is uninformative, an implantable loop recorder is used. → in text

What to do in case of nocturnal bradycardia?

In asymptomatic patients, nocturnal bradyarrhythmias are often physiological and typically do not require intervention. Patients with documented obstructive sleep apnea are treated for apnea, and those being considered for permanent pacing undergo polysomnography. → in text

What is the role of atropine in acute bradycardia in SSS?

In symptomatic or hemodynamically unstable SSS, atropine temporarily increases the sinus rhythm rate; doses of approximately 0.5–2 mg are indicated in the text. → in text

What is the role of atropine in acute bradycardia in AVB?

In second- or third-degree AVB, atropine can improve conduction and heart rate, especially when the block is at the level of the atrioventricular node; temporary pacing is considered if pharmacotherapy is ineffective. → in text

Status
Applied · approved by the Scientific and Practical Council of the Russian Ministry of Health
Database ID
160, edition 2 (ID 160_2)
Publication date
June 18, 2025
Age category
adults
ICD-10 codes
I44I44.0I44.1I44.2I44.3I44.4I44.5I44.6I44.7I45.0I45.1I45.2I45.3I45.4I45.5I45.8I45.9I46I46.0I46.1I46.9I49.5
Specialties
CardiologyCardiovascular Surgery
Developers
Association of Cardiovascular Surgeons of Russia
All-Russian Public Organization «Russian Society of Cardiology»
All-Russian Scientific Society of Specialists in Clinical Electrophysiology, Arrhythmology and Pacing
Official text
Russian Ministry of Health Guidelines — 160_2

Abbreviated Clinical Guidelines: Bradyarrhythmias and Conduction Disorders

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Beginning of abbreviated version — open

1.1 Definition of the disease or condition

Bradyarrhythmias (BA) — a group of cardiac rhythm and conduction disorders.

  • Characteristics:
  • Delayed generation of electrical impulses.
  • Regular or irregular slowed ventricular rhythm associated with conduction block.
  • Include two major symptom complexes:
  • Sick sinus syndrome (SSS).
  • Atrioventricular blocks (AVB) of various levels and intraventricular blocks (IVB).

Key definitions:

  • Sick sinus syndrome (SSS) — a condition in which the atrial contraction rate does not meet the physiological needs of the body.
  • Sick sinus syndrome (SSS) — a symptom complex related to SSS, characterized by the presence of clinical symptoms of bradycardia.
  • Represents a combination of clinical and electrophysiological signs.
  • Reflects structural damage to the sinuatrial node (SAN).
  • Manifests as the node's inability to normally perform the function of a pacemaker and ensure regular impulse conduction to the atria.
  • Atrioventricular block (AVB) — partial or complete interruption of impulse conduction from the atria to the ventricles.
  • Pacemaker (artificial pacemaker) — an artificial pacemaker; a medical device for pacing and correcting the rate and sequence of heart chamber contractions.

1.2 Etiology and Pathogenesis

Causes of bradyarrhythmias (BA) are divided into:

  • Congenital:
  • As a rule, diagnosed and treated in childhood.
  • Based on impaired embryonic development of the cardiac conduction system (CCS).
  • Acquired.

Classification of causes of bradyarrhythmias (BA)

1. Reversible BA:

  • Drug effects.
  • Neurocardiac reflex factors.
  • Post-cardiac surgery states.

2. Irreversible BA:

  • Caused by organic structural changes in: the sinuatrial node (SAN), atrioventricular junction (AVJ), and cardiac conduction system (CCS).
  • Etiology: infectious, inflammatory, degenerative, or ischemic processes.

Sick sinus syndrome (SSS)

  • Major internal cause: replacement of SAN tissue with fibrous and/or adipose tissue.
  • Spread of the process: degeneration usually involves the perinodal zone, atrial myocardium, and atrioventricular node (AVN).

Progressive conduction disorders (AVB)

The main cause of slowly progressive block is degenerative-sclerotic processes:

  • Lenègre's disease: degenerative-sclerotic changes in the intraventricular conduction system.
  • Lev's disease: fibrosis and calcification of conduction structures originating from the connective tissue skeleton of the heart.
  • Characteristics: regarded as a congenital primary electrical disease whose appearance is not associated with other heart pathology.

Secondary causes of CCS damage

  • Valvular heart disease (aortic and mitral): fibrosis and calcification of the valve rings spread to the CCS, causing conduction disorders.
  • IHD: CCS damage due to myocardial infarction or chronic ischemia.
  • Contributing factors to degeneration:
  • Aging factor.
  • Arteriolosclerosis.
  • Concomitant arterial hypertension.

Congenital blocks (AVB)

4 variants of CCS pathology are distinguished (morphologically — fibrous and fatty replacement of specialized tissue, possibly with calcifications):

  • Lack of connection between the atrial myocardium and the AV junction.
  • Interruption of the connection between the AV junction and the bundle of His (at the level of the penetrating part of the bundle).
  • Interruption of integrity at the level of branching of the bundle of His branches.
  • Abnormal formation of the bundle of His with its interruption.
The official full text of the guideline below remains open and free. Only the condensed study version as a whole is paid: section-by-section explanations, self-assessment tests, and integration with platform materials.

List of Abbreviations

AAT – antiarrhythmic therapy

БА – bradyarrhythmia

LBBB – left bundle branch block

BBB – bundle branch block

RBBB – right bundle branch block

SNRT – sinus node recovery time

ВПС – congenital heart disease

Temporary cardiac pacing – temporary transcutaneous cardiac pacing or temporary external cardiac pacing

ГКМП – hypertrophic cardiomyopathy

SND – sinus node dysfunction

ДН – remote heart rate monitoring

ЕОК – European Society of Cardiology

CHD – coronary heart disease

ИКМ – implantable cardiac monitors

ИМ – myocardial infarction

КС – carotid sinus

КТ/МСКТ – cardiac computed tomography with intravenous contrast and ECG synchronization

LV – left ventricle

IVS – interventricular septum

MRI – Magnetic resonance imaging

OMT – optimal medical therapy

RV – right ventricle

AVB – atrioventricular block

AV junction – atrioventricular junction

AV node – atrioventricular node

CHS – cardiac conduction system

EST – exercise stress test using an ergometer

PM – permanent pacemaker

PET – myocardial positron emission tomography

RCS – Russian Society of Cardiology

RF – Russian Federation

HF – heart failure

HFrEF – heart failure with reduced EF

HFpEF – heart failure with preserved LV EF

HFmrEF – heart failure with mildly reduced EF (40–49%)

SAB – sinoatrial block

HBP – His bundle pacing

SAN – sinoatrial node

CRT – Cardiac resynchronization therapy (implantation of a three-chamber pacemaker), implantation of a three-chamber (biventricular) pacemaker via endovascular approach / implantation of a three-chamber (biventricular) pacemaker via open approach

SSS – sick sinus syndrome

TAVI – Transcatheter aortic valve implantation

TV – tricuspid valve

Lead extraction – endovascular removal of endocardial leads or open surgical removal of endocardial leads

LVEF – left ventricular ejection fraction

AF – atrial fibrillation

VHR – ventricular heart rate

HR – heart rate

ECG – electrocardiography, recording of an electrocardiogram

PM – pacemaker

EP study – electrophysiological study

EchoCG – echocardiography

EL – endocardial leads

** – the drug is included in the list of vital and essential drugs

*** – medical device implanted when providing medical care under the state guarantee program for free medical care to citizens

# – medicinal product for medical use used inconsistently with the indications for use, contraindications, routes of administration, and doses contained in the instructions for use of the medicinal product

Terms and Definitions

Evidence-based medicine – the conscientious, explicit, and judicious use of current best evidence (clinical research results) in making decisions about the care and treatment of individual patients [1, 2].

Disease – a disorder of bodily function, working capacity, and the ability to adapt to changing environmental conditions (both external and internal) arising from the impact of pathogenic factors, accompanied by altered protective-compensatory and protective-adaptive reactions and mechanisms of the body [3].

Instrumental diagnostics – diagnostics utilizing various devices, apparatuses, and instruments to examine a patient.

Outcome – any potential result arising from exposure to a causative factor, preventive or therapeutic intervention, or any established changes in health status occurring as a consequence of an intervention [4].

Conflict of interest – a situation in which a medical or pharmaceutical worker, in the exercise of their professional duties, has a personal interest—whether acting personally or through a company representative—in obtaining material gain or another advantage that influences or may influence the proper performance of their professional duties due to a contradiction between the personal interest of the medical or pharmaceutical worker and the interests of the patient [3].

Clinical trial / Clinical study – any investigation conducted in human subjects to discover or verify the clinical and/or pharmacological effects of investigational products, and/or to identify any adverse reactions to investigational products, and/or to study their absorption, distribution, metabolism, and excretion with the object of ascertaining their safety and/or efficacy. The terms "clinical trial" and "clinical study" are synonymous [5].

Laboratory diagnostics – a set of methods aimed at analyzing test material using various specialized equipment.

Medicinal products – pharmacological agents in the form of dosage forms used for the prevention, diagnosis, and treatment of diseases, rehabilitation, and for the preservation, prevention, or termination of pregnancy [6].

Medical intervention – any type of medical examination and/or medical procedure, as well as artificial termination of pregnancy, performed by a medical worker or other employee authorized to engage in medical activities in relation to a patient, affecting the physical or mental state of a person and having a preventive, diagnostic, therapeutic, rehabilitative, or research focus [3].

Medical worker – an individual who has a medical or other education, works in a medical organization, and whose labor (job) duties include the implementation of medical activities, or an individual who is an individual entrepreneur directly engaged in medical activities [3].

Patient – an individual to whom medical care is provided or who applies for medical care regardless of the presence of a disease and their condition [3].

Working group on the development/updating of clinical guidelines – a team of specialists working together and in a coordinated manner to develop/update clinical guidelines and bearing joint responsibility for the results of this work.

Syndrome – a stable set of multiple symptoms with a unified pathogenesis [7].

Thesis-recommendation – a statement reflecting the procedure and correctness of performing a particular medical intervention with proven efficacy and safety.

Level of evidence (LoE) – the degree of confidence that the estimated effect of a medical intervention is true [8].

Grade of recommendation (GoR) – the degree of confidence in the reliability of the intervention's effect and that following the recommendations will bring more benefit than harm in a specific situation [8].

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Bradyarrhythmias (BA) – a group of heart rhythm and conduction disorders characterized by slowed electrical impulse generation, regular, irregular, or slowed ventricular rhythm associated with impaired impulse conduction [9].

BA include two major symptom complexes: sinus node dysfunction (SND) and atrioventricular blocks (AVB) of various levels, as well as intraventricular blocks (IVB). Sick sinus syndrome (SSS) is a symptom complex related to SND and characterized by clinical symptoms of bradycardia.

Sinus node dysfunction (SND) – a condition where the atrial contraction rate does not meet physiological needs.

Sick sinus syndrome (SSS) – a combination of clinical and electrophysiological signs reflecting structural damage to the sinoatrial node (SAN), its inability to normally function as the cardiac pacemaker and ensure regular conduction of automatic impulses to the atria.

Atrioventricular block (AVB) – partial or complete interruption of impulse conduction from the atria to the ventricles.

Pacemaker (PM***) – an artificial cardiac pacemaker, a medical device designed to stimulate and correct the rate and sequence of heart chamber contractions.

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

Causes of BA can be congenital or acquired. Congenital BA are usually diagnosed and treated in childhood. They are based on embryonic developmental disorders of the cardiac conduction system (CHS). The causes of acquired BA can be divided into reversible, which may be due to the influence of drugs or neurocardiac reflex factors, as well as reversible BA after cardiac surgery. Irreversible causes may be due to infectious, inflammatory, degenerative, or ischemic changes in the sinoatrial node (SAN), atrioventricular junction (AVJ), and CHS.

A major intrinsic cause of SND is the replacement of SAN tissue with fibrous and/or adipose tissue, with the degenerative process typically extending to the perinodal zone, atrial myocardium, and atrioventricular node (AVN).

The primary cause of slowly progressive AVB is degenerative-sclerotic changes in the intraventricular conduction system (Lenègre's disease) or fibrosis and calcification of conduction structures originating from the cardiac connective tissue skeleton (Lev's disease). This is a congenital primary electrical heart disease whose appearance is not associated with other cardiac pathology.

In aortic and mitral valve disease, fibrosis and calcification of the valve annuli can extend to the conduction system and thus cause conduction disorders.

In coronary artery disease, damage to the conduction system occurs both as a result of myocardial infarction and under the influence of chronic myocardial ischemia.

The degenerative process is promoted by aging andarteriolosclerosis associated with arterial hypertension.

In congenital atrioventricular block, there are 4 variants of conduction system pathology: absence of atrial myocardial connection with the atrioventricular junction, interruption of the connection between the atrioventricular junction and the bundle of His at the level of the penetrating part of the bundle, interruption of the integrity of the conduction system at the level of bundle branch branching, and abnormal formation with interruption of the bundle of His. The interruptions represent fibrous and fatty replacement, possibly with calcifications, of the specialized tissue of the conduction system.

Normal function of the sinus node is carried out through spontaneous depolarization of its pacemaker N-cells (automaticity function) and conduction of the resulting impulses by transitional T-cells to the atrial myocardium via the sinuatrial zone (sinuatrial conduction). The autonomic nervous system modulates sinus node function, such that parasympathetic influences (acetylcholine) decrease it, while sympathetic influences (norepinephrine) increase it. Impairments of any of these components lead to sick sinus syndrome. An important role in the manifestations of sick sinus syndrome is played by the phenomenon of overdrive suppression—the suppression of pacemaker automaticity by more frequent external pacing. This mechanism determines the activity of the sinus node and the behavior of subsidiary pacemakers at the moment of interruption of atrial tachycardias in tachycardia-bradycardia syndrome. Impulse conduction through the conduction system is also significantly influenced by the autonomic nervous system: the parasympathetic system depresses conduction through the atrioventricular junction without affecting intra-atrial and intraventricular conduction, whereas the sympathetic system improves conduction through the atrioventricular junction and, by shortening the refractory period, improves conduction through the His-Purkinje system. When the conduction system is damaged, parasympathetic influences manifest in a more pronounced form, and sympathetic influences prove unable to improve conduction. In addition, an important role is played by the impulse rate itself: the throughput capacity of the atrioventricular junction decreases when it is damaged. When the intraventricular conduction system is involved in the pathological process, minimal changes in impulse frequency are capable of leading to conduction block: with critical slowing of the rhythm due to spontaneous diastolic depolarization in Purkinje fibers and with critical acceleration of the rhythm due to elongation of their refractory period.

Main causes of atrioventricular block:

Intrinsic causes

Degenerative:

age-related idiopathic degenerative fibrosis —

Lev-Lenègre disease (progressive conduction system disease)

Coronary artery disease:

chronic myocardial ischemia, myocardial infarction

atherosclerotic, thrombotic, or other lesions of the sinus node artery

Infiltrative processes:

amyloidosis, sarcoidosis, hemochromatosis, Hodgkin's lymphoma and other lymphomas, multiple myeloma, consequences of radiation therapy

Infectious diseases:

diphtheria, Chagas disease, Lyme disease, toxoplasmosis, syphilis

Collagenoses:

rheumatic fever, systemic lupus erythematosus, scleroderma, rheumatoid arthritis

Neuromuscular diseases:

myotonic dystrophy, Kearns-Sayre syndrome, Erb's myopathy, and peroneal muscular atrophy

Inflammatory diseases:

myocarditis, pericarditis

Surgical trauma:

correction of congenital heart defects,

aortic and mitral valve replacement, complication of radiofrequency catheter ablation of supraventricular tachycardias, AV junction ablation

Extrinsic causes

Drugs:

beta-blockers, calcium channel blockers (C08 Calcium channel blockers), cardiac glycosides, class I and III antiarrhythmic drugs (C01B Antiarrhythmics, class I and III), etc.

Neurocardiac reflex influences:

carotid sinus hypersensitivity, vasovagal syncope, reflex reactions to coughing, vomiting, urination, defecation

Electrolyte disturbances:

hypokalemia, hyperkalemia.

Endocrine disorders:

hypothyroidism, rarely hyperthyroidism

Hypothermia,

increased intracranial pressure,

hypoxia:

sleep apnea syndrome

1.3 Epidemiology of the Disease or Condition

The prevalence of sick sinus syndrome cannot be estimated adequately due to the impossibility of accounting for asymptomatic cases and the difficulty of diagnosing pathological bradycardia in population studies.

The detection rate of sick sinus syndrome increases with age, but in the group over 50 years old it is only 5/3000 (0.17%). Sick sinus syndrome accounts for about half of all pacemaker*** implantations, but the number of implantations does not adequately estimate the frequency of symptomatic cases of sick sinus syndrome.

In healthy adolescents, transient first-degree atrioventricular block occurs in 12% of cases, in young adults – in 4-6%. A permanent form of first-degree atrioventricular block in adults older than 20 years occurs in no more than 1%, after 50 years it increases to 5% or more, and in individuals older than 65 years it can reach 30%. The incidence of acquired, advanced second-degree atrioventricular block and complete atrioventricular block is estimated at 200 cases per million per year, and congenital complete block at 1/20,000 newborns.

Atrioventricular block associated with surgery is one of the frequent complications of the operation (up to 3%) [10].

Currently, over 85% of patients with congenital heart defects survive to adulthood. Most commonly, atrioventricular conduction disturbances occur (up to 65%), less commonly sick sinus syndrome (up to 29%).

1.4 Features of Coding the Disease or Condition

I44 – Atrioventricular and left bundle-branch block

I44.0 – First-degree atrioventricular block

I44.1 – Second-degree atrioventricular block

I44.2 – Atrioventricular block, complete

I44.3 – Other and unspecified atrioventricular block

I44.4 – Left anterior fascicular block

I44.5 – Left posterior fascicular block

I44.6 – Other and unspecified bundle-branch block

I44.7 – Left bundle-branch block, unspecified

I45.0 – Right bundle-branch block

I45.1 – Other and unspecified right bundle-branch block

I45.2 – Bifascicular block

I45.3 – Trifascicular block

I45.4 – Unspecified intraventricular conduction block

I45.5 – Other specified heart block

I45.8 – Other specified conduction disorders

I45.9 – Unspecified conduction disorder

I46 – Cardiac arrest

I46.0 – Cardiac arrest with successful resuscitation

I46.1 – Sudden cardiac death, so described

I46.9 – Unspecified cardiac arrest

I49.5 – Sick sinus syndrome

1.5 Classification of the disease or condition

Sinus node dysfunction (SND)

SND encompasses a spectrum of arrhythmias:

- sinus bradycardia,

- sinus node arrest,

- sinoatrial block (SAB), which is classified into:

- first-degree SAB (prolongation of SA conduction time),

- second-degree SAB type I – progressive increase in SA conduction time followed by impulse blockage in the SA zone,

- second-degree SAB type II – intermittent blockage of impulses in the SA zone without prior increase in SA conduction time,

- advanced second-degree SAB – blockage of every second or multiple consecutive sinus impulses,

- third-degree SAB – complete block of SA conduction with absence of atrial activations from the sinus node,

- tachy-bradycardia syndrome – alternation of paroxysms of supraventricular tachyarrhythmia (atrial fibrillation (AF) and atrial flutter (AFL)) with episodes of sinus or escape rhythm with a low ventricular rate and/or ventricular asystole,

- chronotropic incompetence.

Atrioventricular blocks

  1. Based on etiology, atrioventricular blocks (AVBs) are divided into acquired and congenital.

  2. Based on the severity of conduction disturbances, they are distinguished as:

    • First-degree AVB – slowing of impulse conduction from the atria to the ventricles with conduction of every impulse.

    • Second-degree AVB – intermittent interruptions in the conduction of atrial impulses to the ventricles.

      It has three variants:

      - Mobitz type I – impulse blockage preceded by progressive slowing of conduction from the atria to the ventricles (Wenckebach periodicity),

      - Mobitz type II – impulse blockage without prior prolongation of atrioventricular conduction time,

      - Advanced second-degree AVB – blockage of every second or multiple consecutive atrial impulses.

    • Third-degree AVB – complete block of atrial impulse conduction to the ventricles with the development of complete atrioventricular dissociation.

  3. Based on the localization of conduction disturbances within the AV conduction system, they are distinguished as:

    - block at the atrial level (intraatrial),

    - at the level of the atrioventricular node (AVN),

    - below the AVN:

    - at the level of the bundle of His – intra-Hisian,

    - at the level of the bundle branch branching – infra-Hisian.

  4. Isolated blocks of the bundle of His branches are designated as fascicular blocks:

    - right bundle branch block (RBBB),

    - left anterior fascicular block (LAFB),

    - left posterior fascicular block (LPFB),

    - combinations of two of the specified blocks are designated as bifascicular blocks: RBBB combined with LAFB, RBBB combined with LPFB, and left bundle branch block (LBBB),

    - trifascicular block refers to alternating intraventricular block (true trifascicular block), when two variants of bifascicular blocks alternate (e.g., RBBB and LBBB), or a combination of a bifascicular block with first- or second-degree AVB.

  5. Based on the clinical course, AVBs are divided into transient (intermittent) and permanent (persistent).

1.6 Clinical presentation of the disease or condition

The clinical manifestations of bradyarrhythmias are diverse and often nonspecific. The course may be asymptomatic. The clinical presentation of bradyarrhythmias includes two main groups of symptoms: cerebral and cardiac. Permanent and long-standing bradyarrhythmias may manifest as fatigue, increased tiredness, sluggishness, apathy, cognitive decline. The most prominent consequences are due to cerebral hypoperfusion: acute disorders may present with sudden dizziness, confusion, and in more severe cases – presyncopal and syncopal states up to the full clinical picture of Stokes-Adams attacks.

Bradyarrhythmias can also lead to the aggravation of angina pectoris, arterial hypertension, and chronic heart failure. A decrease in exercise tolerance with typical manifestations such as rapid fatigue and dyspnea is frequently observed [11,12].

2. Diagnostics, medical indications and contraindications for diagnostic methods

Diagnostic criteria

The diagnosis of bradyarrhythmias and conduction disorders is established in accordance with the classifications presented in section 1.5, based on documented confirmation by any instrumental research method: electrocardiography and/or prolonged ECG monitoring combined with clinical symptoms.

Diagnosis of bradyarrhythmias includes the following stages:

  • inquiry regarding complaints and taking the medical history;

  • performing an ECG and/or prolonged ECG monitoring;

  • physical examination;

  • other additional diagnostic methods – at the second stage of evaluation (as indicated);

  • exclusion of reversible causes if necessary;

It is necessary to classify the anatomical levels of conduction disturbances causing bradyarrhythmias or conduction disorders: sinus node, AV node, and bundle branch conduction disorders.

Principles of diagnosis formulation.

It is necessary to indicate the level of conduction disturbance: sinus node, AV node, or bundle of His branches.

For sinus node disorders: indicate the primary condition – SND, the type of dysfunction: sinus bradycardia, sinus node arrest, SAB with indication of degree, tachy-bradycardia syndrome, or chronotropic incompetence.

Examples of diagnosis formulation:

  • SND. Sinus bradycardia.

  • SND. Second-degree SAB type II.

  • SND. Tachy-bradycardia syndrome.

For AV nodal disorders – if known, the etiological factor of the AVB and the degree of block are indicated.

2.1 Complaints and Anamnesis

Clinical manifestations and complaints depend on whether bradycardia is permanent or paroxysmal. Moreover, even with marked bradycardia, clinical manifestations may be absent. Bradyarrhythmias that occur during sleep are typically asymptomatic. Patients with syncopal and presyncopal conditions deserve special attention.

Symptomatic bradycardia can be defined as documented bradycardia that directly correlates with clinical manifestations of syncope or presyncope, transient dizziness or weakness, symptoms of heart failure, or confusion.

2.2 Physical Examination

With long-standing bradycardia, skin pallor may be observed.

Bradycardia can be detected by palpation of the pulse in peripheral arteries. However, cardiac auscultation is more informative, revealing slow heart sounds.

It is recommended to perform a physical examination of the patient to diagnose the underlying disease that caused the bradyarrhythmia, with special attention to identifying transient, reversible causes of bradyarrhythmias, including those caused by ongoing treatment [15–19].

ESC I B (LoE A, GoR 3)

It is recommended that if an iatrogenic, drug-related nature of bradyarrhythmia is identified, it should be determined whether the use of the causative drugs is mandatory in this patient [13–19].

ESC I B (LoE A, GoR 3)

It is recommended to identify and diagnose concomitant cardiac rhythm disorders and arrhythmogenic electrocardiographic syndromes during the examination process [13–19].

ESC I B (LoE A, GoR 3)

Comments: When taking the medical history, it is necessary to clarify the frequency, timing, duration, time of onset, factors provoking and alleviating symptoms, on the basis of which bradycardia or conduction disorders can be suspected. It is also important to note the relationship of symptoms with medications, food, medical intervention, emotional distress, physical exertion, changes in body position, or other factors (e.g., urination, defecation, cough, prolonged standing, shaving, tight clothing, and head turning), which can help perform differential diagnosis.

Bradycardia and rhythm disorders can be the first manifestation of systemic diseases or heart diseases; therefore, a complete medical history must include a comprehensive assessment of cardiovascular disease risk, and a family history of diseases of other organs and systems.

Physician's action algorithm – Appendix B, Table 1.

2.3 Laboratory Diagnostics

There is no specific laboratory diagnostics for bradyarrhythmias. Changes in laboratory parameters are non-specific and are manifestations of other comorbid conditions.

In individual cases, certain conditions may be associated with bradycardia, and laboratory tests aimed at diagnosing a specific disease may be useful (e.g., blood thyroid-stimulating hormone (TSH) level testing in patients with bradycardia when hypothyroidism is suspected). Nevertheless, there have been no studies systematically examining the incremental value of laboratory tests in bradyarrhythmias.

It is recommended that patients with bradycardia undergo a complete blood count (CBC) and general biochemical blood test as part of the initial examination and during follow-up, as well as a test for thyroid-stimulating hormone (TSH) in dried blood spots, blood sodium level testing, and blood potassium level testing to confirm the primary diagnosis [20–24,721].

ESC I C (LoE C, GoR 4)

2.4 Instrumental Diagnostics

2.4.1. Electrocardiography

In most cases, non-invasive investigation methods provide sufficient information. In the permanent form of bradyarrhythmia, standard electrocardiography is quite informative. In the intermittent course of bradyarrhythmia, long-term electrocardiogram (ECG) monitoring methods are required for detection and clinical-electrocardiographic correlation. In cases where transient bradyarrhythmias are suspected but not documented, the use of provocative tests is required.

  • For patients with suspected bradycardia or conduction disorder, a 12-lead electrocardiography is recommended to document rhythm characteristics, heart rate, conduction, as well as to verify structural and systemic heart diseases [15–18, 715].

ESC I C (LoE A, GoR 3)

Comments: Resting electrocardiographic examination using 12 standard leads is indicated for all patients with existing or suspected bradyarrhythmias. Even if it does not solve the tasks of detecting and diagnosing arrhythmias due to their absence at the time of ECG recording, it provides important information regarding heart rate, the state of atrioventricular and intraventricular conduction, and ventricular myocardial repolarization processes. It allows the detection of abnormalities and signs that strongly indicate the risk of developing certain types of arrhythmias with similar bradyarrhythmia symptoms (syncope). These include manifestations of abnormal atrioventricular conduction (short PR interval, premature ventricular excitation), electrocardiographic manifestations of Brugada syndrome, QT interval prolongation or shortening, and epsilon waves in arrhythmogenic right ventricular cardiomyopathy. Resting ECG recording is mandatory before performing tests such as ambulatory ECG monitoring, exercise electrocardiography, and cardiac electrophysiological study (EPS).

2.4.2. Electrocardiographic data monitoring

There are several types of ambulatory ECG monitoring. The first of these involves the classic form of 24-hour (or longer) Holter ECG monitoring, which allows monitoring of the heart rhythm for 24–48 hours, although recording devices with a recording duration of up to 7 days are available.

The second variety is intermittent ECG recording using portable recorders, grouped under the general name "ambulatory event monitors".

"Continuous loop recorders" are most common for the diagnosis of bradyarrhythmias. They allow recording of symptomatic events for periods ranging from 30 days to 3 years.

  • To detect bradycardia and conduction disorders, and for clinical-electrocardiographic correlation with symptoms, long-term cardiac rhythm monitoring is recommended [25–28, 715].

ESC I C (LoE C, GoR 4)

It is recommended to perform long-term cardiac rhythm monitoring in patients with documented or suspected bradycardia or conduction disorder to establish a correlation between heart rate or conduction disorders and symptoms, using a cardiac monitor selected based on the frequency, nature of symptoms, and patient preference [25–28].

ESC not specified (LoE C, GoR 4)

It is recommended to use various types of long-term ECG monitoring depending on the frequency of clinical symptoms associated with bradycardias or conduction disorders [29–34, 715].

ESC not specified (LoE B, GoR 2)

It is recommended in chronic bradyarrhythmia without hemodynamic instability and in intermittent, rarely occurring bradyarrhythmias, to examine the patient on an outpatient basis or under conditions of hospital remote monitoring of ECG data [29–38, 715].

ESC not specified (LoE B, GoR 2)

Comments: Continuous heart rhythm monitoring may require differentiation between normal phenomena and pathological conditions. Physiological sinus bradycardia can be observed during the daytime at rest and at night as the predominant heart rhythm. The normal lower limit of resting heart rate during the day is determined to be 40 bpm, at night – 35 bpm, and does not depend on sex and age. The development of sinus pauses not exceeding 2 seconds in duration is also acceptable. Often, elite athletes, persons engaged in heavy physical labor, and young men exhibit bradycardia with a rate below the indicated figures, possibly combined with other manifestations of sinus node dysfunction. These conditions can be considered normal only when they are asymptomatic and there is an adequate increase in sinus rhythm rate in response to physical exertion. In healthy individuals, the transient development of first-degree atrioventricular block is not uncommon. When it is permanently recorded, narrow QRS complexes and the disappearance of the block during physical exertion or during the Atropine test** indicate the functional nature of the disorder. In a transient form at night during sleep, second-degree type I atrioventricular block can be recorded in young healthy individuals, especially well-trained athletes. The prognosis in this case is absolutely favorable.

Long-term ECG monitoring methods depending on the frequency of bradyarrhythmia episodes [13, 715]:

Frequency of episodes

Method and duration of monitoring

Daily

24-hour Holter monitoring, hospital monitoring, or telemetric ECG recording

Weekly and more frequently

24-hour Holter monitoring, inpatient monitoring, or telemetric ECG recording for up to 7 days, or ambulatory electrocardiographic monitoring

Monthly

Electrocardiographic monitoring for up to 14–30 days

Less than 1 time per month

Implantation of an insertable loop recorder for long-term recording of the electrical activity of the cardiac conduction system

2.4.3. Imaging modalities

Since bradyarrhythmias or conduction disorders can occur in a wide variety of cardiovascular and systemic diseases, and the prognosis of documented bradyarrhythmias in these cases depends on the underlying heart disease, assessment of the structural and functional characteristics of the heart is necessary. Performing an echocardiographic examination in symptomatic patients makes it possible to diagnose structural heart diseases (e.g., aortic stenosis, hypertrophic cardiomyopathy, or heart failure). Other imaging modalities may also be used to diagnose other heart diseases associated with bradycardia or conduction disorders that are not detected by echocardiography [39].

  • In patients with bradycardia or conduction disorders, left bundle branch block (LBBB) or second-degree atrioventricular block (AVB) Mobitz type II, high-degree AVB, or third-degree AVB, transthoracic echocardiography is recommended if structural heart disease is suspected [40–54, 720].

ESC IB (CGR B, LOR 3)

  • When evaluating patients with asymptomatic sinus bradycardia or first-degree AVB and no clinical signs of structural heart disease, routine cardiac computed tomography is not recommended [55–60].

ESC IIIC (CGR C, LOR 5)

  • To rule out structural heart diseases in the diagnosis of specific conditions associated with conduction disorders requiring pacemaker implantation, additional cardiac imaging methods are recommended [11, 461-465].

ESC IIa C (CGR C, LOR 5)

2.4.4. Diagnosis of bradyarrhythmias and nocturnal apnea

Nocturnal bradyarrhythmias are common both in healthy individuals and in various diseases. Sinus bradycardia is the most frequent bradyarrhythmia during sleep. Nevertheless, sinus arrest, sinoatrial blocks, all degrees of AVB, junctional rhythm, and periods of asystole also occur. This is particularly common in young and well-trained athletes. In most cases, these are physiological, mediated, asymptomatic events that do not require intervention. The frequency of nocturnal bradyarrhythmias appears to decrease in middle-aged and older adults. In such individuals, arrhythmias are rare during wakefulness, and these nocturnal arrhythmias are usually asymptomatic.

Treatment of sleep apnea not only reduces the frequency and severity of apnea/hypopnea episodes, but also eliminates the need for pacemaker implantation in the majority of patients.

  • In patients with nocturnal bradycardia or conduction disorders and documented obstructive sleep apnea, treatment for sleep apnea (e.g., by maintaining continuous positive airway pressure and weight loss) is recommended [61–78].

ESC not stated (CGR B, LOR 3)

  • In patients being considered for permanent cardiac pacing due to bradycardia or conduction disorders, polysomnography is recommended [77–78, 466 - 468].

ESC I C (LoE C, GoR 4)

2.5 Other Procedures

2.5.1. Exercise electrocardiography

To diagnose chronotropic incompetence, the so-called chronotropic index is determined, which is calculated based on the results of exercise electrocardiography using a symptom-limited maximum workload protocol. It represents the ratio of the difference between peak heart rate at maximum workload and resting heart rate (chronotropic response) to the difference between the age-predicted maximum heart rate, calculated using the formula 220 – age bpm, and resting heart rate (chronotropic reserve) [5]. It is believed that a normal chronotropic index is ≥ 80%. Assessing chronotropic function in patients with sick sinus syndrome is extremely valuable in terms of selecting the rate-responsive function of the planned pacemaker*** to be implanted.

Exercise electrocardiography is recommended to assess chronotropic function in patients with sick sinus syndrome (SSS) [86, 272–274, 469, 715].

ESC IIa B (CGR C, LOR 4)

Exercise electrocardiography is recommended to assess chronotropic function in patients with congenital third-degree AVB when deciding on participation in physical education and sports [272–274, 715].

No ESC guideline (LoE C, GoR 5)

It is recommended to consider exercise electrocardiography to detect SSS or AVB in patients with clinical symptoms of bradyarrhythmias provoked by physical activity [76, 75, 86, 405–408, 470-472, 715].

ESC IIb C (CGR C, LOR 4)

Comments: By increasing the impulse firing rate of the sinus node or by inducing myocardial ischemia, exercise testing can reveal SSS (rate-dependent sinoatrial block, marked bradycardia, or sinus arrest during exercise) and atrioventricular conduction disorders (second- and third-degree AVB during exercise). This may be an important explanation for syncope occurring during physical activity.

2.5.2. Orthostatic test

Passive head-up tilt table testing is recommended for patients with syncope of unclear origin after excluding cardiac pathology (ECG, echocardiography, ECG monitoring, intracardiac electrophysiological study) to identify vasovagal causes [410-423, 470, 715, 720].

ESC IIa B (CGR A, LOR 2)

Comments: The "passive" nature of the test indicates, on the one hand, the absence of active leg muscle movements during transition to and maintenance of the orthostatic position, as the patient rests their feet against a special platform. On the other hand, "passivity" means the absence of pharmacological provocations (administration of isoproterenol or nitroglycerin).

The duration of the orthostatic position in various protocols ranges from 10 minutes to 1 hour. The test continues until the onset of a syncopal episode (a result significant for diagnosis) or up to 45 minutes in case of a negative result. At the time of syncope, ECG, blood pressure, electroencephalography, and other parameters are recorded. Upon completion of the test, a conclusion is made indicating the absence or presence of loss of consciousness, the time of onset, and the type of induced episode.

2.5.3. Carotid sinus massage

For differential diagnosis with conditions associated with parasympathetic effects on the heart, tests involving mechanical stimulation of the vagus nerve branches are used.

  • In patients with bradycardia or syncope of unclear origin in the absence of cardiac causes, carotid sinus massage is recommended to diagnose carotid sinus syndrome [424-426, 473].

ESC I B (CGR B, LOR 3)

Comments: The test is based on the reflex enhancement of parasympathetic effects on the heart during mechanical stimulation of the carotid sinus region. Manifestations include a decrease in sinus rhythm rate and slowing of AV conduction. A sinus pause lasting more than 3 seconds is of diagnostic significance (cardioinhibitory response variant).

Before performing the massage, it is necessary to ensure the absence of a bruit over the carotid arteries. Duplex scanning of brachiocephalic arteries with color Doppler flow mapping is recommended. The test is performed with the patient in the supine position. ECG and blood pressure are recorded. Massage of the carotid sinus regions is performed alternately on the right and left sides, usually for no more than 10 seconds.

If a patient with suspected carotid sinus syndrome has a negative result in the supine position, the test is repeated in the standing position. A drop in systolic blood pressure of more than 50 mmHg in the presence of symptoms (dizziness, syncope) is considered a significant result (vasodepressor response variant). Mixed response variants also occur.

2.5.4. Pharmacological tests

A provocative graded pharmacological test with Atropine* is recommended to identify functional sinus bradycardia and first-degree AVB in asymptomatic patients [137, 430–432, 710-713].

ESC not specified (LoE C, GoR 4)

Comments: Intravenous administration of Atropine** at a dose of 0.02–0.04 mg/kg (maximum up to 2 mg) is indicated to detect functional sinus bradycardia and first-degree AVB in asymptomatic patients [710-713]. An increase in sinus rhythm rate by more than 25% from baseline or above 90 bpm indicates the vagal nature of sick sinus syndrome. The disappearance of AV conduction disorders indicates their vagal nature and localization at the AV node level.

2.5.5. Genetic testing

Most cardiac conduction system disorders are associated with age-related or structural anomalies of the cardiac conduction system. At the same time, genes responsible for hereditary heart diseases associated with conduction disorders have been identified [474-476].

Genetic mutations are associated with a range of anomalies that may manifest in isolation as conduction disorders or in combination with cardiomyopathies, congenital heart disease, or extracardiac diseases. Most genetically mediated conduction disorders have an autosomal dominant inheritance pattern [474,475].

It is recommended to determine gene variants leading to monogenic diseases in the blood using high-throughput whole-exome sequencing in patients with early manifestation (< 50 years) of bradyarrhythmias and conduction disorders [474,477].

ESC IIa C (CGR C LOR 5)

It is recommended to consider scheduling a genetic counseling session (examination, consultation) for family members after identifying a pathological genetic variant that explains the clinical phenotype of the cardiac conduction system disorder in the index patient [474].

ESC IIa C (CGR C LOR 5)

2.5.6. Implantable cardiac monitors***

Patients may experience infrequent episodes of bradycardia that cannot be detected by standard external ECG monitoring methods. If the diagnosed events occur very rarely, as well as in cases of syncope of undetermined cause, implantable loop recorders (ILRs) ***—which are a type of loop recorder—can be used. They are capable of long-term monitoring (up to 3 years) and do not depend on the patient's active participation. ILRs*** increase the duration of ambulatory monitoring and make it possible to obtain the necessary correlation between bradycardia, symptoms, and syncope.

ILRs*** are designed to automatically detect rhythm disturbances and record episodes of arrhythmia in the patient. In doing so, the device can recognize both bradyarrhythmias (asystole for more than 3–4.5 seconds, sinus bradycardia) and tachyarrhythmias (ventricular tachycardia, atrial tachycardias, and atrial fibrillation). The recognition algorithm is mainly based on measuring the cycles

of RR intervals of the ECG recorded by the device. When an RR interval exceeds a specified time, the devices fix this ECG segment in memory. When AF occurs, the devices determine irregular, chaotic RR intervals and classify them as AF, after which the ECG prior to the onset of detection is recorded and subsequently saved in memory.

A number of devices can also be activated by the patient to record the heart rhythm during a symptomatic attack. ILRs*** make it possible to establish the relationship between symptoms and heart rhythm in patients with rare arrhythmia attacks, record rhythm disturbances over a long period, and detect asymptomatic, clinically significant rhythm disturbances. The main advantage of the method is the possibility of visualization and analysis of the ECG recording retrospectively after the occurrence of an event.

Therefore, currently, implantable devices are used both to detect bradyarrhythmias and for the diagnosis and management of patients with tachyarrhythmias and atrial fibrillation.

  • Patients with rare symptoms (> 30 days between symptoms) presumably caused by bradycardia are recommended to undergo implantation of an implantable loop recorder*** for long-term recording of the electrical activity of the cardiac conduction system if initial non-invasive evaluation by other methods is uninformative [87–102,478-480].

ESC I A (CGR B, LOR 2)

  • Implantation of an implantable loop recorder*** for long-term recording of the electrical activity of the cardiac conduction system is recommended instead of a full traditional workup in patients with syncope of unclear origin and preserved left ventricular function when clinical or ECG data suggest an arrhythmogenic nature of the syncope [87–102].

ESC I A (CGR B, LOR 2)

Comments: The diagnostic significance of ILR*** data is beyond doubt and has been studied in the following clinical situations: in patients with suspected epilepsy unresponsive to treatment for this condition; in patients with a preliminary diagnosis of neurogenic syncope without an established mechanism of spontaneous syncope; in patients with bundle branch block in whom paroxysmal atrioventricular block is a probable diagnosis despite negative results of a complete electrophysiological study; in patients with structural heart disease and/or nonsustained ventricular tachyarrhythmia in whom ventricular tachyarrhythmia is likely present despite negative results of a complete electrophysiological study.

ILR*** data are considered diagnostically non-significant in cases where the connection between syncope and rhythm disturbances (bradycardia or tachycardia) is obvious; presyncopal states with signs of second- and third-degree AV block and pauses of more than 3 seconds (excluding situations during sleep and situations associated with rate-lowering pharmacotherapy), as well as tachycardias with a heart rate of more than 160 bpm lasting more than 32 complexes; during syncopal states, no arrhythmogenic cause is detected according to ILR*** data. ILR*** data have no diagnostic significance in cases where the arrhythmia is asymptomatic (except for the rhythm disturbances listed above); sinus bradycardia without a connection to symptoms.

2.5.7. Intracardiac electrophysiological study

Intracardiac electrophysiological study (EPS) is an invasive catheter procedure that can be used to check the cardiac conduction system and assess the potential inducibility of various tachyarrhythmias. EPS is well tolerated, and the risk of serious complications, such as cardiac tamponade and life-threatening arrhythmias, is minimal. The goal of EPS in the context of bradycardia evaluation is to identify the presence of abnormal sinus node function or atrioventricular conduction, as well as the anatomical level of the conduction disturbance. Pharmacological agents may be administered during EPS as part of the study protocol to modulate autonomic tone or provide "stress" to the sinus node, atrioventricular conduction, and intraventricular conduction. EPS in patients suspected of having bradycardia can reveal potential mechanisms for the onset of symptoms. EPS is typically not performed as a first-line diagnostic evaluation in patients with suspected bradycardia. Most patients undergoing EPS have already undergone a series of non-invasive cardiac investigations, such as an ECG, head-up tilt table test, echocardiogram, and/or ambulatory ECG monitoring, which were inconclusive.

EPS is also performed in patients with unexplained syncope or presyncope to verify cases associated with bradycardia.

  • In patients with symptoms presumably related to bradycardia, an intracardiac electrophysiological study is recommended to diagnose and determine the mechanism of bradycardia if initial non-invasive methods are uninformative [52,103–123,481,482].

ESC IIb B (CGR B, LOR 3)

  • In patients with syncope and bifascicular block, it is recommended to consider performing an EPS if, after non-invasive diagnostic methods, the cause of syncope remains unclear or if immediate decision-making regarding pacemaker implantation is required in severe patients in cases where empirical pacemaker implantation is undesirable (especially in elderly and frail patients) [482-488].

ESC IIa B (CGR B, LOR 3)

Comments: Clinical detection of possible sick sinus syndrome (SSS) is often difficult because the symptoms-causing anomalies may be transient and benign, and asymptomatic patients may exhibit significant variability in sinus rhythm. Various invasive tests have been proposed to assess sinus node function. Sinus node recovery time (SNRT) is used to assess the effect of overdrive suppression on sinus node automatism. Often, a correction for the sinus cycle length (SCL) is introduced, which is defined as the corrected sinus node recovery time (CSNRT = SNRT – SCL). Sinoatrial conduction can be measured directly or by delivering an atrial premature stimulus during sinus rhythm (3-5) or during atrial pacing.

It is possible to record sinus node electrograms using a catheter, and these direct measurements of conduction time correlate well with the indirect measurements described above. Sinus node recovery time and sinoatrial conduction time often differ from normal in patients with structural sinus node disease, but are usually normal in patients with sinus bradyarrhythmias caused by transient factors such as autonomic nervous system influences.

A full assessment of sinus node function often requires continuous or intermittent ambulatory ECG recording, stress tests to evaluate chronotropic incompetence, a tilt table test, or autonomic nervous system manipulations along with EPS data. Patients with SSS may also be prone to other arrhythmias that can be detected during EPS.

The His bundle electrogram allows the differentiation of three anatomical levels of heart block.

1. Proximal (above the bundle of His), reflecting conduction delay or block in the AV node: prolongation of the AH interval.

2. Medial (intra-Hisian), reflecting conduction delay or block within the bundle of His: prolongation of the His bundle spike duration.

3. Distal (infra-Hisian), reflecting a block or delay at the recording site distal to the His bundle spike: prolongation of the HV interval [129–131].

According to the electrocardiographic classification, the ventricular conduction system consists of three fascicles: the anterior and posterior fascicles of the left bundle branch and the right bundle branch. The HV interval in patients with bifascicular block characterizes the conditions and time of impulse conduction through the remaining intact bundle branch. Most data regarding the prognostic significance of the HV interval for the subsequent development of AV block were obtained in patients with bifascicular block. Patients with bifascicular block and a prolonged HV interval (>55 ms) have an increased risk of developing trifascicular block. Although HV interval prolongation is quite common, complete block develops rarely (usually 2–3% per year, especially if the HV interval exceeds 100 ms). At the same time, the probability of its progression in the absence of acute factors (drugs, electrolyte imbalances, ischemia) is low.

Thus, the HV interval has a high sensitivity (82%) but low specificity (63%) for predicting the development of complete trifascicular block. To increase the specificity of electrophysiological testing in patients with bifascicular block, rapid atrial pacing is used during EPS. An abnormal response consists of the occurrence of conduction block distal to the bundle of His during incremental atrial pacing with 1:1 AV nodal conduction. A functional block occurring distal to the bundle of His and associated with a sudden shortening of the coupling interval (such as during short-long cycle sequences in Wenckebach periodicity or at the onset of pacing) is not considered a positive result. The occurrence of distal His-Purkinje block induced by atrial pacing has a relatively low sensitivity, but its predictive value for the development of complete AV block is high.

Intracardiac electrophysiological study in patients with intraventricular conduction disturbances and unexplained symptoms should also include evaluation of the AV node and assessment of sinus node function, as well as programmed atrial and ventricular pacing to induce tachyarrhythmias in order to predict the development of both bradyarrhythmias and life-threatening tachyarrhythmias.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

Treatment should be divided into the correction of acute conditions, which may be reversible, and elective treatment of bradyarrhythmias.

In acute conditions, this involves pharmacotherapy and temporary cardiac pacing. For chronic bradycardias and conduction disturbances – permanent cardiac pacing using implantable pacemakers (PM***).

In some cases, sinus bradyarrhythmias are caused by potentially reversible factors, such as acute myocardial infarction, atrial tachyarrhythmias, electrolyte disturbances, hypothyroidism, medications, infections, and metabolic disorders.

  • Patients with sick sinus syndrome are recommended to undergo evaluation and treatment of reversible causes [124–131].

ESC I C (LoE C, GoR 4)

Comments: If patients are stable and have minimal symptoms of sick sinus syndrome, requiring therapy as well as evaluation of the syndrome and potentially reversible causes, this can be performed in an outpatient setting. In some cases, upon evaluating reversible causes, treatment may not be required (e.g., discontinuation of selective beta-blockers in a patient without angina who has sinus bradyarrhythmia following a myocardial infarction). Notably, some patients with tachycardia-bradycardia syndrome may experience an improvement in sinoatrial conduction after treatment aimed at maintaining sinus rhythm.

3.1. Pharmacological treatment of emergency conditions

For the acute treatment of bradycardia, pharmacotherapy can be used to temporarily increase heart rate and resolve the acute situation.

3.1.1. Acute medical treatment of sick sinus syndrome

  • In symptomatic or hemodynamically unstable patients with sick sinus syndrome, administration of Atropine** is recommended to increase the sinus rhythm rate [132–146].

ESC not specified (LoE C, GoR 4)

Comments: Atropine** is a belladonna alkaloid that blocks muscarinic acetylcholine receptors. In the sinus node, its action improves sinoatrial conduction and increases sinus node automaticity at doses ranging approximately from 0.5 to 2 mg, with a half-life of about 2 hours.

  • In symptomatic or hemodynamically unstable patients with sick sinus syndrome and a low probability of coronary artery disease, #Dopamine** is recommended as a drug to increase heart rate and improve symptoms [127–134].

ESC not specified (LoE B, GoR 2)

3.1.2. Acute medical treatment of AV block

  • In symptomatic or hemodynamically significant second- and third-degree atrioventricular block, administration of Atropine** is recommended to improve atrioventricular conduction and increase heart rate [137, 430–432].

ESC not specified (LoE C, GoR 4)

  • In symptomatic or hemodynamically significant second- and third-degree atrioventricular block with a low probability of coronary artery disease, #Dopamine** is recommended as a drug to improve atrioventricular conduction, increase heart rate, and improve symptoms [127–134].

ESC not specified (LoE B, GoR 2)

Comments: Acute treatment of bradycardia associated with atrioventricular block begins promptly with the identification and elimination of potential causative factors, as well as pharmacotherapy. Atropine** has a long history of use in these situations due to its ease of administration and relatively low risk of adverse reactions. It is most effective in atrioventricular block at the AV nodal level and in bradycardias caused by excessive vagal tone. Due to its short duration of action, it is usually used as a "bridge" to longer-term therapy, such as the administration of adrenergic agents (C01CA Adrenergic and dopaminergic agents) or transcutaneous temporary cardiac pacing or external temporary cardiac pacing.

Drug administration regimens – Appendix B, Table 2.

3.2. Temporary pacing for emergency bradyarrhythmia conditions

Placement of a temporary single-chamber non-rate-responsive pacemaker is used for the acute treatment of bradycardias causing hemodynamic instability, such as prolonged and symptomatic pauses, bradycardia-mediated life-threatening ventricular arrhythmias, or severe symptomatic bradycardias due to a reversible cause.

Placement of a temporary single-chamber non-rate-responsive pacemaker is used as an interim step before permanent pacemaker implantation*** or until the acute situation resolves. Temporary cardiac pacing is performed transcutaneously, via a transesophageal approach, or by introducing a transvenous pacing lead or pacing catheter. Emergency temporary pacing for the treatment of bradycardia associated with hemodynamic instability typically involves right ventricular pacing due to the ease of transvenous access in bradycardias caused by sick sinus syndrome or atrioventricular block. In rare cases, temporary right atrial pacing is used (alone or in combination with ventricular pacing) when maintaining atrioventricular synchrony is critical.

  • Temporary cardiac pacing is recommended for hemodynamically significant bradyarrhythmia refractory to intravenous heart-rate-increasing drugs [153, 489].

ESC I C (LoE C, LoR 3)

  • Temporary cardiac pacing is recommended for hemodynamically significant bradyarrhythmia when temporary transvenous cardiac pacing is impossible or unavailable [164, 490, 491].

ESC IIa C (LoE B, LoR 3)

  • Temporary cardiac pacing is recommended for reversible causes and emergency pacing indications, as well as in myocardial ischemia, myocarditis, electrolyte disturbances, intoxication, or following cardiac surgery [147, 151, 166].

ESC IIa C (LoE C, LoR 4)

  • Temporary cardiac pacing is recommended as a temporary measure prior to permanent pacemaker implantation when this procedure is unavailable or contraindicated due to concomitant infection [147, 151, 166].

ESC IIa C (LoE C LoR 4)

  • For long-term temporary cardiac pacing, active-fixation leads connected to an external temporary pacemaker are recommended [183, 492, 493, 494].

ESC IIa C (LoE B LoR 2)

3.2.1. Temporary pacing in sick sinus syndrome

  • In patients with persistent hemodynamically unstable sick sinus syndrome refractory to pharmacotherapy, placement of a temporary single-chamber or dual-chamber non-rate-responsive pacemaker*** is recommended to increase heart rate and improve symptoms until a permanent pacemaker is implanted or the bradycardia is resolved [143–161].

ESC Ι C (LoE B, LoR 2)

  • In patients with SSS and prominent symptoms or unstable hemodynamics, temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is recommended to increase heart rate and improve symptoms prior to permanent pacemaker implantation or resolution of bradycardia [143–161].

ESC no (LoE A, GoR 2)

  • In patients with SSS and minimal and/or infrequent symptoms without hemodynamic instability, temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is not recommended [162–165].

ESC/RSC IIIC (LoE C, GoR 4)

Comments: The use of temporary transvenous pacing for bradycardias is rare because the risk of acute adverse cardiovascular events associated with SSS or AV block is low, while temporary pacing is associated with complications. Adverse event rates related to temporary transvenous access are reported to range from 14 to 40%. Complications include venous thrombosis (18–85% when inserted via the femoral route, with the femoral vein used for access), pulmonary embolism (50–60% with the femoral approach), life-threatening arrhythmias (usually related to lead instability or position in the right ventricle), loss of capture (10–37%), perforation, and death.

The risk of complications can be reduced by using balloon valvuloplasty or fluoroscopy. The risk of infectious complications during permanent pacemaker insertion increases in patients who have had a temporary pacemaker prior to permanent implantation. However, in some cases, temporary single-chamber non-rate-responsive pacemaker insertion is considered clinically necessary.

3.2.2. Temporary pacing in AV block

  • In patients with symptomatic grade II and III AV block with hemodynamic instability and refractoriness to medical therapy, temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is recommended to increase heart rate and improve symptoms [166–183].

ESC IC (LoE C, GoR 4)

  • In patients with symptomatic grade II and III AV block with hemodynamic instability and refractoriness to medical therapy, temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is recommended prior to permanent pacemaker implantation [183–192].

No ESC guideline (LoE C, GoR 5)

Comments: The cause of AV block should be considered when determining the timing and necessity of temporary pacing. Developed over 60 years ago, temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion plays a role in the acute management of AV block.

Algorithm of physician actions – Appendix B, Table 3.

3.2.3. Temporary pacing in patients at risk of bradycardia during non-cardiac surgery

  • In patients at high risk of intraoperative or periprocedural bradycardia due to patient characteristics or type of procedure, prophylactic temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is recommended [456-460].

ESC not specified (LoE C, GoR 4)

  • In patients with LBBB who require pulmonary artery catheterization for intraoperative monitoring, routine prophylactic temporary single-chamber or dual-chamber non-rate-responsive pacemaker insertion is not recommended [456-460]

ESC not specified (LoE C, GoR 4)

3.3. Permanent cardiac pacing

Permanent cardiac pacing is performed by implanting a pacemaker (PM) – single-, dual-, or triple-chamber. Access routes for implantation can be endocardial or epicardial. In the first case, the leads of the pacing system are implanted transvenously into the heart, while in the second case, implantation is performed epicardially during open-heart surgery or separately via minithoracotomy.

3.3.1. Permanent pacing in sick sinus syndrome

Permanent cardiac pacing has been shown to alleviate symptoms of cerebral hypoperfusion associated with bradycardia when other possible treatable or reversible causes have been excluded. Symptomatic SSS is the most common indication for permanent pacing, followed by atrioventricular block. The best response to pacing is demonstrated when a correlation between symptoms and bradycardia is identified. The benefit of pacing in SSS is a significant improvement in quality of life.

  • In patients with symptomatic bradycardia due to SSS, pacemaker implantation is recommended to increase heart rate and improve symptoms [193–197].

ESC IB (LoE C, GoR 4)

  • In patients with symptomatic sinus bradycardia who are receiving medical therapy and continuation of treatment is clinically necessary, pacemaker implantation is recommended to increase heart rate and improve symptoms [193–197].

No ESC guideline (LoE C, GoR 5)

  • In patients with tachycardia-bradycardia syndrome and symptoms related to bradycardia, pacemaker implantation is recommended to increase heart rate and reduce hypoperfusion-related symptoms [434-443].

ESC IB (LoE B, GoR 2)

  • In patients with symptomatic chronotropic incompetence, pacemaker implantation with rate-responsive programming is recommended to increase heart rate and improve symptoms [434-443].

ESC IIA (LoE B, GoR 2)

  • In patients with symptomatic SSS and normal atrioventricular conduction, pacemaker implantation is recommended [434-443].

ESC IB (LoE B, GoR 2)

  • In patients with symptomatic SSS who have an implanted dual-chamber PM*** and normal atrioventricular conduction, a dual-chamber mode minimizing ventricular pacing is recommended [434-443,501-507].

ESC IA (LoE A, GoR 2)

  • Pacemaker implantation is recommended in symptomatic patients with brady-tachy syndrome to treat bradyarrhythmia and enable medical therapy if tachyarrhythmia ablation is not preferred [436,437,508-511,513].

ESC IB (LoE A, GoR 2)

  • In patients with symptomatic chronotropic incompetence, dual-chamber rate-responsive pacemaker implantation is recommended [514-515].

ESC IIa B (LoE A, GoR 2)

Catheter ablation of atrial fibrillation is recommended as a treatment strategy to avoid pacemaker implantation in patients with AF-associated bradycardia and symptomatic pre-ablation pauses after AF conversion, depending on the clinical scenario [510-518].

ESC IIa C (LoE C, GoR 3)

  • In patients with brady-tachy syndrome, PM*** programming should be considered [519-520].

ESC IIb C (LoE A, GoR 2)

  • In patients with syncope, pacemaker implantation should be considered to reduce recurrent syncope if an asymptomatic pause > 6 s due to sinus arrest is documented [96, 521].

ESC IIb C (LoE A, GoR 2)

  • Pacemaker therapy is not recommended in patients with bradycardia due to SSS if it is asymptomatic or attributable to reversible causes that can be corrected and prevented [353].

ESC III C (LoE C, GoR 5)

Comments: In SSS, atrial pacing modes offer advantages over ventricular pacing. A positive impact of atrial pacing modes on the prevention of atrial fibrillation, heart failure, or stroke has been noted, and these modes also help improve patients' quality of life.

3.3.2. Permanent pacing in AV block

  • In patients with acquired Mobitz type II second-degree AV block, high-grade AV block, or third-degree AV block not due to reversible or physiological causes, permanent pacemaker implantation is recommended regardless of symptoms [444-450].

ESC IC (LoE B, GoR 3)

  • In patients with neuromuscular diseases associated with conduction disorders, including muscular dystrophy (e.g., myotonic dystrophy type), who have evidence of grade II or III AV block or an HV interval of 70 ms or greater, permanent pacing is recommended regardless of symptoms [200–210].

ESC IB (LoE B, GoR 3)

  • In patients with permanent atrial fibrillation and symptomatic bradycardia, permanent pacing is recommended [198–210].

ESC I C (LoE C, GoR 4)

  • In patients who develop symptomatic AV block as a result of therapy where no alternative treatment exists and continued treatment is clinically necessary, permanent pacing is recommended to increase heart rate and improve symptoms [200–210].

ESC not specified (LoE C, GoR 4)

Comments: All bradycardias with potentially reversible causes are classified under Class III in ESC guidelines—permanent pacing is unnecessary.

  • In patients with sarcoidosis or amyloidosis, Mobitz type II second-degree AV block, high-grade AV block, or third-degree AV block, permanent pacing is recommended [198–210].

ESC not stated (CGR B, LOR 3)

  • In patients with muscular dystrophies with a PR interval exceeding 240 ms and LBBB, permanent pacing is recommended [200–210].

ESC not stated (CGR B, LOR 3)

  • In patients with first-degree or Mobitz type I second-degree AV block with clear symptoms characteristic of AV block, permanent pacing is recommended [200–210].

ESC IIA (LoE B, GoR 3)

  • In patients with neuromuscular diseases (such as myotonic dystrophy type I) with a PR interval > 240 ms, QRS duration > 200 ms, or trifascicular block, permanent pacing is recommended [205–210].

ESC not stated (CGR B, LOR 3)

Comments: The presence of symptoms is an important factor in determining the need for permanent pacing. If a patient is symptomatic, regardless of the level of AV block and the likelihood of future AV block progression, permanent pacing is indicated. However, unlike sinus node dysfunction, infranonadal AV block, regardless of the presence or absence of symptoms, mandates permanent pacing because symptoms may start abruptly as a result of complete AV block and manifest as syncope.

  • In patients with sinus node dysfunction and AV block who require permanent pacing, dual-chamber pacing is recommended [198–225].

ESC IIA (LoE A, LoR 2)

  • In patients with sinus rhythm and single-chamber ventricular pacing who develop pacemaker syndrome, switching to dual-chamber pacing is recommended [198–225].

ESC IA (LoE A, LoR 2)

  • In patients with AV block who have an indication for permanent pacing with an LVEF between 30% and 50% and are expected to require ventricular pacing for > 40% of the time, pacing modalities that maintain physiological ventricular activation (e.g., biventricular pacing or His bundle pacing) are recommended as they have advantages over conventional right ventricular pacing [444–454].

ESC not specified (LoE C, GoR 4)

  • In patients with permanent or persistent AF who are not scheduled for a rhythm control strategy, implantation of a dual-chamber pacemaker is not recommended [210–225].

ESC IC (LoE B, LoR 2)

  • In patients with permanent AF and an indication for pacing***, implantation of a dual-chamber pacemaker with rate-adaptive function is recommended [522-524].

ESC IC (LoE B, LoR 2)

  • Pacemaker implantation is recommended in patients with symptomatic second-degree Mobitz I AV block, or when intra- or infra-Hisian AV block is detected on EPS [200,525,526,444].

ESC IIa C (LoE C, LoR 4)

  • Permanent pacemaker implantation is recommended in patients with persistent symptoms characteristic of pacemaker syndrome associated with first-degree AV block (PR > 300 ms) [527-529].

ESC IIa C (LoE C, LoR 4)

Comments: The advantages of dual-chamber pacing over ventricular pacing in AV block are obvious, but dual-chamber pacing has not been shown to affect all-cause mortality or cardiovascular mortality. This has not been demonstrated due to ethical reasons—the impossibility of planning prospective studies on this issue.

However, regardless of the pacing mode, patients with AV block require the implantation of a dual-chamber pacemaker. Specialized pacing modalities, such as transvenous biventricular pacemaker implantation, can mitigate the adverse effects of right ventricular pacing in these patients. When determining the pacemaker type (single-chamber, dual-chamber, biventricular) in many patients, all factors must be considered, including the projected percentage of ventricular pacing and LVEF. Biventricular pacing is considered beneficial for patients with an LVEF ≤ 35% with an expected need for significant ventricular pacing (> 40%).

3.3.3. Cardiac Pacing for Conduction Disorders (with 1:1 Atrioventricular Conduction and Normal PR Interval)

  • In patients with syncope and bundle branch block (BBB) with an HV interval of 70 ms or more, or intra-nodal block proven by EPS, pacemaker implantation is recommended [226–230].

ESC IB (LoE B, LoR 3)

  • In patients with alternating bundle branch block, with or without symptoms, permanent pacing is recommended [226–236].

ESC IC (LoE A, LoR 3)

  • In asymptomatic patients with isolated conduction disturbance, 1:1 atrioventricular conduction, and no other indications for pacing, permanent pacing is not recommended [226–236].

ESC IIIB (LoE A, LoR 3)

It is recommended to consider permanent pacing in patients with unexplained syncope and bifascicular block without EPS data (elderly, frail patients, high-risk patients, or those with recurrent syncope) [530].

ESC IIb C (LoE A, GoR 2)

Comments: Patients with conduction disorders with 1:1 atrioventricular conduction and a normal PR interval require further diagnosis of the underlying heart disease, assessment of symptoms, and analysis of baseline ECG (bundle branch block, nonspecific intraventricular conduction delay, isolated fascicular block, or a combination of abnormalities).

Management depends on the presence or absence of symptoms in the setting of an underlying disorder associated with disease progression (e.g., muscular dystrophy). Alternating bundle branch block (QRS complexes with alternating LBBB and RBBB morphologies) indicates significant conduction system disease and a high probability of sudden-onset complete heart block with a slow or absent ventricular escape rhythm.

3.3.4. Implantation of a triple-chamber pacemaker (cardiac resynchronization therapy)

The concept of CRT is based on the fact that patients with HF and systolic LV dysfunction frequently exhibit marked intraventricular conduction disturbances, with a QRS duration > 120 ms in 25–50% of cases and LBBB in 15–27% of cases. Moreover, in such patients, RV dyssynchrony is frequently combined with PR interval prolongation on the surface ECG [531-533].

The described electrical abnormalities can cause atrioventricular, interventricular, and intra-left ventricular mechanical dyssynchrony [534-535].

In a recent large meta-analysis of 5 RCTs (COMPANION, CARE-HF, MADIT-CRT, RAFT, and REVERSE) involving 6523 participants (1755 patients with non-LBBB QRS morphology), CRT was not associated with a reduction in mortality and/or HF hospitalizations in patients without LBBB (RR, 0.99, 95% CI 0.82-1.2) [536].

Since in almost all studies and post-hoc analyses of the favorable impact of QRS morphology on CRT efficacy, patients were grouped into the "non-LBBB" category, it is impossible to provide separate recommendations for CRT in patients with diffuse intraventricular conduction delays and RBBB [537-542]. Patients with RBBB do not derive a positive effect from CRT [543] unless they have a so-called masked LBBB on the ECG [542], characterized by a broad, notched or slurred R wave in leads I and aVL combined with left axis deviation. In these patients, individualized LV lead positioning is crucial.

Recently, the concept of a potential role for a prolonged PR interval in HF patients without LBBB has emerged. Several single-center studies and two post-hoc analyses of large RCTs (COMPANION and MADIT-CRT) have demonstrated potential benefit from CRT implantation in this patient subgroup [543-544].

ECG criteria for intraventricular conduction disturbances, LBBB and non-LBBB QRS morphologies were not previously defined or presented in CRT studies [543-544]. Furthermore, the method of QRS duration measurement (automated or manual, and the ECG machine used) was not reported. However, the choice of ECG criteria likely influences hard endpoints [545-549]. Similarly, the method of ECG recording and the ECG machine manufacturer have been shown to influence the automatically measured QRS duration.

Recommendations for CRT in Sinus Rhythm Patients with LBBB QRS Morphology

  • Implantation of a biventricular pacemaker (cardiac resynchronization therapy) is recommended for symptomatic patients with HF in sinus rhythm, LVEF ≤ 35%, QRS duration ≥ 150 ms, and LBBB QRS morphology despite optimal medical therapy, to improve symptoms, reduce morbidity, and decrease mortality [550-567].

ESC I A (LoE B, LoR 1)

  • CRT is recommended for symptomatic patients with HF in sinus rhythm, LVEF ≤ 35%, QRS duration 130-149 ms, and LBBB QRS morphology despite optimal medical therapy, to improve symptoms, reduce morbidity, and decrease mortality [550-567].

ESC IIa B (LoE C, LoR 1)

Recommendations for CRT in Sinus Rhythm Patients with Non-LBBB QRS Morphology

  • CRT is recommended for symptomatic patients with HF in sinus rhythm, LVEF ≤ 35%, QRS duration ≥ 150 ms, and non-LBBB QRS morphology despite optimal medical therapy, to improve symptoms, reduce morbidity, and decrease mortality [550-567].

ESC IIa B (LoE B, LoR 1)

It is recommended to consider CRT in symptomatic patients with HF in sinus rhythm, LVEF ≤ 35%, QRS duration 130-149 ms, and non-LBBB QRS morphology despite optimal medical therapy, to improve symptoms and reduce morbidity [568-574].

ESC IIb B (LoE B, LoR 1)

  • CRT is not recommended in patients with HF and QRS duration < 130 ms without an indication for RV pacing [563, 575].

ESC III A (LoE A, GoR 2)

Recommendations on CRT in patients with persistent and permanent AF.

Patients with permanent AF and HF who are candidates for CRT:

  • CRT is recommended for patients with permanent AF, NYHA functional class III and IV, and LVEF ≤35% on optimal medical therapy with a QRS duration ≥130 ms, providing effective biventricular pacing to reduce symptoms, morbidity, and mortality risk [576-579].

ESC IIa C (LoE B, GoR 2)

  • Creation of iatrogenic atrioventricular block is recommended when biventricular pacing is suboptimal during AF (< 90-95%) [579-580].

ESC IIa B (CGR A, LOR 2)

Patients with symptomatic AF and uncontrolled ventricular rate who are candidates for AV junction ablation (regardless of QRS duration):

  • CRT is recommended for patients with HFmrEF/HFrEF [580-583].

ESC I B (LoE A, GoR 2)

  • CRT is recommended instead of RV pacing in patients with HFmrEF [580-583].

ESC IIa C (LoE C, LoR 4)

  • RV pacing is recommended in patients with HFpEF [217,582,585].

ESC IIa B (CGR A, LOR 2)

  • It is recommended to consider CRT implantation in patients with HFmrEF [584].

ESC IIb C (LoE C, GoR 5)

Recommendations on upgrading from RV pacing to CRT

Replacement of an implanted pacemaker (coded under NMU as Removal or replacement of an implanted pacemaker) with CRT is recommended in patients with a high percentage of RV pacing, LVEF ≤35%, and progressive HF despite optimal medical therapy [586-600].

ESC IIa B (CGR A, LOR 2)

An RV pacing burden of 20%, considered a threshold capable of inducing pacing-induced HF, is supported by observational data. However, there is no evidence that any specific percentage of RV pacing can be defined as a limit below which RV pacing is safe and above which it is harmful. For patients with preserved LVEF, data regarding the benefit of CRT on HF hospitalizations are conflicting. No survival benefits of CRT have been demonstrated either [585,601-603]. However, biventricular pacing prevented adverse remodeling induced by RV pacing, particularly during long-term follow-up [585,601-604]. In any case, adverse remodeling caused by RV pacing can be prevented by biventricular pacing, especially in the long term. A single-center study showed that RV pacing > 20% was associated with pathological LV remodeling in patients with AV block and preserved LVEF [605].

  • CRT, rather than RV pacing, is recommended for patients with HFrEF (< 40%), regardless of NYHA functional class, who have an indication for ventricular pacing and high-grade AV block to reduce morbidity, including patients with AF [580-583].

ESC I A (LoE A, GoR 2)

3.3.5. Conduction system pacing

Alternative RV pacing sites (as opposed to RV apical pacing) include the RV outflow tract, mid and basal interventricular septum (IVS), His bundle pacing (HBP), parahisian pacing, and left bundle branch area pacing (LBBAP), which includes left ventricular septal pacing and left bundle branch pacing.

Implantation of an additional right ventricular lead is recommended for patients undergoing HBP in the following situations (pacemaker dependence, high-degree AV block, infranodal AV block, high pacing threshold, anticipated AV block) or to improve sensing (e.g., risk of undersensing ventricular events or oversensing atrial events and His potentials) [609,613,614].

ESC IIa C (LoE B, GoR 3)

  • In patients with a pacemaker*** and HBP or LBBAP, device programming is recommended to be performed in accordance with specific requirements for cardiac conduction system pacing [606-607].

ESC I C (LoE C, GoR 5)

It is recommended to consider HBP or LBBAP as an alternative to RV pacing in patients with AV block and LVEF > 40% who are expected to require > 20% ventricular pacing [619-620].

RSC IIb C (LoE C, GoR 4)

Comment: in some patients with reduced LVEF, LBBAP may be considered as an alternative to left ventricular lead implantation after careful weighing of potential risks and benefits, and taking into account the lack of long-term follow-up results from multicenter randomized trials comparing LBBAP with conventional CRT, which should also be discussed with the patient and/or their legal representative [706].

HBP or LBBAP is recommended for CRT candidates in whom previous attempts to implant a left ventricular lead via the coronary sinus vein have failed [608-612].

ESC IIb B (LoE C, GoR 4)

It is recommended to consider HBP or LBBAP in patients who have an indication for the "pace-and-ablate" strategy for tachyarrhythmic supraventricular arrhythmias, especially those with a narrow intrinsic QRS complex [615-618].

ESC IIb C (LoE C, GoR 4)

Comment: a backup ventricular lead may be implanted at the operator's discretion.

3.4. Indications for pacing in specific types of pathological conditions

3.4.1. Pacing in patients after certain cardiac surgeries

The risks of bradyarrhythmias after cardiac surgery are largely related to the type of cardiac surgical intervention. Specific cardiac surgeries and conditions include coronary artery bypass grafting, open heart valve surgeries (including aortic, tricuspid, and mitral valve interventions), transcatheter aortic valve implantation; congenital heart disease surgeries; heart transplantation, and postoperative complications following AF treatment.

Recovery of atrioventricular conduction after surgery occurs in approximately 12% to 13% of patients within 6 months and depends on the type of surgery, preoperative conduction disturbances, presence of endocarditis, and the postoperative course, specifically whether transient postoperative atrioventricular conduction abnormalities are observed.

  • Permanent pacing is recommended for patients with postoperative symptomatic SSS, AV block, or hemodynamic instability that does not resolve following cardiac surgery [237-253, 260–268, 269–278].

ESC IC (LoE B, LoR 2)

A clinical observation period of at least 5 days is recommended in patients with advanced or complete AV block after cardiac surgery to rule out potential transient cardiac rhythm disturbances [621, 623].

ESC I C (LoE C, GoR 5)

Surgical treatment of valvular endocarditis and intraoperative complete AV block

Implantation of a temporary/permanent epicardial pacing system is recommended in patients with complete AV block after cardiac surgery for valvular endocarditis if one of the following factors is present: pre-existing conduction disturbances, Staphylococcus aureus infection, intracardiac abscess, tricuspid valve involvement, or previous valve surgery [622].

ESC IIa C (LoE C, GoR 4)

  • Temporary epicardial pacing system implantation is recommended in patients undergoing open-heart surgery [237–271, 275–278].

ESC not specified (LoE B, GoR 2)

  • Left ventricular epicardial lead implantation is recommended in patients undergoing open-heart surgery who require biventricular or ventricular pacing [250–259, 265–268, 270–274].

ESC not specified (LoE C, GoR 4)

Comments: The incidence of conduction disturbances following open isolated coronary artery bypass grafting ranges from 2% to 58%, and this complication is associated with factors such as chronic degenerative heart disease, direct surgical trauma to the conduction system, myocardial ischemia, or inadequate myocardial protection. Advances in modern surgical practice help reduce the incidence of conduction disturbances but cannot eliminate them entirely.

Successful surgical correction of AF is associated with improved patient survival compared to patients with recurrent AF, with 65% of patients undergoing mitral valve replacement simultaneously undergoing surgical correction of AF. A single-center study showed that 11% of patients undergoing the Cox-Maze IV procedure in addition to mitral valve repair require postoperative pacemaker implantation [278–279].

The most common causes of conduction disturbances following surgical aortic valve replacement are edema, manipulation trauma during calcium removal, or injury due to deeply placed sutures. The need for cardiac pacing after aortic valve replacement is common, ranging from 3% to 8.5%, with the highest risk likely in patients with preoperative conduction disturbances. Available data indicate that most patients do not recover atrioventricular conduction. One study showed that patients who required pacemaker implantation within 30 days after aortic valve replacement had a higher risk of death.

The occurrence of complete heart block is noted in 23.5% of patients undergoing mitral valve replacement or plastic repair. A study of 55 hearts from patients without cardiac surgery evaluated the relationship between the atrioventricular node, the atrioventricular nodal artery, and the mitral annulus. In 23% of cases, the atrioventricular nodal artery passed close to the mitral valve, suggesting that injury to the artery may play a role in the development of complete heart block after mitral valve surgery. The need for pacing*** after mitral correction ranges from 1% to 9%. This depends on the type of surgery and may be lower after reconstructive procedures, in which incomplete annuloplasty may be associated with a lower risk of atrioventricular nodal artery injury.

3.4.2. Recommendations for cardiac pacing in patients after surgical interventions on the tricuspid valve:

It is recommended to implant an epicardial pacing system and avoid the implantation of endoventricular leads across the tricuspid valve. During tricuspid valve surgery, preference should be given to removing a previously implanted right ventricular lead rather than anchoring it between the fibrous annulus of the valve and an allogenic patch, or between the fibrous annulus and the support ring. In the case of isolated tricuspid annuloplasty, weighing individual risks and benefits, a previously implanted right ventricular lead may be left in place and not secured between the fibrous and support rings [622, 624].

ESC IIa C (LoE C, LoR 4)

Patients requiring pacing after tricuspid valve replacement or tricuspid ring annuloplasty

  • When indications for ventricular pacing are present, it is recommended to prefer minimally invasive open surgical implantation of a single- or dual-chamber pacemaker or open surgical implantation of a triple-chamber (biventricular) pacemaker [624].

ESC IIa C (LoE C, LoR 4)

Patients requiring pacing after mechanical tricuspid valve replacement

  • Pacemaker implantation across a mechanical tricuspid valve is not recommended [624].

ESC III C (LoE C, GoR 4)

Comments: The atrioventricular node is closely connected to the tricuspid valve and is located between its anterior and septal cusps; this makes the atrioventricular node particularly susceptible to injury during any interventions on the tricuspid valve. Surgeons have developed numerous reconstruction techniques to avoid node trauma, including several options for incomplete tricuspid annuloplasty rings that have gaps between the anterior and septal cusps. These techniques avoid suture placement in the area of the atrioventricular node and, consequently, its injury.

It has been reported that the need for a pacemaker after reconstruction is as low as 2.3%. Because isolated tricuspid surgery is rarely performed in the adult population, the exact incidence of conduction disturbances associated with isolated tricuspid intervention is difficult to establish. Several studies have reported a much higher incidence of AV conduction disturbances—up to 22%. The management of conduction disturbances in patients with a mechanical tricuspid valve is further complicated by the impossibility of using transvenous leads for pacing, as the artificial valve impairs closure when a lead is implanted in the right ventricle. Even native valves exhibit significant tricuspid regurgitation in nearly a quarter of cases after lead placement. In patients who receive a pacemaker*** after tricuspid valve reconstruction, the incidence of varying degrees of regurgitation is up to 42%.

3.4.3. Recommendations for cardiac pacing in patients after transcatheter aortic valve implantation:

The rate of pacemaker implantation following TAVI ranges from 3.4% to 25.9% in RCTs and large registries [625-637]. While the association between pacing after TAVI and outcomes is controversial [638-643], right ventricular pacing is believed to lead to worsening left ventricular function [216, 644, 645].

Therefore, unnecessary permanent right ventricular pacing must be minimized.

Predictors of permanent pacing after TAVI [455]

ECG:

- Right bundle branch block

- PR interval prolongation / left bundle branch block

- Patient-related:

- Advanced age (risk increases with each year)

- Male sex

- Higher body mass index (risk increases with each unit of weight)

Anatomical features:

- Severe mitral annular calcification

- Left ventricular outflow tract calcification

- Length of the membranous interventricular septum

- “Porcelain aorta”

- High mean aortic valve gradient

Procedural characteristics:

- Self-expanding valve

- Deeper valve implantation

- Higher ratio between prosthesis diameter and annular or LV outflow tract diameter

- Balloon post-dilation

- TAVI in native valve vs. “valve-in-valve” implantation

  • Permanent pacing is recommended for patients with advanced or complete AV block persisting > 24–48 h after TAVI [279–296, 647].

ESC I B (CARR A, LER 2)

  • Permanent pacing is recommended for patients with new-onset alternating bundle branch block after TAVI [279–296, 646].

ESC I C (CARR A, LER 2)

Early permanent pacing is recommended in patients with pre-existing RBBB who develop progressive or new conduction disturbances after TAVI [279–296, 646].

ESC IIa B (CARR B, LER 2)

Multi-day ECG monitoring or EPS is recommended for patients with new-onset LBBB with QRS > 150 ms or PR > 240 ms without further progression after > 48 h following TAVI [648–650, 715].

ESC IIa C (LoE C, LoR 4)

It is recommended to consider ambulatory ECG monitoring or EPS for patients with pre-existing conduction disturbances with a subsequent increase in QRS duration or PR > 20 ms after TAVI [648–650, 715].

ESC IIb C (LoE C, GoR 5)

  • In patients with RBBB who do not have indications for permanent pacing, prophylactic permanent pacemaker*** implantation before the TAVI procedure is not recommended [648–650].

ESC III C (CARR C, LER 3)

Comments: Following transcatheter aortic valve implantation, LBBB occurs in 19–55% of patients and high-degree AV block in approximately 10% of patients. In half of the cases, conduction disturbances resolve. Furthermore, only about half of the patients will subsequently develop conduction disturbances requiring a permanent pacemaker***. The likelihood of new conduction disturbances depends on patient clinical features and peri-procedural factors. Following aortic valve implantation, new conduction disturbances are associated with an increased risk of pacemaker*** implantation and an increased risk of late all-cause and cardiac mortality, regardless of whether a new pacemaker*** was implanted.

3.4.4. Recommendations for pacing after heart transplantation

  • For SSS following cardiac surgery and heart transplantation, it is recommended to allow an observation period of up to 6 weeks before permanent pacemaker implantation [651, 714].

ESC IIa C (CARR C, LER 5)

Permanent pacemaker*** implantation is recommended for chronotropic incompetence persisting for more than 6 weeks after heart transplantation to improve quality of life [652, 714].

ESC IIa C (CARR C, LER 5)

3.4.5. Pacing in patients with congenital heart disease

Permanent pacing in patients with complex and moderately complex congenital heart disease should be performed in specialized centers with a multidisciplinary team of congenital heart disease and pacing specialists. As a rule, the decision to implant a permanent pacemaker in patients with congenital heart disease is based on expert consensus and individual assessment due to the lack of evidence from randomized controlled trials. In the presence of an intracardiac shunt between the systemic and pulmonary circulations, endocardial lead implantation is relatively contraindicated due to the risk of systemic arterial embolism [314].

  • Permanent pacing is recommended for adult patients with congenital heart disease and symptomatic SSS or chronotropic incompetence [297–314].

ESC IIb B (CARR A, LER 3)

  • Permanent pacing is recommended for adult patients with congenital heart disease and symptomatic AV block [297–314].

ESC IC (LoE A, LoR 3)

  • In adult patients with congenital heart disease (CHD) and congenital AV block of any degree with symptomatic bradycardia, wide QRS, daytime mean heart rate below 50 bpm, ventricular ectopy, or ventricular dysfunction, pauses exceeding three times the escape ventricular cycle length, or long QT interval, permanent pacing is recommended [297–314].

ESC IC (LoE A, LoR 3)

  • In adult patients with CHD and postoperative second-degree Mobitz II AV block, high-grade AV block, or third-degree AV block that does not resolve spontaneously, permanent pacing is recommended [297–314].

ESC no (Level of Evidence A, Grade of Recommendation 3)

  • In asymptomatic adult patients with CHD and congenital complete AV block, permanent pacing is recommended even in the absence of risk factors [305–314].

ESC IIb (Level of Evidence B, Grade of Recommendation 3)

  • In adult patients with CHD and pre-existing sinus node dysfunction and/or AV block during cardiac surgery, intraoperative implantation of an epicardial pacing system is recommended [305–314].

ESC not specified (LoE C, GoR 4)

  • In adult patients with CHD and systemic intracardiac shunts, implantation of a pacemaker with endocardial leads is not recommended [297–314].

ESC not specified (LoE C, GoR 4)

  • In patients with persistent postoperative bifascicular block combined with transient complete AV block, it is recommended to consider permanent pacemaker implantation*** [653, 714].

ESC IIb C (LoE C, GoR 5)

  • In patients with complex CHD and asymptomatic bradycardia (resting heart rate < 40 bpm or pauses > 3 s), it is recommended to consider permanent pacemaker implantation*** on an individual basis [653, 714].

ESC IIb C (LoE C, GoR 5)

Comments: Adult patients with CHD represent a diverse group of patients with varying conduction system anatomy, venous return to the heart, and progression of conduction disturbances. Many CHD types have specific features related to atrial hypertrophy, dilation, or remodeling. All factors should be considered before endocardial lead placement. Specialized care for such patients should be provided in specialized centers with experience in managing this patient population.

3.4.6. Pacing in patients with myocardial infarction

  • In patients with acute myocardial infarction (MI), temporary pacing is recommended for symptomatic or hemodynamically significant bradycardia refractory to medical therapy associated with sinus node dysfunction or AV block [315–336].

ESC IC (LoE B, LoR 2)

  • In patients with acute MI and second-degree Mobitz II AV block, high-grade AV block, bundle branch blocks, or third-degree AV block, permanent pacing is recommended [315–336].

ESC not specified (LoE B, GoR 2)

  • In patients with acute MI and symptomatic or hemodynamically significant sinus bradycardia or AV block, administration of Atropine** is recommended [325].

ESC not specified (LoE C, GoR 4)

  • In patients with acute MI and transient AV block that resolves spontaneously, permanent pacing is not recommended [315–336].

ESC not specified (LoE B, GoR 2)

  • In patients with acute MI and bundle branch block or isolated fascicular block in the absence of second- and third-degree AV block, permanent pacing is not recommended [315–336].

ESC not specified (LoE B, GoR 2)

Implantation of a permanent pacemaker*** is recommended in accordance with guidelines for the general population when AV block does not resolve during a waiting period of at least 5 days after MI [315–336].

ESC I C (Level of Evidence B, Grade of Recommendation 3)

  • In selected patients with AV block and acute heart failure occurring in the setting of anterior wall MI, it is recommended to consider early implantation of a biventricular pacemaker (cardiac resynchronization therapy) (CRT-P/CRT-D) [654].

ESC IIb C (LoE C, GoR 5)

  • Pacemaker implantation*** is not recommended if AV block resolves spontaneously [319, 323, 326, 655].

ESC III C (Level of Evidence B, Grade of Recommendation 3)

Comments: Although transient sinus node dysfunction may occur in the context of acute MI, irreversible damage to the atrioventricular junction accounts for the majority of pacing indications. For example, sinus node dysfunction and AV block in inferior wall MI may be influenced by transiently increased vagal tone or reduced blood supply to the atrioventricular junction – or, less commonly, the sinoatrial junction. The need for temporary pacing is not an indication for permanent pacing per se. The long-term prognosis for patients after MI and AV block depends primarily on the extent of myocardial damage and the nature of intraventricular conduction disturbances, rather than on the AV block itself. Regardless of whether the infarction is anterior or inferior, the development of intraventricular conduction disturbances rather reflects extensive myocardial injury.

3.4.7. Pacing in patients with syncopal conditions

  • In patients aged > 40 years with spontaneous symptomatic verified asystole > 3 seconds or asymptomatic pauses > 6 seconds due to sinus arrest, AV block, or their combination, pacemaker implantation*** is recommended to reduce syncope recurrence [337–352].

ESC I A (CGR B, LOR 2)

  • In patients aged > 40 years with recurrent unpredictable syncope and cardioinhibitory carotid sinus syndrome, pacemaker implantation*** is recommended to reduce syncope recurrence [337–352].

ESC I A (LoE A, GoR 2)

  • In patients aged > 40 years with recurrent unpredictable syncope and documented asystole during head-up tilt testing, pacemaker implantation*** is recommended to reduce syncope recurrence [348–352].

ESC I A (CGR B, LOR 2)

  • In patients with clinical features of adenosine-sensitive syncope, implantation of a dual-chamber pacemaker*** is recommended to reduce syncope recurrence [348–352].

ESC IIbB (Level of Evidence B, Grade of Recommendation 2)

  • In patients without a verified cardioinhibitory reflex response, pacemaker implantation*** is not recommended [337–353].

ESC IIIB (Level of Evidence A, Grade of Recommendation 2)

  • Pacemaker implantation*** is not recommended in patients with unexplained syncope without confirmed sinus node dysfunction or conduction disturbance [656, 657].

ESC IIIC (Level of Evidence C, Grade of Recommendation 4)

Comments: The decision to implant a pacemaker*** should be made taking into account the clinical context of the benign nature of the condition and its frequent occurrence in young patients. Thus, pacemaker implantation is indicated in a limited number of patients selected according to strict criteria, presenting with severe reflex syncope. Older patients with a history of recurrent syncope onset in middle or older age and frequent injuries, likely due to a lack of prodromal symptoms, should be considered for pacemaker implantation. In a small number of patients, syncope recurrence is possible despite cardiac pacing.

The tilt-test response is the strongest predictor of cardiac pacing efficacy. Patients with a negative tilt-test response are expected to have a low risk of syncope recurrence, comparable to that of patients with implanted pacemakers due to idiopathic AV block. Further studies are unlikely to change this estimate of effect. Conversely, patients with a positive tilt-test response have a higher risk of syncope recurrence with a wide confidence interval, making it difficult to assess the benefit of cardiac pacing. (Appendix B, Table 4).

3.4.8. Pacing in patients with hypertrophic cardiomyopathy

In patients with symptoms caused by left ventricular outflow tract obstruction, treatment options include medical therapy, surgery, alcohol septal ablation, and sequential AV pacing with a short AV delay. Three small placebo-controlled RCTs and several observational studies with long-term follow-up have reported a reduction in left ventricular outflow tract gradient, improvement in symptoms, and enhanced quality of life with sequential atrioventricular pacing [658–663]. Myectomy has demonstrated better hemodynamic results compared to sequential dual-chamber pacing [664], but this intervention is more invasive and carries higher risk. Subgroup analysis in one study showed that older patients (> 65 years) are more likely to benefit from sequential dual-chamber pacing [660]. A recent meta-analysis including 34 studies and 1135 patients demonstrated that pacing resulted in a 35% reduction in the left ventricular outflow tract gradient and a non-significant trend toward improvement in heart failure functional class (NYHA) [665]. Shared decision-making should be employed when selecting a treatment strategy for patients with obstructive hypertrophic cardiomyopathy.

It is recommended to consider sequential AV pacing with a short AV delay in patients in sinus rhythm who have other indications for pacing or ICD, if symptoms persist despite medical therapy or the maximum left ventricular outflow tract gradient at rest or with provocation is ≥ 50 mmHg [658–662, 665, 666].

ESC IIb B (Level of Evidence B, Grade of Recommendation 2)

It is recommended to consider sequential AV pacing with a short AV delay in selected patients in sinus rhythm whose symptoms persist despite medical therapy, or whose maximum left ventricular outflow tract gradient at rest or with provocation is ≥ 50 mmHg, and who are at high risk for developing AV block during alcohol septal ablation [667, 668].

ESC IIb C (CGR C, LOR 4)

3.4.9. Pacing in rare diseases

  • In patients with neuromuscular diseases, such as myotonic dystrophy type 1 combined with any second- or third-degree AV block or HV interval ≥ 70 ms, permanent pacemaker implantation*** is recommended regardless of the presence or absence of symptoms [207-208, 451, 669].

ESC not stated (CGR B, LOR 3)

It is recommended to consider sequential AV pacing with a short AV delay in selected patients in sinus rhythm whose symptoms persist despite medical therapy, or whose maximum left ventricular outflow tract gradient at rest or with provocation is ≥ 50 mmHg, and who are at high risk for developing AV block during alcohol septal ablation [667, 668].

ESC not specified (LoE C, GoR 4)

  • In patients with neuromuscular diseases, such as myotonic dystrophy type 1 combined with a prolonged PR interval ≥ 240 ms or widened QRS duration ≥ 120 ms, permanent pacemaker implantation may be considered [208, 204, 205].

ESC IIb C (LoE B, GoR 3)

  • In patients with cardiac sarcoidosis, persistent or transient AV block, pacemaker implantation is recommended to be considered [220, 629, 673].

ESC IIa C (LoE B, GoR 3)

  • In patients with cardiac sarcoidosis, LVEF < 50%, and indications for permanent pacing, CRT device implantation is recommended [671, 672].

ESC IIa C (CARR C, LER 5)

3.5. Pacemaker implantation and perioperative patient management

  • To reduce the risk of infectious complications in patients prior to pacemaker or lead implantation or replacement, preoperative antibiotic prophylaxis is recommended no earlier than 1 h before skin incision [455].

ESC I A (LoE C, GoR 5)

  • For skin disinfection, it is recommended to use an alcoholic chlorhexidine solution** rather than an alcoholic povidone-iodine solution** [674, 675].

ESC IIa B (LoE A, GoR 2)

  • For venous access, it is recommended to consider the cephalic or axillary vein [676, 677].

ESC IIa B (LoE C, GoR 4)

  • To confirm the target position of the ventricular lead during pacemaker implantation, it is recommended to use X-ray imaging in multiple projections (chest radiography) [718, 719].

ESC IIa C (LoE A, GoR 3)

  • When implanting leads into the coronary sinus, it is recommended to prefer multi-pole leads [678-680].

ESC IIa C (LoE A, GoR 3)

  • Before wound closure, it is recommended to flush the device pocket with sodium chloride solution** [681, 682].

ESC IIa C (LoE A, GoR 1)

It is recommended to consider mid-septal pacing in patients at high risk of perforation (e.g., elderly, previous perforation, low body mass index, female) [685-686].

ESC IIb C (LoE C, GoR 4)

  • In patients with a low body mass index, Twiddler syndrome, or for aesthetic reasons, it is recommended to consider submuscular device pocket formation [685-686].

ESC IIb C (LoE C, GoR 4)

  • In patients receiving anticoagulant therapy, bridging therapy with sodium heparin** is not recommended [687-688].

ESC III A (LoE A, GoR 2)

  • Permanent pacemaker implantation is not recommended in patients with fever. Implantation should be deferred until the patient has been afebrile for at least 24 hours [455, 674, 675].

ESC III B (LoE C, GoR 5)

3.6. Selection of pacing modes

An important task when choosing a cardiac pacing system – is to prevent progression of automaticity and conduction disorders. It is necessary to choose a system that will prevent the development of pacing-induced heart failure. In this regard, it is advisable to select a pacemaker*** with greater programming capabilities than are necessary at the time of implantation. For example, a patient with SSS and paroxysmal AF may subsequently develop AVB (as a result of disease progression, medication use, or catheter ablation), and the presence of a dual-chamber pacemaker*** in such a patient necessitates a mode-switching function.

In international practice, a 5-letter nomenclature code is used, which was jointly developed by working groups of the North American Society of Pacing and Electrophysiology (NASPE) and the British Pacing and Electrophysiology Group (BPEG), known as the generic NBG-NASPE/BPEG code. As a rule, the first 3 letters are used, and the letter R (4th position) is used for rate-responsive programmable pacemakers — VVIR, DDDR (rate-modulated). The 5th letter in the NBG code relates to antitachycardia functions. In October 2001, the working groups of the North American Society of Pacing and Electrophysiology (NASPE) and the British Pacing and Electrophysiology Group (BPEG) adopted an updated 5-letter nomenclature code for antibradycardia devices (Appendix B, Table 5).

For the correct functioning of the pacing system, the physician requires practical programming skills, as well as the technical equipment of the hospital. After pacemaker implantation, the physician needs to select and program certain pacemaker parameters.

In modern single-chamber pacemakers, programmable parameters include the choice of pacing mode, lower rate limit, pulse amplitude and duration, sensitivity amplitude, and refractory period.

In dual-chamber pacemakers, in addition to these, the maximum tracking rate limit, AV delay, and mode-switching algorithm in response to atrial tachycardias are programmed. Rate-responsive pacemakers contain a program to regulate the ratio of physical activity sensitivity to pacing rate, and limitations on the maximum detectable pacing rate. With the advent of more complex cardiac pacing systems, optimal programming becomes even more complex and specific, requiring specialized knowledge from the physician (Appendix B, Table 6) [353–357].

3.7 Alternative methods of treatment for sick sinus syndrome and AV block

In some patients with pronounced parasympathetic tone activation, often combined with temporary sympathetic system suppression, vasovagal reflex syncope, vagus-induced sinus bradycardia, sinus arrest, or atrioventricular block may occur. Radiofrequency ablation of arrhythmogenic zones with a high concentration of parasympathetic ganglia leads to an increase in heart rate and a decrease in heart rate variability. This is accompanied by the absence of a heart rate response to atropine administration**.

  • In patients with documented recurrent vasovagal syncope occurring predominantly via the cardioinhibitory mechanism, it is recommended to consider the destruction of cardiac conduction pathways and arrhythmogenic zones – ganglionated plexuses (cardioneuroablation) as an alternative to pacing, provided the syncope is not accompanied by trauma [707, 708, 709].

RSC IIb B (LoE C, GoR 3)

Comment: the decision to perform cardioneuroablation must be made jointly with the patient and/or their representative after discussing potential benefits and risks, and taking into account the lack of long-term follow-up results (exceeding 24 months) in multicenter randomized trials. Screening for the causes of syncope and the advisability of catheter ablation of ganglionated plexuses must be performed in an experienced center. The patient evaluation program should include, among other things, a head-up tilt table test.

4. Medical Rehabilitation and Sanatorium-Resort Treatment

If the patient has an underlying disease, rehabilitation measures should be carried out in accordance with that underlying disease.

Patients do not require special rehabilitation after pacemaker implantation and wound healing. In individual cases, restriction of significant physical exertion is suggested for 1 month after pacemaker implantation, along with a sparing physical activity regimen and restriction of large-amplitude movements of the limb on the implantation side. Subsequently, the level of physical activity is expanded.

5. Prevention and Dispensary Observation

  • Patients with pacemakers are recommended to visit a medical organization regularly to monitor data obtained from the implantable antiarrhythmic device [455].

No ESC class (LoE C, GoR 5)

Comments: Following pacemaker implantation, careful follow-up of the patient is recommended. Prior to discharge, it is necessary to monitor the parameters obtained from the implantable antiarrhythmic device that were programmed during implantation. These parameters should be adjusted if necessary during subsequent patient visits, taking into account the results of retrieved baseline statistics, lead and implantable device parameter testing, and patient complaints. Programming of amplitude, pulse duration, and diagnostic functions affecting the pacemaker battery status must not compromise patient safety.

Automatic functions, such as automatic pacing threshold testing, are widely used in pacemaker models and facilitate the monitoring of remote patients. However, automatic functions are not universal and cannot replace individual programming and testing, especially when accompanied by history-taking and physical examination of the patient.

Clinic-based follow-up includes physical examination of the patient and monitoring of data obtained from the implanted antiarrhythmic device, selection of single- or dual-chamber pacemaker operating parameters, and programming of the single- or dual-chamber pacemaker. The schedule of clinic visits is determined at the discretion of the physicians managing these patients.

Remote monitoring of pacemaker (PM) function may be used as a patient follow-up method***, and the data transmission frequency is determined by the follow-up schedule depending on the PM type*** and the patient's clinical status [356].

Visit

Visit + remote monitoring

All implantable devices

72 hours in the clinic and 2–12 weeks post-implantation

CRT or HBP

Every 6 months

Remotely every 6 months and in-person every 12 months

Single- and dual-chamber PMs

Every 12 months, then every 3–6 months upon signs of battery depletion

Remotely every 6 months and in-clinic every 18–24 months

Guidelines for the follow-up of patients with implanted PMs***:

It is recommended to optimize PM*** function individually for each patient [353–356].

ESC IC (LoE C, SoR 5)

  • In adult patients with a PM*** after implantation, it is recommended to perform PM*** testing twice within the first six months, then annually; for patients with dual-chamber PMs*** after implantation – twice within 6 months, then at least once a year [353–356].

No ESC guideline (LoE C, GoR 5)

  • In patients implanted with a PM*** capable of wireless remote monitoring, remote heart rate monitoring is recommended as an integral part of the standard long-term follow-up strategy [353–356].

ESC IA (LoE C, SoR 5)

  • When monitoring patients with SSS and an implanted PM***, it is recommended to monitor parameters obtained from the implantable antiarrhythmic device for the early detection of AF, including subclinical AF, due to the need for timely initiation of anticoagulant therapy [353–356].

ESC IA (LoE C, SoR 5)

  • In patients with persistent SSS for whom PM implantation is not recommended at the time of diagnosis, it is recommended to avoid prescribing or to prescribe with caution medications that depress the function of the sinus node (antihypertensive drugs, beta-blockers, psychoanaleptics, etc.) [353–356].

ESC IC (LoE C, SoR 5)

  • In patients with persistent first- and second-degree AV block for whom PM implantation is not recommended at the time of diagnosis, it is recommended to avoid prescribing or to prescribe with caution medications that depress AV conduction (antihypertensive drugs, cardiac glycosides, beta-blockers, calcium channel blockers, psychoanaleptics, etc.) [355–356].

ESC IC (LoE C, SoR 5)

Comments: The frequency and method of follow-up depend on multiple factors, including various cardiovascular diseases and medical conditions, the age of the PM***, and the patient's geographical distance from the medical center. Some centers prefer using remote monitoring for general surveillance with periodic clinical data review. Other centers perform most follow-up procedures in-person at the clinic.

Remote monitoring of patients with implantable devices (remote heart rate surveillance)

Remote heart rate surveillance allows continuous monitoring of the implanted system and individual patient parameters, reducing the number of scheduled and unscheduled doctor visits by severalfold. Remote monitoring also enables prompt detection or prevention of emerging complications, which in some cases can be life-saving. The economic benefit for both healthcare and the patient by reducing the cost of treating complications is also significant. Patient-to-physician data transmission can occur in just a few minutes. A closed-loop information network "patient – service center – physician" has been created for clinics and their patients, providing a new high-tech level of patient monitoring and treatment.

  • Remote monitoring is recommended to reduce in-person clinic visits for PM*** patients who experience difficulties visiting the clinic (e.g., due to limited mobility or other circumstances, or according to patient preference) [689-681].

ESC I A (LoE A, SoR 2)

  • Remote monitoring is recommended in cases where a device has been recalled due to malfunctions or requires closer surveillance to ensure early detection of events requiring intervention, especially in high-risk patients (e.g., PM-dependent patients) [455, 689-690].

ESC I C (LoE C, GoR 5)

  • During remote monitoring in patients with single- and dual-chamber pacemakers, it is recommended to program these devices with an interval of up to 24 months [689-690].

ESC IIa A (LoE A, SoR 2)

It is recommended to use remote monitoring of pacing devices to ensure earlier detection of clinical (e.g., arrhythmias) or technical issues (e.g., lead fracture or battery depletion) [690, 810].

ESC IIa B (CGR A, LOR 2)

6. Organization of Medical Care

The type of care and the procedure for its provision depend on the type of bradyarrhythmia and concomitant pathology. Structural or functional heart disease and its progression may be the primary indication for hospitalization.

Medical care for patients with bradyarrhythmias is limited to:

- patients with bradyarrhythmias requiring examination or treatment of concomitant or underlying pathology;

- evaluation of patients with bradyarrhythmias related to medication intake;

- patients with acute bradyarrhythmias requiring emergency care;

- monitoring of patients after permanent PM implantation.

Emergency preventive measures or drug therapy must be initiated by the emergency medical team for patients with bradyarrhythmias, cardiac arrest/syncope.

The patient must be hospitalized in a medical facility capable of providing intensive care and continuous monitor monitoring. Hospitalization in a center with the capabilities and experience for temporary and permanent cardiac pacing is optimal.

Implantation of a pacemaker if indicated or, in the absence of technical capability, referral to a facility where PM implantation is performed.

Full implementation of the clinical and instrumental examination standard, justified prescription, execution, and analysis of additional diagnostic methods, including invasive ones, and establishment of a detailed clinical diagnosis corresponding to ICD-10, indicating a defined or confirmed underlying disease. The diagnosis must be established in a timely manner, both etiologically and clinically/electrocardiographically.

A set of urgent and elective measures is required to relieve an acute condition or normalize a chronic condition associated with bradyarrhythmias, optimize the underlying disease therapy, and determine indications for and perform temporary or permanent cardiac pacing.

Post-implantation, it is necessary to optimize the programmed parameters of the implanted pacemaker to personalize its function, reduce energy consumption, prevent complications, perform regular device programming, and utilize remote telemonitoring capabilities (if available).

Indications for elective hospitalization

  1. Appearance of symptoms potentially associated with bradycardia (syncope, presyncope, fatigue, dizziness, etc.).

  2. Clinical deterioration and escalation of symptoms associated with bradyarrhythmias.

  3. Elective permanent PM implantation.

  4. Elective replacement of an implanted PM.

  5. Malfunction of an implanted PM requiring surgical intervention.

  6. Infectious complications related to the pacing system.

  7. Adjustment of antiarrhythmic therapy when it is not feasible in an outpatient setting or carries a high risk of complications.

Indications for emergency hospitalization

  1. Acute-onset bradyarrhythmia requiring emergency care.

  2. Conditions requiring temporary cardiac pacing.

  3. Hemodynamic instability associated with bradyarrhythmias or PM malfunctions.

  4. Acute PM malfunction requiring surgical correction.

  5. Condition following cardiac arrest and restoration of normal cardiac activity.

Indications for hospital discharge

  1. Patients with implanted PMs who no longer require inpatient treatment.

  2. Stabilization of patients with reversible or drug-induced bradyarrhythmias.

  3. Successful adjustment of drug therapy.

7. Additional Information

7.1. Lead extraction

In certain cases of pacing system dysfunction – due to implanted leads or infectious complications – the removal of leads from the heart is considered. This can be performed transvenously or via open-heart surgery through thoracotomy.

Reasons for lead extraction include infectious causes due to systemic infection: infective endocarditis, lead-associated endocarditis, sepsis or pocket infection, venous thrombosis or stenosis, chronic pain, and lead malfunctions (dislodgement, fractures, insulation failure).

In all cases, indications for extraction and the method of its performance are discussed individually, and the issue of transvenous lead extraction (TLE) is highlighted as a separate area in arrhythmology.

Recommendations for lead extraction in infectious complications:

  • In verified systemic infection – infective endocarditis, lead-associated endocarditis, sepsis – removal of transvenous leads is recommended [357–371, 716, 717].

ESC not stated (CGR B, LOR 3)

  • In verified infection associated with the implant site of the PM system*** – pocket abscess, pressure sore, skin erosion, fistula – even in the absence of clinical evidence of intravascular portion involvement of the leads, removal of the leads is recommended [357–371, 716, 717].

ESC not stated (CGR B, LOR 3)

  • In confirmed infection of the intrathoracic part of epicardial leads, complete lead removal is recommended [357–371, 716, 717].

ESC not stated (CGR B, LOR 3)

  • In clinical manifestation (bacteremia) of latent Gram-positive infection, removal of the leads and all previously implanted transvenous leads is recommended [360–371, 716, 717].

ESC: none (LoE B, LoR 3

  • In persistent bacteremia caused by Gram-negative microorganisms, complete removal of the pacing system and all previously implanted transvenous leads is recommended [360–361].

ESC not specified (LoE C, GoR 4)

  • In superficial infection of the postoperative wound in the implantation area, removal of the pacing system and its components is recommended [360–371].

ESC not stated (CGR B, LOR 3)

  • During prolonged antibiotic therapy for chronic bacteremia in cases where another source of infection is proven, removal of the pacing system and its components is not recommended [357–371].

ESC no (Level of Evidence A, Grade of Recommendation 3)

Recommendations for lead extraction in non-infectious complications:

  • In patients with severe chronic pain in the implanted device area when its manifestations cannot be eliminated medically or surgically and there is no alternative solution to the problem, removal of the pacing system or its components is recommended [372–404].

ESC not stated (CGR B, LOR 3)

  • In patients with clinically significant manifestations of thromboembolic syndrome associated with thrombus formation on the lead or lead fragment, removal of transvenous leads is recommended [372–404].

ESC not stated (CGR B, LOR 3)

  • In patients with bilateral subclavian vein thrombosis or superior vena cava (SVC) thrombosis preventing the implantation of a required transvenous lead, removal of transvenous leads is recommended [372–404].

ESC no (Level of Evidence A, Grade of Recommendation 3)

  • When stenting is required in a vein already used for lead delivery to the heart (containing a lead), removal of transvenous leads is recommended [372–404].

ESC not stated (CGR B, LOR 3)

  • In occlusion or stenosis of the SVC with significant clinical manifestations, removal of transvenous leads is recommended [372–404].

ESC no (Level of Evidence A, Grade of Recommendation 3)

  • In the presence of four or more transvenous leads on one side, or five or more transvenous leads passed through the SVC, removal of transvenous leads is recommended [390–404].

ESC not stated (CGR B, LOR 3)

  • In patients with subclavian vein thrombosis on the side of a previously implanted device when there is a need to implant an additional transvenous lead, but contraindications to using the contralateral subclavian vein exist – arteriovenous fistula, permanent venous port, prior mastectomy, etc. – removal of transvenous leads is recommended [372–404].

ESC not specified (LoE C, GoR 4)

  • Removal of transvenous leads is recommended in patients with life-threatening arrhythmias caused by the leads themselves [372–404].

ESC: none (LoE C, LoR 4

  • Removal of transvenous leads is recommended when leads, due to their design or compromised integrity, present an immediate threat (e.g., fracture and protrusion of the stylet in J-shaped leads) [369–404].

ESC not specified (LoE C, GoR 4)

  • Removal of transvenous leads is recommended when they impede the delivery of antitumor treatment (radiation therapy, reconstructive surgery) [372–404].

ESC not specified (LoE C, GoR 4)

  • Removal of transvenous leads is recommended when patient diagnosis necessitates contrast-enhanced cardiac magnetic resonance imaging (MRI) as the only viable option [372–404].

ESC not stated (CGR B, LOR 3)

  • Removal of transvenous leads is not recommended in patients with a predicted life expectancy of less than 1 year [364–404].

ESC not specified (LoE C, GoR 4)

  • Removal of transvenous leads is not recommended in situations where abnormal lead positioning is identified (implanted via the subclavian artery, aorta, pleura, mediastinum, atrial, or ventricular wall). In cases of clinical necessity, additional techniques are used with mandatory surgical backup [372–404].

ESC: none (LoE B, LoR 3)

7.2. Cardiac Magnetic Resonance Imaging in Patients with Implanted Devices

Patients with implanted pacemakers frequently require contrast-enhanced cardiac MRI. This can potentially cause undesirable side effects, such as device malfunction due to device reset or sensing issues, current induction leading to myocardial capture, heating of the lead tip with altered sensing or pacing thresholds, or lead perforation.

Currently, most pacemaker manufacturers offer MR-conditional devices. The manufacturer typically specifies a waiting period of 6 weeks post-implantation, but MRI may be performed earlier if clinically justified.

There is sufficient evidence that MRI can be safely performed in patients with MR-non-conditional pacemakers if a number of precautions are followed [692-694]. In 2017, the Heart Rhythm Society published an expert consensus statement on MRI in patients with cardiac implantable electronic devices (CIEDs), developed and endorsed by several associations, including the European Heart Rhythm Association and multiple radiological associations [693]. Assessment of the need for MRI should be performed on a case-by-case basis, weighing the benefits of MRI against potential risks and the availability of alternative imaging modalities using shared decision-making. Typically, MRI should be performed strictly in accordance with an institutional standardized approved protocol under appropriate conditions (including CIED programming) [395, 692, 694–696].

  • In patients with MR-conditional pacemaker systems***, MRI is recommended to be safely performed in accordance with the manufacturer's instructions [395, 693, 696].

ESC I A (LoE A, LoR 2)

  • In patients with MR-non-conditional devices, MRI is recommended in the absence of alternative imaging modalities, as well as in the presence of epicardial leads, capped or damaged leads, and lead adapters/extensions [692, 694].

ESC IIa B (LoE C, LoR 4)

It is recommended to consider performing MRI in patients with pacemaker systems*** and capped transvenous leads if no alternative imaging modalities are available [697-700].

ESC IIb C (LoE C, LoR 4)

7.3. When permanent pacing is no longer indicated

Several management options are available for patients with implanted pacing systems who no longer have an indication for pacing:

  1. Leave the entire pacing system in place, including the device and leads

  2. Remove the device and leave the leads

  3. Remove the device and leads

The choice of Option 1 depends on the service life of the implanted device, which varies by manufacturer and in rare cases may lead to complications [701]. Option 1 is preferred for frail and elderly patients.

Option 2 is associated with a lower intraoperative risk, but subsequent complications from the retained lead may occur, for example, during MRI. Especially in younger patients, there may be a need to remove abandoned leads in the future due to infection and a higher risk of complications due to increased implantation procedure duration. Several studies have shown that the procedure for removing capped leads is associated with a higher risk of complications and lower efficacy [702-705].

Option 3 is associated with a high risk of complications related to the lead removal procedure, but eliminates the likelihood of device-related complications in the future. High efficacy and a low complication rate are observed during lead extraction procedures performed in specialized high-volume centers with modern equipment [704]. The latter option may be appropriate when combining factors such as young patient age, low lead extraction risk, and operator expertise.

As part of patient-centered management, decisions in such situations should be based on an individual risk-benefit ratio and made jointly with the patient and their caregivers. Providing sufficient information is essential to obtain informed consent. Factors such as the patient's age, clinical condition, comorbid conditions, pacemaker system, duration of lead implantation, and life expectancy should be taken into account.

  • When a cardiac pacemaker*** is no longer indicated, an individual risk-benefit analysis is recommended to make a joint decision with the patient. [714]

ESC I C (LoE C, GoR 5)

Criteria for Evaluating the Quality of Medical Care

Quality of care criteria for primary health care in adults with bradyarrhythmias and conduction disorders (ICD-10 codes: I44 (I44.0, I44.1, I44.2, I44.3, I44.4, I44.5, I44.6, I44.7), I45 (I45.0, I45.1, I45.2, I45.3, I45.4, I45.5, I45.8, I45.9), I46 (I46.0, I46.1, I46.9), I49.5)

No.

Quality assessment criteria

Evaluation of compliance

1.

Performed an examination including the collection of complaints and anamnesis, and a physical examination of the patient

Yes/No

2.

Performed a 12-lead electrocardiogram recording

Yes/No

3. 

Performed long-term heart rhythm monitoring using a cardiac monitor selected based on the frequency and nature of symptoms, as well as patient preference

Yes/No

4.

Performed transthoracic echocardiography in patients with bradycardia or conduction disorders (left bundle branch block and Mobitz type II second-degree atrioventricular block, high-grade atrioventricular block, or third-degree atrioventricular block) when structural heart disease is suspected, or the patient was referred to a specialized medical organization for the procedure

Yes/No

5.

Performed pacemaker function testing after implantation twice within 6 months, then at least annually; for patients with dual-chamber pacemakers after implantation, twice within 6 months, then at least annually, or the patient was referred to a specialized medical organization for pacemaker testing

Yes/No

6.

Initiated follow-up care (dispensary observation) by a cardiologist in the presence of an implanted electronic cardiac pacemaker, bradyarrhythmias, or intracardiac conduction disorders associated with a high risk of death

Yes/No

Quality of care criteria for specialized medical care in adults with bradyarrhythmias and conduction disorders (ICD-10 codes: I44 (I44.0, I44.1, I44.2, I44.3, I44.4, I44.5, I44.6, I44.7), I45 (I45.0, I45.1, I45.2, I45.3, I45.4, I45.5, I45.8, I45.9), I46 (I46.0, I46.1, I46.9), I49.5)

No.

Quality assessment criteria

Evaluation of compliance

1.

Performed an examination including the collection of complaints and anamnesis, and a physical examination of the patient

Yes/No

2.

Performed a 12-lead electrocardiogram recording

Yes/No

3.

Performed long-term heart rhythm monitoring using a cardiac monitor selected based on the frequency and nature of symptoms, as well as patient preference

Yes/No

4.

Performed transthoracic echocardiography in patients with bradycardia or conduction disorders (left bundle branch block and Mobitz type II second-degree atrioventricular block, high-grade atrioventricular block, or third-degree atrioventricular block) when structural heart disease is suspected, or the patient was referred to a specialized medical organization for the procedure

Yes/No

5.

Performed permanent pacemaker implantation providing single-chamber or dual-chamber cardiac pacing to eliminate bradycardia and its clinical symptoms when irreversible or non-physiological in nature, or the patient was referred to a specialized medical organization for implantation

Yes/No

6.

Performed pacemaker function testing after implantation twice within 6 months, then at least annually; for patients with dual-chamber pacemakers after implantation, twice within 6 months, then at least annually, or the patient was referred to a specialized medical organization for pacemaker testing

Yes/No

References

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Appendix A1. Working Group Composition

Steering Committee of the Working Group:

1. Revishvili A.Sh., Academician of the RAS, MD, Professor (Moscow), VNOA

2. Glezer M.G., MD, Professor (Moscow), RKO

3. Artyukhina E.A., MD (Moscow)

Working Group:

4. Bazaev V.A., MD (Moscow)

5. Batalov R.E., MD (Tomsk), ASSH

6. Bokeria L.A., Academician of the RAS (Moscow), ASSH

7. Davtyan K.V., MD, Professor (Moscow)

8. Lebedev D.S., MD, Professor (St. Petersburg), RKO, VNOA

9. Lebedeva V.K., MD (St. Petersburg), RKO

10. Lyubimtseva T.A., PhD (St. Petersburg), RKO, VNOA

11. Mikhailov E.N., MD (St. Petersburg), RKO

12. Popov S.V., Academician of the RAS, MD, Professor (Tomsk), VNOA, RKO

13. Romanov A.B., MD (Novosibirsk)

14. Serguladze S.Yu., MD (Moscow)

15. Sopov O.V., PhD (Moscow)

16. Filatov A.G., MD (Moscow)

The working group members confirmed no financial support/conflict of interest. In the event of a reported conflict of interest, the working group member(s) were excluded from discussions related to the area of conflict of interest.

Appendix A2. Methodology for Developing Clinical Guidelines

These guidelines were developed based on:

- Clinical Practice Guidelines for Electrophysiological Studies, Catheter Ablation, and Implantable Antiarrhythmic Devices of the All-Russian Scientific Society of Clinical Electrophysiology, Arrhythmology, and Cardiac Pacing (VNOA) (2017);

- Clinical Practice Guidelines on "Bradyarrhythmias" of the Russian Ministry of Health (2017);

- ACC/AHA/HRS Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay (2018);

- ESC Guidelines for the Diagnosis and Management of Syncope (2018);

- 2017 HRS Expert Consensus Statement on Management of Cardiovascular Implantable Electronic Devices and Lead Extraction;

- 2018 EHRA Expert Consensus Statement on Lead Extraction;

- ESC Guidelines on Cardiac Pacing and Cardiac Resynchronization Therapy (2013);

- ESC Guidelines for the Management of Infective Endocarditis (2015).

The guidelines present the basic diagnostic principles and modern approaches to the treatment of bradyarrhythmias and conduction disorders based on a generalized and systematic analysis of available clinical studies on the prevention, diagnosis, and treatment of patients with BA.

Target audience for these clinical guidelines:

  1. Cardiologist.

  2. General practitioner / Physician.

  3. General practitioner (family physician).

  4. Cardiovascular surgeon.

  5. Endovascular surgeon.

Due to the fact that the Russian Society of Cardiology (RSC) is a constituent of the European Society of Cardiology (ESC), and RSC members are also ESC members, all European guidelines are formulated with the participation of Russian experts who co-authored the guidelines. Thus, the existing ESC guidelines reflect the consensus of leading Russian and European cardiologists.

In this regard, national guidelines were developed based on ESC guidelines, taking into account national specifics, features of examination, treatment, and the availability of certain types of medical care. For this reason, during the development of Russian clinical practice guidelines by the RSC, international recommendation classes were used to assess the necessity of a guideline statement and the levels of evidence for these classes (Tables A2/1 and A2/2).

In cases where European guidelines lacked classes and levels of evidence, but in the opinion of RSC experts these statements were extremely necessary for implementation or, conversely, strictly not recommended for use, RSC experts—based on the rules for formulating recommendation classes according to the European Society of Cardiology guidelines—assigned the classes and levels themselves. In such instances, the ESC designation is replaced with RSC classes and levels assigned by RSC experts.

In addition, a new grading system for levels of evidence (LoE) and grades of recommendation (GoR) for therapeutic, rehabilitation, preventive, and diagnostic interventions (Tables A2/3, A2/4, and A2/5) introduced in 2018 by the Center for Healthcare Quality Assessment and Control of the Russian Ministry of Health was added. Thus, the text of clinical practice guidelines developed by RSC experts simultaneously utilizes two grading scales (Tables A2/1, A2/2, A2/3, A2/4, A2/5).

Table A2/1. Classes of recommendations according to the European Society of Cardiology (ESC) guidelines

ESC Class of Recommendation

Definition

Suggested Wording

I

Evidence and/or general agreement that a given diagnostic procedure, intervention/treatment is beneficial, useful, effective

Recommended / Indicated

II

IIa

IIb

Conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of the diagnostic procedure, intervention, or treatment

Weight of evidence/opinion is in favor of usefulness/efficacy

Usefulness/efficacy is less well established by evidence/opinion

Should be considered

apply

May be considered

apply

III

Evidence or general agreement that a diagnostic procedure, intervention, or treatment is not useful/effective, and in some cases may be harmful

Not recommended

Table A2/2. Levels of evidence according to the European Society of Cardiology (ESC) guidelines

ESC Level of Evidence

A

Data derived from multiple randomized clinical trials or meta-analyses

B

Data derived from a single randomized clinical trial or large non-randomized studies

C

Consensus of expert opinion and/or small studies, retrospective studies, registries

Table A2/3. Scale for assessing levels of evidence (LoE) for diagnostic methods (diagnostic interventions)

LoE

Definition

1

Systematic reviews of studies with reference standard control or systematic review of randomized clinical trials with meta-analysis

2

Individual studies with reference-method control, or individual randomized controlled trials and systematic reviews of studies of any design except randomized controlled trials incorporating meta-analysis

3

Studies without consecutive reference standard control, or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies

4

Non-comparative studies, case report

5

Only justification of mechanism of action or expert opinion is available

Table A2/4. Scale for assessing levels of evidence (LoE) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitation interventions)

LoE

Definition

1

Systematic review of RCTs incorporating meta-analysis

2

Individual RCTs and systematic reviews of studies of any design, except RCTs, with meta-analysis

3

Non-randomized comparative studies, including cohort studies

4

Non-comparative studies, case reports or case series, case-control studies

5

Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available

Table A2/5. Scale for assessing grades of recommendation (GoR) for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitation interventions)

GoR

Definition

A

Strong recommendation (all evaluated efficacy criteria (outcomes) are important, all studies are of high or moderate methodological quality, and their conclusions regarding the outcomes of interest are consistent)

B

Conditional recommendation (not all evaluated efficacy criteria (outcomes) are important, not all studies are of high or moderate methodological quality, and/or their conclusions regarding the outcomes of interest are inconsistent)

C

Weak recommendation / lack of evidence of appropriate quality (all evaluated efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions regarding the outcomes of interest are inconsistent)

Procedure for updating clinical guidelines

The guideline update mechanism provides for their systematic revision—at least once every three years, as well as upon the emergence of new data from the perspective of evidence-based medicine regarding the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or the presence of justified additions/comments to previously approved CPGs, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

Drugs registered in Russia for pharmacological testing of BA in patients with syncope.

Drug (class)

Doses and regimens

Administration

ECG parameters requiring attention

Adverse side effects

Drug interactions

Atropine**

1-2 mg (up to 0.04 mg/kg),

Intravenously

Pharmacological test in the evaluation of patients with syncope

Functional sinus bradycardia and first-degree AV block.

An increase in sinus heart rate by more than 25% from baseline or above 90 bpm indicates a vagal origin of sick sinus syndrome. Disappearance of AV conduction abnormalities indicates their vagal origin and localization of the block at the AV node level

Dry mouth, mydriasis, accommodation paresis, tachycardia, urinary hesitancy, intestinal atony, dizziness, headache

With agents possessing anticholinergic activity: enhancement of the anticholinergic effect.

With phenylephrine**: increase in blood pressure.

With organic nitrates: increase in intraocular pressure.

With procainamide**: enhancement of the anticholinergic effect.

Medication testing is not recommended for patients with impaired renal and hepatic function, the elderly, and pregnant women.

Medications registered in Russia for the acute pharmacological treatment of bronchial asthma

Drug (class)

Doses and regimens

Administration

ECG parameters requiring attention

Adverse side effects

Drug interactions

Atropine**

Initial dose: 0.5–1 mg,

Repeat administration: every 3–5 min up to a maximum dose of 3 mg,

Intravenously

Acute pharmacological treatment of sick sinus syndrome and AV block

Heart rate increase

Dry mouth, mydriasis, accommodation paresis, tachycardia, urinary hesitancy, intestinal atony, dizziness, headache

With anticholinergic agents and agents possessing anticholinergic activity: enhancement of the anticholinergic effect.

With phenylephrine**: increase in blood pressure.

With organic nitrates: increase in intraocular pressure.

With procainamide**: enhancement of the anticholinergic effect.

#Dopamine** [11]

5–20 mcg/kg/min

Starting at 5 mcg/kg/min and increasing by 5 mcg/kg/min every 2 min, intravenously

Acute pharmacological treatment of sick sinus syndrome and AV block

Heart rate increase

Headache, anxiety, agitation, hand tremor, decreased blood pressure, conduction disturbances, QRS complex widening, vasospasm, nausea, vomiting

With diuretics: the diuretic effect of #dopamine** is potentiated.

With certain drugs (furazolidone, procarbazine**): enhancement of the intensity and duration of the cardiostimulatory and pressor effects of #dopamine**.

With beta-blockers: reduction of effect.

With oxytocin**: increased vasoconstrictive effect, risk of ischemia and gangrene, as well as severe arterial hypertension up to intracranial hemorrhage.

With cardiac glycosides: possible increased risk of cardiac rhythm disorders, additive positive inotropic effect.

Administration of the drugs is not recommended in patients with impaired renal and hepatic function, the elderly, and pregnant women.

Related Documents

  1. Order of the Ministry of Health of the Russian Federation No. 918n dated November 15, 2012, "On Approval of the Procedure for Providing Medical Care to Patients with Cardiovascular Diseases."

  2. Standard of medical care for patients with atrial fibrillation and atrial flutter (in the provision of specialized care), approved by Order of the Ministry of Health of the Russian Federation No. 1622n dated December 28, 2012.

  3. Order of the Ministry of Health of the Russian Federation No. 520n dated July 15, 2016, "On Approval of Criteria for Assessing the Quality of Medical Care."

  4. Order of the Ministry of Health of the Russian Federation No. 103n dated February 28, 2019, "On Approval of the Procedure and Deadlines for the Development of Clinical Guidelines, Their Revision, the Standard Form of Clinical Guidelines, and Requirements for Their Structure, Composition, and Scientific Validity of Information Included in Clinical Guidelines" (registered on May 8, 2019, No. 54588).

Appendix B. Physician Action Algorithms

Table 1. Algorithm for assessing patient status in suspected bradycardia and conduction disturbances

Appendix B. Physician Action Algorithms — Bradyarrhythmias and Conduction Disorders

- ECG: recording of an electrocardiogram

- Extended monitoring: Holter monitoring of the cardiac rhythm

- Cardiac monitor implantation: implantation of a loop recorder for long-term recording of the electrical activity of the cardiac conduction system

Table 2. Acute pharmacological treatment of bradycardia in symptomatic sick sinus syndrome and AV block

Atropine**

Intravenously

Initial dose: 0.5–1 mg

Repeat administration every 3–5 min up to a maximum dose of 3 mg

#Dopamine** [11]

Intravenously

5–20 mcg/kg/min

Starting at 5 mcg/kg/min and increasing by 5 mcg/kg/min every 2 min

Table 3. Algorithm of actions in acute bradycardia

Appendix B. Physician Action Algorithms — Bradyarrhythmias and Conduction Disorders

Table 4. Decision-making algorithm in reflex syncope

Appendix B. Physician Action Algorithms — Bradyarrhythmias and Conduction Disorders
Table 5. NBG-NASPE/BPEG Generic Pacemaker Code*** (2001)

Appendix B. Physician Action Algorithms — Bradyarrhythmias and Conduction Disorders
Table 6. Recommendations for pacemaker model selection*** according to specific pacing indications

Appendix B. Physician Action Algorithms — Bradyarrhythmias and Conduction Disorders

Algorithm for monitoring patients with implanted pacemakers***:

  1. First pacemaker programming***: intraoperatively.

  2. Repeat pacemaker programming***: before patient discharge from the inpatient facility (earlier if necessary).

  3. 3–4 months after pacemaker implantation***, the patient must be evaluated in the programming clinic.

  4. The maintenance monitoring period for the patient in the absence of pacemaker malfunctions*** is conducted 3–4 months after implantation, with subsequent follow-up visits scheduled every 6–12 months.

  5. Period of intensive patient monitoring: upon reaching the recommended pacemaker replacement time***.

Algorithms for programming patients with implanted pacemakers***:

  • Initial pacemaker programming*** is performed by specialists directly involved in the pacemaker implantation procedure***.

  • Intraoperatively: entry of patient data, clinical indications for implantation, reference and serial numbers of implanted leads, measurement of atrial and/or ventricular pacing capture thresholds, atrial and/or ventricular intracardiac electrogram amplitudes, lead resistance (impedance), and lead polarity. Basic pacing parameters are set: modes (AAI(R), VVI(R), DDD(R), or others applicable to the specific clinical situation).

  • Basic and maximum heart rate of cardiac pacing, duration of sensed and paced AV delay, sensitivity for atrial and/or ventricular leads. Test data and initial programming parameters are entered into the surgical report. Repeat pacemaker programming*** is performed before patient discharge from the inpatient facility (earlier if necessary) by specialists of the pacemaker programming clinic***.

  • Detailed information is provided to the patient regarding the implanted device, its operation, and safety rules.

  • A completed pacemaker identification card*** must be issued to the patient.

  • The date for the follow-up visit is scheduled.

    3–4 months after pacemaker implantation***, the patient must be evaluated in the programming clinic.

  • During the visit, a 12-lead ECG is recorded, and all pacemaker parameters are checked according to the protocol.

    Period of intensive patient monitoring:

  • Upon reaching the recommended pacemaker replacement time***.

  • Pacemaker reimplantation must be performed within 3 months of this period.

  • If prompt pacemaker replacement is not possible, the evaluation is performed at least once a month to assess pacing efficacy.

  • Upon reaching the elective replacement indicator (ERI), replace the pacemaker immediately.

Pacemaker programming protocol:

  • Clinical evaluation of the patient's condition (complaints, subjective assessment of well-being, quality of life, exercise tolerance; presence of symptoms such as syncope, weakness, dyspnea, edema, dizziness, etc.). Comparison of the clinical picture dynamically with the previous visit.

  • Evaluation of diagnostic test data obtained since the previous visit.

1. Electrocardiography (to assess pacing/sensing efficacy and pacemaker malfunction***).

2. Assessment of the patient's clinical status at the time of examination and comparison with pre-implantation status (clarification of subjective well-being and complaints, exercise tolerance).

3. External inspection of the pacemaker pocket*** (condition of the skin in the pacemaker area***, exclusion of Twiddler's syndrome).

  • 4. Testing of the permanent implantable antiarrhythmic device – recording of electrograms from implanted leads, annotated electrogram or ECG (marker channel) if such functions are available in the pacemaker.

5. Assessment of lead impedances.

6. Assessment of pacing thresholds for active leads.

7. Assessment of sensing thresholds for leads.

8. Testing of the permanent implantable antiarrhythmic device – performing a test with pacemaker cessation or temporary switching to a minimum pacing rate of 30 ppm to assess the recovery time of the spontaneous rhythm, its type, stability and rate, presence of ventricular arrhythmias, symptoms, and the degree of pacemaker dependence.

9. Testing of the permanent implantable antiarrhythmic device – performing a magnet test, predicting battery depletion time, and determining the timing for pacemaker replacement***.

10. Programming of basic pacing parameters:

  • pacing mode (VVI(R), DDI(R), DDD®, etc.);

  • basic pacing rate;

  • maximum tracking/pacing rate;

  • duration of sensed and paced atrioventricular delays;

  • autocapture function;

  • lead polarity;

  • pulse amplitude, pulse width, and sensitivity threshold for the atrial and/or ventricular lead.

11. Programming of advanced pacing parameters (for DDI(R), DDD(R)):

  • activation of the "search for intrinsic conduction" algorithm;

  • activation of the "positive/negative hysteresis search" algorithm;

  • activation of the mode switch algorithm;

  • enabling the ventricular safety pacing period;

  • enabling pacemaker-mediated tachycardia prevention and termination algorithms (PVC options, PMT options).

12. Programming of the rate-adaptive function (depending on pacemaker availability; according to indications):

  • rate-responsive atrioventricular/paced-ventricular delay (rate-responsive AV/PV delay);

  • sensor activation.

13. Assessment of the stability of lead contact with the myocardium and the pacemaker housing*** (respiratory test, body position change, pressure on the pacemaker housing area***, etc.).

14. Diagnosis and resolution of pacemaker malfunctions*** (undersensing/oversensing, low/high pacing threshold, myopotential inhibition, crosstalk, far-field, pacemaker-mediated tachycardias, skeletal muscle and diaphragmatic stimulation, etc.).

  • Evaluation of the efficacy of ongoing pharmacotherapy, with its adjustment if necessary.

  • Evaluation of concomitant arrhythmias and, if necessary, determination of indications for possible surgical treatment.

  • If necessary, emergency hospitalization in an intensive care unit, recommendations for scheduled hospitalization in a cardiology department, or consultations with relevant specialists.

  • Entry of examination and pacemaker programming results into the computer database.

  • Detailed briefing of the patient and relatives regarding their medical condition, the functioning of the implanted system, changes made to programmable parameters and therapy, and behavioral guidelines.

15. Issuance of a report on the visit results, performed examinations and diagnostic tests, and programmed operating parameters.

Appendix C. Patient Information

WHAT IS BRADYCARDIA?

Bradycardia is a condition in which the heart beats too slowly. A healthy heart beats at a rate of 60 to 100 beats per minute, pumping approximately 284 liters of blood every hour. In bradycardia, the heart beats at a rate of less than 60 beats per minute. At this rate, the heart cannot pump enough oxygen-rich blood through the body during normal activities or physical exertion. As a result, you may experience dizziness, fatigue, shortness of breath, or fainting spells.

WHAT CAUSES BRADYCARDIA?

Bradycardia can develop for a variety of reasons. The most common include:

- inherited heart disease (a condition you were born with);

- certain medical conditions or the use of specific medications;

- the natural aging process;

- heart scars following a heart attack;

- sick sinus syndrome, also known as sinus node dysfunction (disorder of the natural pacemaker);

- heart block (irregularity or blockage of the electrical impulse transmission from the upper heart chamber to the lower one).

SYMPTOMS OF BRADYCARDIA

When your heart beats too slowly, you may experience various symptoms:

- dizziness or feeling faint,

- chronic fatigue,

- shortness of breath.

These symptoms will help your doctor evaluate the severity of your condition and determine the appropriate treatment.

DIAGNOSIS OF BRADYCARDIA

Only a physician can diagnose bradycardia and its severity. To rule out or confirm a diagnosis of bradycardia, certain tests may be required depending on the suspected heart rhythm disorder. These include:

- electrocardiogram (ECG),

- exercise ECG test (measures heart rate during physical exercise),

- Holter or ambulatory monitoring,

- external loop recorder,

- implantable cardiac monitor.

- passive orthostatic tilt test,

- electrophysiological study.

TREATMENT OF BRADYCARDIA

Treatment of bradycardia depends on its underlying cause. Bradycardia may be caused by thyroid hormone deficiency (hypothyroidism), electrolyte imbalance, or medications taken for certain conditions. Replacing these medications with alternatives or adjusting the dose can restore a normal heartbeat. If drug therapy is ineffective or the slow heartbeat is caused by damage to the heart's electrical system, pacemaker insertion or implantation may be indicated.

WHAT IS A PACEMAKER?

When referring to a traditional pacemaker, it generally means a pacing system consisting of a pulse generator and leads. A pacemaker is a small device implanted subcutaneously, typically in the subclavian region. This device helps regulate irregular, intermittent, or slow heartbeats. Leads are thin, flexible, insulated wires with a diameter of about 2 mm. Leads transmit electrical impulses from the pacemaker to the heart and relay information about the heart's natural activity back to the pacemaker.

HOW DOES A PACEMAKER WORK?

A pacemaker is designed as an artificial counterpart to the natural pacemaker – the sinus node. A pacemaker has two primary functions: pacing and sensing.

PACING: The pacemaker delivers an electrical impulse to the heart through a lead when the heart's intrinsic rhythm is slowed or interrupted. The electrical impulse stimulates a heartbeat.

SENSING: The pacemaker also "senses" (monitors) the heart's intrinsic electrical activity. When the pacemaker detects a normal heart rhythm, it does not send out a pacing stimulus.

IMPLANTATION OF A TRADITIONAL PACEMAKER

Pacemaker implantation does not require open-heart surgery, and most patients are discharged home within 24 hours. Prior to the surgical procedure, you will be given a medication with sedative and hypnotic properties. Typically, the procedure is performed under local anesthesia.

STANDARD STEPS OF PACEMAKER IMPLANTATION:

A small incision, approximately 5-10 cm long, is made in the upper chest just below the collarbone. One or two leads are guided through a vein to the heart and connected to the pacemaker. The pacemaker settings are programmed, the device is tested to ensure proper function, the pacemaker is placed subcutaneously, and the incision is closed with sutures.

FOLLOW-UP CARE AND MONITORING

Follow-up appointments allow for checking the pacemaker's function. During a check-up, the physician may:

- check the pacemaker battery status;

- test the leads to determine their interaction with the pacemaker and the heart;

- check the pacemaker settings to ensure they meet your needs,

- adjust the pacemaker settings outside of regular in-person clinic visits,

Your clinic may provide remote pacemaker monitoring. Remote monitoring can replace some (but not all) in-person doctor visits. Your doctor may still need to perform a physical examination or adjust pacemaker settings or drug therapy.

IF THE SYSTEM IS EQUIPPED WITH REMOTE MONITORING

The system allows you to transmit information stored in the implanted pacemaker to the clinic, according to your doctor's instructions, using a cellular-enabled patient monitor or via your smartphone. The data from your pacemaker is then sent to a secure website where your clinic can access it to review your heart's status and pacemaker function. The system provides your doctor with the same pacemaker data that can be obtained during an in-office visit. Discuss optimal treatment options for your condition with your doctor.

INDICATIONS FOR URGENT MEDICAL CONSULTATION

  1. Recurrence of symptoms, including dizziness, presyncope, or fainting spells.

  2. Appearance of ineffective pacing on an ECG.

  3. Pacing of the shoulder girdle muscles or diaphragm.

  4. Trauma to the pacemaker site (blow, fall).

  5. Before and after undergoing medical procedures that may affect pacemaker function.

  6. Electrical injury.

  7. External skin changes (redness, cyanosis, thinning of the skin over the device), pain, or discharge in the area of the suture or pacemaker pocket.

FREQUENTLY ASKED QUESTIONS

CAN I USE A MOBILE PHONE?

Yes. When talking on a mobile phone, keep the phone antenna at least 16 cm away from the pacemaker and hold the phone to the ear on the opposite side of the body from the pacemaker. We also recommend not placing your mobile phone in a pocket directly over the pacemaker.

IS IT SAFE TO USE HOUSEHOLD APPLIANCES?

Yes. Household appliances are generally safe to use if properly maintained and in good working order. This includes microwave ovens, major household appliances, electric blankets, and heating pads.

DO MAGNETS AFFECT MY DEVICE?

Items containing magnets, including magnetic therapy products, stereo speakers, and hand-held massagers, can temporarily affect pacemaker operation. Therefore, it is recommended to keep items containing magnets at least 16 cm away from the implanted pacemaker. We do not recommend using magnetic mattresses and pillows, as it is difficult to maintain the required 16 cm distance from the device.

WILL I BE ABLE TO TRAVEL?

Given the brief duration of screening, it is unlikely that metal detectors (walk-through arches and handheld detectors) as well as body scanners (millimeter-wave and 3D scanners) found in airports, courthouses, and prisons will affect your pacemaker. A metal detector may react to the metal casing of your pacemaker. To minimize the risk of temporary interaction with the pacemaker during screening, do not touch the metal surfaces of the equipment. Do not stop or linger in the archway; simply walk through at a normal pace. If a handheld detector is used, ask the security operator not to hold it over the pacemaker area or wave it close to the pacemaker. Alternatively, you may be asked to undergo a manual pat-down search. If you have concerns about screening procedures, show your pacemaker identification card, request alternative screening, and follow the staff's instructions.

WILL I BE ABLE TO EXERCISE? WILL I BE ABLE TO PERFORM DAILY ACTIVITIES?

You will be able to return to your normal activities as long as they do not exceed your current physical exertion levels.

LIVING WITH A PACEMAKER

Many people with a pacemaker resume their normal daily activities after recovering from the implantation procedure. Your doctor may ask you to avoid certain situations. Discuss your activity level and lifestyle with your doctor to develop a plan that is optimal for you.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

Not used.

Scales and calculators from this guideline

Dopamine calculatorCalculation of dopamine infusion rate (mcg/kg/min → ml/h) with automaticNYHA functional classClassification of chronic heart failure severityNorepinephrine calculatorCalculation of norepinephrine infusion rate in ml/h based on dose in mcg/kg/min, weightVAS for Pain (Visual Analog Scale)Assessment of pain intensity using a 10-point visual analog scale

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