Takotsubo syndrome

Classification and terminology

This nosology belongs to the group of unclassified cardiomyopathies (Unclassified Cardiomyopathy). According to the Fourth Universal Definition of Myocardial Infarction, the disease is considered one of the cardiac causes of myocardial injury (necrosis).

In clinical practice, the synonym transient left ventricular apical ballooning syndrome (transient left ventricular apical ballooning syndrome) is also widely used.

Clinical presentation

The disease manifestation largely mimics the development of acute coronary syndrome (ACS) and presents with typical chest pain.

Paroxysmal symptoms and signs include:

• Severe headache.
• Diaphoresis.
• Palpitations and tachycardia.
• Significant blood pressure variability.

Significantly less frequently, patients may experience skin pallor, tremor, nausea, as well as panic attacks or unmotivated anxiety. Manifestations of cardiovascular disease may include myocardial infarction, various rhythm disorders (arrhythmias), and Takotsubo cardiomyopathy itself.

Diagnostic workup

Since the clinical picture of Takotsubo syndrome is indistinguishable from ACS, the key method of differential diagnosis is invasive coronary angiography. In stress cardiomyopathy, angiography results will be negative—there are no hemodynamically significant coronary artery stenoses.

It is important to distinguish Takotsubo syndrome from other causes of myocardial necrosis. Cardiac causes of myocardial injury also include:

• Heart failure and myocarditis.
• Cardiomyopathies of any etiology.
• Myocardial revascularization procedures and other cardiac surgical interventions.
• Catheter ablation, defibrillation (electrical cardioversion).
• Cardiac contusion due to trauma.

Non-cardiac causes include severe systemic conditions: sepsis, infections, chronic kidney disease (CKD), stroke, subarachnoid hemorrhage, pulmonary embolism, pulmonary hypertension, infiltrative diseases (sarcoidosis, amyloidosis), chemotherapy toxicity, heavy physical exertion, and critical states.

Other unclassified cardiomyopathies

Along with Takotsubo syndrome, left ventricular non-compaction cardiomyopathy (LVNC) is included in the group of unclassified cardiomyopathies.

It is characterized by the presence of deep intertrabecular recesses and prominent trabeculations (most commonly in the mid-apical segments of the lateral wall and interventricular septum). Segments are formed where a thin epicardium is combined with a thick endocardium. The disease is often familial, and can be isolated or combined with muscular dystrophies and congenital malformations (e.g., Ebstein's anomaly).

Methods used to diagnose LVNC include:

1. Echocardiography: the baseline method (especially in pediatrics), detecting increased trabeculation.
2. MRI: used in complex cases. The diagnostic criterion (cut-off) is a non-compaction-to-compaction ratio greater than 2.3. Native T1 mapping allows for the detection of fibrosis in early stages (even without late gadolinium enhancement), while late gadolinium enhancement (LGE) detects fibrosis in the compacted layer.

Class and exam questions

What else to study on this topic

• Diagnostic criteria for left ventricular non-compaction cardiomyopathy (LVNC) by MRI and echocardiography
• Differential diagnosis of cardiac and non-cardiac causes of myocardial necrosis
• Fourth Universal Definition of Myocardial Infarction
• Arrhythmogenic right ventricular cardiomyopathy (ARVC): MRI findings and right ventricular dilation

Prepared by WhiteCoat Pro faculty in accordance with the medical school curriculum. For educational purposes only.