What is Takotsubo (stress) cardiomyopathy?

What is Takotsubo Cardiomyopathy?

Takotsubo cardiomyopathy is a reversible stress-induced cardiac syndrome characterized by transient left ventricular dysfunction that mimics acute myocardial infarction but occurs in the absence of obstructive coronary artery disease. 1

Core Defining Features

The syndrome is distinguished by three essential characteristics that separate it from other cardiac conditions:

• Reversible myocardial dysfunction is the hallmark, with the left ventricle developing acute dysfunction that completely resolves over days to weeks (typically 1-4 weeks) 1
• Absence of obstructive coronary disease on coronary angiography, with no significant stenosis despite presentation mimicking acute coronary syndrome 1
• Stress-triggered pathophysiology following emotional or physical stressors, precipitating a catecholamine surge that causes myocardial dysfunction 1

Clinical Presentation

Patients present identically to acute myocardial infarction, making initial differentiation impossible without imaging:

• Acute chest pain or dyspnea as the primary symptom 2, 3
• ST-segment elevation or T-wave inversion on ECG, most often in precordial leads 2, 4
• Elevated cardiac troponin levels that are modest and disproportionately low relative to the extensive wall motion abnormalities seen 1, 2
• Some patients present with hypotension or cardiogenic shock rather than chest pain 4

Characteristic Morphological Patterns

The classic appearance involves specific regional wall motion abnormalities:

• Apical ballooning pattern (most common): left ventricular apical akinesia with basal hyperkinesis, creating the characteristic "takotsubo" (Japanese octopus trap) appearance 1, 2
• Variant forms include mid-ventricular involvement with sparing of apex and base, basal involvement (inverse takotsubo) with apical hyperkinesis, and biventricular involvement 5, 1
• Wall motion abnormalities typically involve all three major coronary artery territories despite absence of coronary stenosis 4

Pathophysiological Mechanism

The underlying mechanism centers on catecholamine toxicity:

• Catecholamine surge is the primary mechanism, with supraphysiological elevations of plasma catecholamines documented during acute episodes 5
• β2-adrenergic receptor signaling switches from Gs to Gi protein signaling with high circulating epinephrine levels, causing negative inotropy while protecting against apoptosis 5
• Regional differences in adrenergic receptor density explain apical involvement—the ventricular apex has increased β2-adrenergic receptor density despite sparse sympathetic innervation 5
• Contributing factors include microvascular vasospasm, impaired fatty acid metabolism, and base-to-apex perfusion gradient differences 5

Epidemiology and Risk Factors

The syndrome has a striking demographic pattern:

• Female predominance with approximately 90% of cases occurring in women 5
• Postmenopausal status is the strongest risk factor, with 96% of cases in women ≥50 years of age 5
• Mean age of 66.8 years 5
• Pre-existing psychiatric disorders (anxiety, depression) may increase susceptibility 5
• Accounts for 2% of myocardial infarction with nonobstructive coronary arteries (MINOCA) 6

Triggering Events

Both emotional and physical stressors can precipitate the syndrome:

• Emotional stressors: major depressive episodes, mourning, profound psychological stress 5, 7
• Physical stressors: acute medical illness (neurological conditions, respiratory failure, sepsis), surgical procedures, chemotherapy 5
• Endocrine disorders (thyroid dysfunction) can alter cardiovascular response to catecholamines 5

Acute Complications

Despite reversibility, serious complications occur during the acute phase:

• Heart failure and cardiogenic shock (27% in one series) are the most serious complications and leading causes of death 7
• Life-threatening ventricular arrhythmias due to QT-interval prolongation 6, 8
• Left ventricular thrombus formation, particularly with severe LV dysfunction and extended apical ballooning 9
• Transient left ventricular outflow tract obstruction 6, 5
• Ventricular rupture (rare) 6
• Transient right ventricular dysfunction, which is associated with more complications, longer hospitalization, and worse left ventricular systolic dysfunction 2, 4

Diagnostic Confirmation

Diagnosis requires specific criteria and complete recovery:

• Diagnosis is made based on Mayo Clinic or InterTAK diagnostic criteria 6
• Complete functional recovery is required to confirm the diagnosis, with left ventricular function normalizing typically within 1-4 weeks 1
• Serial echocardiography is essential to document improvement in cardiac function 4
• Cardiac MRI is increasingly used to differentiate from acute coronary syndrome in patients with abnormal coronary arteries 2

Prognosis

The long-term outlook is generally favorable but not without risk:

• In-hospital mortality occurs at similar levels to acute coronary syndrome, significantly affected by underlying diseases 8
• Most deaths occur in the first 10 days of hospitalization due to cardiogenic shock 7
• Recurrence rate is approximately 8% 7
• Long-term prognosis after recovery is overwhelmingly favorable with supportive therapy 3