Takotsubo cardiomyopathy with typical ECG changes. Wagdy K, Glob Cardiol Sci Pract (2015). Open

Definition

Takotsubo cardiomyopathy causes chest pain and heart failure that often occur simultaneously with other critically serious illness, including critical systemic lupus (SLE), serious phases of other systemic connective tissue disorders or Vasculitis (Lin W, 2021). Takotsubo cardiomyopathy also occurs after severe emotional stress ("broken heart"). Ultrasound of the heart (Echo Doppler) shows impaired function of left ventricle. ECG abnormalities can be similar to Anterior heart attack. Blood tests show increases in heart attack markers in some cases. Coronary angiography survey (of coronary arteries) reveals normal results

Disease Cause

Stress hormones like catecholamines are significantly elevated such as at pheochromocytoma which can cause similar heart disease. SLE or other serious disease may trigger Takotsubo cardiomyopathy, but a definitive clarification of the cause of the disease is not available.

Symptoms

• Acute pain behind the breastbone such as by heart attack is most common (more than 80%)
• Some are debuting with heavy breathing (about 50%).
• Fainting and shock are rare (2%)
• Within a few days, there may be signs of heart failure (approximately in 40%), irregular heartbeat, heavy breathing and pulmonary edema (approximately 10%)

Occurrence

Takotsubo cardiomyopathy occurs among 3,3 per 100.000 inhabitants annually (incidence) and amounts to approx. 2% of cases of suspected acute coronary heart disease. The disease is most common in women (90%) and over 80% are over 60 years old (reference: Gunnes P, 2019)

Diagnosis

• Echocardiography shows transient reduced mobility (hypokinesia) of the left heart ventricle. The affected areas often include more than one single coronary artery provides
• Coronary angiography without stenoses or signs of plaque rupture
• ECG shows new changes, often as in the front wall infarct (ST swelling, T-wave inversion or long QT interval)
• Myocardial Enzymes (troponins) are normal or moderately elevated

More about diagnosis here (in Danish): (reference: Scantlebury DC, 2014)

Incorrect diagnosis? (Similar diseases / differential diagnoses)

• Heart / Coronary Disease for another reason
  • Acute coronary syndrome
  • Angina pectoris
  • Aortic dissection
  • Kardial tamponade
  • Cardiomyopathy
    • With dilation
    • With hypertrophy
• Pulmonary embolism
• pneumothorax
• Pheochromocytoma
• Myocarditis

Treatment

No good studies have been done that test various drugs systematically. ACE inhibititor if not LVOT gradient. If heart failure, diuretics are appropriate (not for LVOT gradient).

Disease and medical prognosis

• Cardiogenic, hypotensive shock may occur (echocardiography to rule out LVOT obstruction).
• Normalization of ventricular function after 1-4 weeks
• Hospital mortality (mortality) is approx. 2%

Literature

• Lin W, 2021
• Wang Y, 2015
• Gunnes P, 2019 (Norwegian study)
• Wikipedia (English)

• Bindevevssykdommer.no
• Vaskulitt.no