Broken heart syndrome

Broken heart syndrome

• This is a rare heart disease characterized by acute heart failure triggered by emotional or physical stress. 
• Cardiac wall movement abnormalities resolve within a few months. 
• Symptoms resemble those of acute coronary syndrome (ACS).

Synonyms

• Ampullary cardiomyopathy
• Bulge cardiomyopathy
• Stress-induced cardiomyopathy
• Takotsubo cardiomyopathy
• Apical swelling syndrome
• Transient apical bulge syndrome of the left ventricle
• Tako-Tsubo syndrome
• Takotsubo syndrome

Incidence

How common is Broken heart syndrome?

• Although the frequency is likely understated, 1-3% of patients with ACS symptoms are diagnosed with Takotsubo syndrome (TTS).
• Since more people are becoming aware of the condition, Broken heart syndrome is now being identified in more male and younger patients, however 90% of patients are female and typically experience the disease during menopause.

Age of Onset

Any age

What are the symptoms of Broken heart syndrome?

• In the acute phase, the clinical picture, electrocardiogram (ECG) and cardiac biomarkers are similar to those of ACS. 
• Acute chest pain and dyspnea are the characteristic clinical signs, while other symptoms may arise as complications of TTS (heart failure, cardiogenic shock or cardiac arrest). 
• Patients may also have other ECG changes or elevated levels of cardiac biomarkers.

Very Common Symptoms

• Mildly reduced ejection fraction

Common Symptoms

• Chest pain
• Increased circulating troponin T concentration
• ST segment elevation
• T-wave inversion

Occasional Symptoms

• Abnormal B-type natriuretic peptide level
• Angina pectoris
• Decreased QRS voltage
• Dilatation of the ventricular cavity
• Dyspnea
• Hypertension
• Hypotension
• Low-output congestive heart failure
• Mildly elevated creatine kinase
• Obesity
• Palpitations
• Prolonged QT interval
• Prolonged QTc interval 
• ST segment depression
• Vomiting

Rare Symptoms

• Arrhythmia
• Atrial fibrillation
• Bradycardia
• Cardiogenic shock
• Mitral regurgitation
• Pulmonary edema
• Seizure
• Syncope
• Thromboembolic stroke
• Ventricular arrhythmia
• Ventricular fibrillation

What are the causes of this condition?

• The etiology is unknown. Recent research has highlighted the presence of specific alterations in the neurological response and sympathetic activation following emotional stimuli, confirming the importance of brain-heart interaction in this process. 
• Constriction of the microcirculation has also been hypothesized as a cause of the disease.

How to diagnose this condition?

• Diagnosis is often made difficult by the similarity of TTS to SCA. Coronary angiography with left ventriculography is considered the gold standard for diagnosis. 
• In order to facilitate the diagnosis of the disease, the InterTAK (International Takotsubo Criteria) diagnostic criteria have been developed, which include: 1) transient dysfunction of the left ventricle which manifests itself in the form of wall movement anomalies at the apical, mid- to ventricular, basal or focal, with possible involvement of the right ventricle; 2) the presence of a triggering factor of an emotional, physical or combined nature (non-essential criterion); 3) neurological disorders may represent triggering factors; 4) the presence of other ECG abnormalities (ST segment elevation or depression, T wave inversion, or QTc prolongation); 5) elevated levels of cardiac markers (in particular brain natriuretic peptide); 6) any significant concomitant coronary artery disease; 7) the absence of myocarditis.

What is the differential diagnosis?

• The differential diagnosis includes
• acute myocardial infarction (AMI)
• myocarditis
• spontaneous coronary artery dissection
• peripartum cardiomyopathy

How is this treated?

• Due to the difficulty in differentiating TTS from ACS, it is recommended that patients be transferred to a chest pain unit to receive guideline treatment for ACS. 
• There are no randomized clinical trials available that can provide recommendations for the specific treatment of TTS in the acute and long-term phase. 
• However, the shared statement of an international expert group for TTS provides some recommendations for the optimal treatment of patients. Electrocardiographic monitoring is critical because a prolonged QT interval could trigger malignant ventricular arrhythmias (torsades de pointes) or atrioventricular block. 
• Cardiogenic shock or post-cardiac arrest syndrome requires intensive care and treatment based on the possible presence of pulmonary edema, low left ventricular ejection fraction, hypotension, and bradycardia. In any case, it is recommended to avoid inotropic active ingredients. 
• For long-term therapy, angiotensin-converting enzyme inhibitors (ACEi) and angiotensin II receptor blockers (ARBs) are indicated and are associated with improved survival and lower recurrence rates. 
• In the acute phase, echocardiography is recommended to exclude right ventricular involvement, left ventricular outflow tract obstruction, and intraventricular thrombosis. Follow-up echocardiography after discharge is also recommended to confirm recovery.

What is the prognosis of Broken heart syndrome?

• The clinical picture of TTS is broad and includes low-risk forms up to very high-risk forms. 
• Hospital mortality and long-term outcomes of TTS are similar to those of ACS. 
• Regional left ventricular systolic dysfunction usually resolves within 4 to 8 weeks. The risk of recurrence is approximately 5%.