Makindo Medical Notes

Related Subjects: |Dilated Cardiomyopathy |Hypertrophic cardiomyopathy (HCM - HOCM) |Peripartum cardiomyopathy |Restrictive Cardiomyopathy |Takotsubo Cardiomyopathy

Often triggered by severe emotional or physical stress, hence the term "Broken Heart Syndrome." Patients typically present with chest pain, shortness of breath, and symptoms similar to acute coronary syndrome (ACS). Unlike typical MI, it is reversible, with most patients recovering normal heart function within days to weeks. Early recognition and management are crucial to prevent complications.

About

• This is a retrospective diagnosis—initial management should follow Acute Coronary Syndrome protocols due to similar presentations.
• A stress-related, reversible cardiomyopathy characterized by "apical ballooning of the left ventricle (LV)," giving it the name "Takotsubo," after the Japanese octopus trap with a similar shape.
• First described in 1990 in Japan, it mimics ACS but lacks significant coronary artery obstruction.
• Triggered by intense emotional or physical stress—often called "Broken heart syndrome" or "Stress cardiomyopathy."
• Most commonly affects post-menopausal women (90% are female), particularly those of Asian or Caucasian descent.

Aetiology

• The exact cause remains unclear, but potential mechanisms include catecholamine surge, microvascular dysfunction, and coronary artery spasm.
• May be provoked by intense stress, such as grief, severe illness, financial loss, or physical strain (e.g., surgery, exercise).
• Associated with increased levels of catecholamines (adrenaline and noradrenaline) that can be toxic to the myocardium.
• There is typically no evidence of coronary obstruction on angiography.

Clinical Features

• Presentation is similar to myocardial infarction (MI) with acute chest pain and dyspnoea (shortness of breath).
• May result in cardiogenic shock, heart failure, and pulmonary oedema in severe cases.
• Potential complications include dysrhythmias (e.g., atrial fibrillation, ventricular tachycardia), left ventricular thrombus formation, and rarely, left ventricular free wall rupture.
• Emotional triggers are common, but physical triggers such as severe infections, trauma, or intense exercise can also precipitate symptoms.

Differential Diagnosis

• Important to exclude other causes of paroxysmal symptoms, such as pheochromocytoma, which can present similarly.
• Other differential diagnoses include acute coronary syndrome (ACS), myocarditis, and acute heart failure.

Investigations

• ECG: May show ST-segment elevation, T-wave inversion, QT prolongation, or nonspecific changes, mimicking STEMI.
• Echocardiography: Reveals apical akinesis with a characteristic ballooning of the apex while the basal segments remain hypercontractile, creating the classic "takotsubo" appearance.
• Troponin levels: Mild to moderately elevated, often not as high as in typical STEMI.
• BNP: Often significantly elevated due to heart stress.
• Coronary Angiogram: Shows normal or non-obstructive coronary arteries, helping to differentiate from ACS.
• Cardiac MRI: Useful for confirming diagnosis, showing absence of delayed gadolinium hyperenhancement, indicating no significant myocardial fibrosis or scarring.
• Assess for elevated catecholamine levels (often higher than in STEMI).

Management

• Acutely, treat as acute coronary syndrome until Takotsubo is confirmed. Administer aspirin, beta-blockers, and ACE inhibitors as per ACS protocol.
• Manage heart failure symptoms with diuretics if required, and consider anticoagulation if there is evidence of LV thrombus or severe LV dysfunction.
• Beta-blockers (e.g., metoprolol) can be beneficial to reduce catecholamine impact, especially in cases of LV outflow tract obstruction.
• ACE inhibitors or ARBs may help improve heart function during the recovery phase.
• Patients usually recover within 1-4 weeks, with normalization of LV function in most cases.
• Follow up with repeat echocardiography to ensure resolution of LV dysfunction and to monitor for recurrence.

Prognosis

• Prognosis is generally good with complete recovery in most patients, but there is a 10% recurrence risk and potential for long-term complications like heart failure.
• Complications such as cardiogenic shock or ventricular arrhythmias can be life-threatening if not treated promptly.
• Ongoing psychological support may be beneficial due to the association with emotional triggers.

References

• Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review
• The clinical features of takotsubo cardiomyopathy
• ACC: Management of Takotsubo Syndrome