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Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT) obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology and pathophysiology of HCM includes hypertrophy of the left ventricle with or without right ventricular hypertrophy, systolic anterior motion of mitral valve, dynamic and mechanical LVOT obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and fibrosis. Thorough understanding of pathology and pathophysiology is important for anesthetic and surgical management.

The Warden technique is a simple and effective surgical option, which should be preferred for patients with anomalous drainage of the pulmonary veins to SVC. Though arrhythmias are rare in the early follow-up, longer follow-up is required to rule out their late development.

Acute mitral regurgitation and cardiac tamponade were the causes of emergency surgery after balloon valvotomy. Transthoracic echocardiography underestimated the severity of valve pathology.

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