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Assessing a murmur, saving a life: current trends in the management of hypertrophic cardiomyopathy
Insights
Hypertrophic cardiomyopathy (HCM) is a genetic heart condition causing sudden cardiac death in young individuals. Diagnosis involves echocardiograms, with treatment ranging from medication to surgery for symptomatic patients.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM), previously known as idiopathic hypertrophic subaortic stenosis (IHSS), is a primary cause of sudden cardiac death in young populations.
- This non-dilated cardiomyopathy affects the left ventricle, left atria, intraventricular septum, and mitral valve, stemming from an autosomal dominant genetic disorder.
- HCM impairs both diastolic and systolic functions, presenting diagnostic challenges due to disease heterogeneity and variable clinical signs.
Abstract:
Hypertrophic cardiomyopathy, formerly called idiopathic hypertrophic subaortic stenosis (IHSS), is the leading cause of sudden cardiac death in young people. Hypertrophic cardiomyopathy is a non-dilated cardiomyopathy primarily affecting the left ventricle, left atria, intraventricular septum, and mitral valve. It is an autosomal dominant genetic disorder that impairs diastolic and systolic function. Diagnosis is complex due to the heterogeneity of the disease. Symptoms and morphology are not always related and clinical signs may be absent or limited to a soft systolic murmur. The first symptom of hypertrophic cardiomyopathy is frequently sudden cardiac death. Echocardiogram is an accurate diagnostic tool. Asymptomatic patients are generally not treated. Treatment for symptomatic patients begins with beta or calcium channel blockers. Antiarrhythmics may be added to protect against sudden cardiac death. Surgical intervention is done if other treatments fall and involves removal of a portion of the obstructive septum. Operative mortality is 5% with a 60% reduction in symptoms. A promising alternative to surgery is dual-chamber pacemakers. Patient and family teaching is the emphasis of long-term management.