26 year old man died while playing. His autopsy of the heart revealed myocyte hypertrophy. Diagnosis is –
**Core Concept**
Myocyte hypertrophy is a hallmark of hypertrophic cardiomyopathy (HCM), characterized by an abnormal thickening of the heart muscle (myocardium) leading to impaired diastolic relaxation and increased risk of sudden cardiac death, particularly in young individuals engaged in strenuous physical activity.
**Why the Correct Answer is Right**
Hypertrophic obstructive cardiomyopathy (HOCM) is a specific type of HCM where the hypertrophied septum obstructs the left ventricular outflow tract (LVOT), leading to a dynamic obstruction that worsens with exercise. This obstruction can cause sudden death due to arrhythmias, such as ventricular fibrillation, triggered by the mechanical stress on the heart during physical exertion. The autopsy findings of myocyte hypertrophy in a young individual with a history of sudden death during exercise are highly suggestive of HOCM.
**Why Each Wrong Option is Incorrect**
**Option B:** Dilated cardiomyopathy (DCM) is characterized by a dilatation and thinning of the heart muscle, which is opposite to the myocyte hypertrophy seen in HOCM. DCM typically presents with symptoms of heart failure and is not typically associated with sudden death during exercise.
**Option C:** Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a condition characterized by fibrofatty replacement of the myocardium, leading to arrhythmias and sudden death. However, ARVC typically presents with right ventricular involvement and is not associated with myocyte hypertrophy.
**Option D:** Restrictive cardiomyopathy is characterized by a stiff heart muscle that impairs diastolic relaxation, leading to symptoms of heart failure. However, it is not typically associated with myocyte hypertrophy or sudden death during exercise.
**Clinical Pearl / High-Yield Fact**
HOCM is a leading cause of sudden cardiac death in young athletes, and a history of unexplained syncope or near-syncope during exercise should raise suspicion for this condition.
β Correct Answer: A. HOCM. Hypertrophic obstructive cardiomyopathy.