Snapshot A 17-year-old boy suddenly collapsed and died on the soccer field shortly after scoring the winning goal. He had no known past medical history. Introduction Form of cardiomyopathy that may cause cause death both acutely and chronically young athlete: sudden cardiac death most commonly due to arrythmia (ventricular tachyarrythmias) can also be due to outflow tract obstruction while most common in young athletes can present at any age chronic: progressive heart failure most commonly takes form of diastolic filling defects can also be due to systolic dysfunction Separated into obstructive and nonobstructive subtypes obstructive disease - about 70% of patients characterized by >30 mmHg gradient in the left ventricular outflow tract non-obstructive - smaller gradient across outflow tract Genetics 50% of cases are are familial (inherited) inherited as autosomal dominant trait due to mutations in myocyte contractile elements Presentation Symptoms like other cardiomypathies, may present with acute sudden symptoms slow, chronically-progressing symptoms symptoms include chest pain dizziness syncope lightheadedness dyspnea Physical exam typical signs of CHF obstructive type associated with preload dependent maneuvers increased murmur intensity with valsalva decreased murmur intensity with hand grip decreased murmur with squatting (due to increased preload and afterload) due to changing gradient across outflow tract Evaluation Echocardiography diagnostic gold standard may show asymmetrically thickened LV walls thickening of the interventricular septum banana-like LV chamber may show normal EF Differential Other cardiomyopathies, and other causes of CHF Treatment Medical medications to improve cardiac function medications include beta-blockers CCBs disopyramide avoid certain medications including inotropes (i.e. digoxin) vasodilators overdiuresis Prognosis, Prevention, and Complications Prognosis excellent in some people who remain asymptomatic for life poor in others, resulting in sudden death Prevention screen close relatives treat known hypertension to reduce risk of sudden death Complications dilated cardiomyopathy CHF arrhythmias syncope-related injury