In this autopsy series, HCM represented a common cause of sudden death in young and previously undiagnosed African American male athletes, in sharp contrast with the infrequent clinical identification of HCM in a hospital-based population (i.e., by seven-fold). This discrepancy suggests that many HCM cases go unrecognized in the African American community, underscoring the need for enhanced clinical recognition of HCM to create the opportunity for preventive measures to be employed in high-risk patients with this complex disease.

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A strong association exists between degree of SICAD and myocardial scarring seen on CMR.

The study population is part of an ongoing institutional review boardapproved registry of HCM patients at a single tertiary care center (Cleveland Clinic, Ohio), with the initial visit between January 1997Background-We report the predictors of long-term outcomes of symptomatic hypertrophic cardiomyopathy patients undergoing surgical relief of left ventricular outflow tract obstruction. Methods and Results-We studied 699 consecutive patients who have hypertrophic cardiomyopathy with severe symptomatic left ventricular outflow tract obstruction (47±11 years, 63% male) intractable to maximal medical therapy, who were referred to a tertiary hospital between January 1997 and December 2007 for the surgical relief of left ventricular outflow tract obstruction. We excluded patients <18 years of age, those with an ejection fraction <50%, those with hypertensive heart disease of the elderly, and those with more than mild aortic or mitral stenosis. Clinical, echocardiographic, and Holter data were recorded. A composite end point of death, appropriate internal cardioverter defibrillator discharges, resuscitated from sudden death, documented stroke, and admission for congestive heart failure was recorded. During a mean follow-up of 6.2±3 years, 86 patients (12%) met the composite end point with 30-day, 1-year, and 2-year event rates of 0.7%, 2.8%, and 4.7%, respectively. The hard event rate (death, defibrillator discharge, and resuscitated from sudden death) at 30 days, 1 year, and 2 years was 0%, 1.5%, and 3%, respectively. Stepwise multivariable analysis identified residual postoperative atrial fibrillation (hazard ratio, 2.12; confidence interval, 1. The degree of left ventricular (LV) hypertrophy in the study population was deemed significantly out of proportion to the duration and degree of hypertension; this decided after a thorough clinical evaluation by experienced physicians. We excluded the following patients:(1) patients <18 years and >65 years of age, (2) those with hypertensive heart disease of the elderly and concomitant LVOT obstruction, 13-16 (3) those with an ejection fraction <50%, and (4) those with the presence of more than mild aortic or mitral stenosis on the initial echocardiogram. Patients with hypertensive heart disease and concomitant LVOT obstruction were defined as those >65 years of age with a long-standing history of hypertension and characteristic sigmoid-shaped basal septal hypertrophy identified on echocardiography. These patients were excluded because they have a different pathophysiological and genetic profile from the typical HCM patients.13-16 Additionally, we eliminated the potentially confounding effects of end-stage disease (manifesting as depressed LV function) on outcomes by excluding such patients. Clinical DataDemographic, clinical (including standard and HCM-related risk factors, detailed family history, and examination), medication use (including antiarrhythmics and anticoagulation), and electrocardiographic data obtained at the time of the initial and follow-up visits were recor...