Predominant, Severe Right Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy is a primary genetic heart disorder with variable phenotype that involves myocardial thickening and obstruction of the ventricular outflow tract. In contrast to left ventricular pathology, the involvement of the right ventricle is uncommon, occurring in up to 15% of patients. Histological findings appear to be similar to those in the left ventricle, suggesting similar pathogenesis, but the rarer right-side flow obstruction may result in more severe symptoms. Occasionally, predominant right ventricular disease can be seen.1–7 A 52-year-old male with a history of alcohol and psychotropic drug abuse was referred to our department with New York Heart Association class III symptoms of heart failure after a resuscitated cardiac arrest. An ECG showed left atrial abnormality, left axis deviation, and left ventricular strain pattern (Figure 1). Transthoracic echocardiogram revealed marked symmetric left ventricular hypertrophy (diastolic thickness 21 to 25 mm) without signs of outflow tract obstruction. Normal contractile function of both ventricles was found (left ventricular ejection fraction 61%). A significant thickening of …