[Hypertrophic myocardiopathy with isolated obstruction of the right ventricle]

The authors report one case of biventricular hypertrophic cardiomyopathy in a 23 year old female patient with severe localized obstruction of the right ventricle (105 mm Hg gradient, almost complete angiographic disappearance of the middle and apical portions of the right ventricle during systole). Echocardiography, hemodynamic findings, and angiography demonstrated nonobstructive involvement of the left ventricle. Among other family members, only her father was noted to have minimal hypertrophic cardiomyopathy. A favorable response to therapy was obtained over 14 months with 320 mg of propranolol given daily (disappearance of palpitations and ventricular tachycardia which had previously been noted on a continuous 24 hour recording before treatment). Other very rare similar cases in the literature are analyzed. It is not possible to distinguish these cases from those with predominantly left ventricular involvement by clinical and radiological findings alone. However, the ECG can often demonstrate right ventricular involvement (right ventricular hypertrophy and/or right bundle branch block). Lastly, it appears that biventricular hypertrophic cardiomyopathies in young patients can initially be localized to only the right ventricle.