[Hypertropic obstructive cardiomyopathy. A family study].

Eight patients with HOCM from the same family are presented. The clinical findings and the diagnostic and therapeutic procedures are discussed. Chest pain, palpitations and effort dyspnea were the main clinical symptoms. A characteristic systolic ejection murmur of late onset was present in all the cases. In 6 patients there were electrocardiographic signs of left ventricular hypertrophy and only 4 patients exhibited abnormal septal depolarization. Echocardiography revealed an enhanced diastolic interventricular septal to left ventricular posterior wall ratio (above 1.3), diminished left ventricular septum movement, systolic anterior motion of the mitral valve and mid systolic closure of the aortic valve. Cardiac catheterization showed a left ventricular out-flow gradient which was increased by isoproterenol infusion. In 5 patients the left ventricular angiogram disclosed systolic obliteration of the left ventricle and in 4 cases mitral regurgitation. In the therapy of HOCM, calcium antagonists are now widely used and seem to have replaced betablocking agents.