Familial Hypertrophic Cardiomyopathy Generating a Marked Left Ventricular Pressure Gradient in an 82-Year-Old Woman

Abstract

Familial nonobstructive hypertrophic cardiomyopathy in 78-year-old woman was diagnosed in 1988. Since then she has been treated with a beta-blocking drug and a calcium antagonist. Her clinical condition was NYHA I or II for several years. Echocardiography revealed asymmetric septal hypertrophy in 1988 (interventricular septal thickness=21mm, posterior wall thickness=10mm). Systolic anterior motion of the mitral valve was first observed at the end of 1992. The left ventricular pressure gradient also gradually increased; it was about 138mmHg in 1993 (age 82 yrs). The left ventricular ejection time index increased from 376msec in May, 1992 to 459msec in May, 1994. In September, 1994, the patient's condition gradually deteriorated (NYHA IV), and she was admitted to our hospital. To attenuate the left ventricular pressure gradient, 150mg of disopyramide was administered. Her condition markedly improved: the left ventricular pressure gradient decreased from 180mmHg to 76mmHg, and the left ventricular ejection time index decreased from 485msec to 419msec. These results indicate that a left ventricular pressure gradient can be generated rapidly even in a very old patient, and that disopyramide may be useful to attenuate the left ventricuolar pressure gradient.