Abstract
About 10% of patients suffering from Duchenne muscular dystrophy (DMD) died of left heart failure at relatively younger age, however there have been few reports on the treatment of left heart failure in DMD. Thus we studied the cardiac functions and their responses to vasodilators and catecholamine preparations in 4 DMD patients with left heart failure. All cases were autopsied and the results indicated that they had cardiomyopathy.
These 4 patients had dyspnea, edema in the lower extremities, discomfort and palpitation, and their chest roentogenograms showed marked cardiomegaly. Out of these 4 patients with DMD (mean age 17 years), 2 had also mitral regurgitation (MR). Digitalis and diuretics were clinically effective in the initial stage of the heart failure, but these drugs became gradually ineffective as heart failure aggravated. On the results of the Swan-Ganz catheterization (SGC), they belonged to Forrester's subset 4, the mean values of pulmonary capillary wedge pressure and cardiac index were 31mmHg and 1.61/min/m2, respectively. The results of the tests indicated that all of them had severe left heart failure.
Hemodynamic data showed the improvement of cardiac function after administration of isosorbide dinitrite tapes as a vasodilator. Since their symptoms became worse with the passage of time, dopamine in 3 patients and dobutamine in 1 patient was also given, resulting in temporary improvement of the cardiac function to Forrester's subset 1 or 2. However they died within a short period after the treatment, one year and 3 months from the beginning of subjective symptoms.
In recent years, vasodilators and catecholamine preparations have been used for the treatment of heart failure together with digitalis and diuretics. Based on our experience, even in the case that digitalis and diuretics became ineffective, vasodilators and catecholamine preparations were temporarily effective. Especially dobutamine seemed to be more effective, since we observed an increase in cardiac output. We propose that the usefulness of the vasodilators and catecholamines remains to be further determined.
