SEQUENTIAL-CHANGES IN CARDIAC STRUCTURE AND FUNCTION IN PATIENTS WITHDUCHENNE TYPE MUSCULAR-DYSTROPHY - A 2-DIMENSIONAL ECHOCARDIOGRAPHIC STUDY

Background Most patients with progressive muscular dystrophy of the Du chenne type (DMD) die cf respiratory failure at approximately 20 years of age. However, some patients with DMD die of heart failure in relat ively short periods of time. We investigated the long-term progression of cardiac impairment in patients with DMD by two-dimensional echocar diography. Methods We monitored 74 patients for 4 years with two-dimen sional echocardiography. Patients were classified into four groups acc ording to the 8-grade Swinyard-Dever system. We also evaluated the ech ocardiographic features of 22 other patients with DMD with studies per formed within 1 year before their deaths. Results During the 4-year fo llow-up the left ventricle expanded, and regional left ventricular wal l motion abnormalities developed in the posterior wall and apex. Almos t all patients had myocardial dysfunction that progressed in parallel with their Swinyard-Dever stage. However, in a few patients who died o f congestive heart failure, left ventricular dilation and circumferent ial left ventricular wall motion were severely impaired. Conclusions M yocardial impairment is accelerated in patients with DMD who died of h eart failure. Two-dimensional echocardiography is a useful tool for th e early diagnosis of left ventricular dysfunction and provides useful information for the treatment of patients with DMD.