PREVALENCE OF LEFT-VENTRICULAR SYSTOLIC DYSFUNCTION IN DUCHENNE MUSCULAR-DYSTROPHY - AN ECHOCARDIOGRAPHIC STUDY

The prevalence and hemodynamic consequences of regional wall motion abnormalities (RWMA) were evaluated in 84 advanced-stage Duchenne muscular dystrophy (DMD) patients who underwent echocardiographic and systolic time interval (STI) examination. A satisfactory echocardiogram was obtained in 72 patients who were divided into two groups: group I (33 patients) had normal wall motion or minor changes, and group II (39 patients) had akinetic and/or dyskinetic areas. In group II, 15 patients had ventricular dilation; 8 of the 15 had a history of cardiac failure and 4 died during the study. Wall motion index (WMI), end-diastolic volume (EDV), and STI values increased significantly from group I to group II. In the whole group, a strong correlation was found between WMI and STI ratios. This study demonstrates a high prevalence of RWMA and supports the viewpoint that RWMA, consequences of the myopathic process, result in progressive left ventricular dilation, heart failure, and cardiac death.