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

The prevalence and hemodynamic consequences of regional wall motion ab normalities (RWMA) were evaluated in 84 advanced-stage Duchenne muscul ar dystrophy (DMD) patients who underwent echocardiographic and systol ic 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 pa tients) had akinetic and/or dyskinetic areas. In group II, 15 patients had ventricular dilation; 8 of the 15 had a history of cardiac failur e and 4 died during the study. Wall motion index (WMI), end-diastolic volume (EDV), and STI values increased significantly from group I to g roup II. In the whole group, a strong correlation was found between WM I and STI ratios. This study demonstrates a high prevalence of RWMA an d supports the viewpoint that RWMA, consequences of the myopathic proc ess, result in progressive left ventricular dilation, heart failure, a nd cardiac death.