Electrocardiographic and echo-doppler study in Duchenne muscular dystrophy

ABSTRACT

Twenty six patients with Duchenne muscular dystrophy [DMD] [4-18 years] and 16 controls [4-18 years] were subjected to cardiac and neurological examination. Patients were divided into gp I [early Duchenne 17 patients] and gp II [late Duchenne, 7 patients]. The electrocardiogram showed sinus tachycardia [77%], sinus arrhy thmias [69%] P wave abnormalities [19%], tall R in v 1 and V 2 [35%]. RSr [31%]. R/S >1 [54%], deep Q [42%] and ST-T changes in [58%]. Six cases [23%] had mitral valve prolapse by echocardiography. Ejection fraction [EF%] and fractional shortening [FS%] were significantly less in Duchenne patients compared to controls [p < 0.01]. EF and FS were significantly less in gp II [late Duchenne] compared to gp I [early Duchenne] [p < 0.01]. Significant impairement was also reported in peak velocity of early diastolic inflow [PE] [p < .05] and PE/peak velocity of late diastolic inflow PE/PA [p < 0.01] in the total gp and gp I compared to controls. PE/PA was significantly less in gp II compared to controls [p < 0.01] and compared to gp I [p < 0.01]. In 14 of the 26 patients we found significant impairment in atrial filling rat [p < 0.01] and rapid filling index [p < 0.05] as compared to controls. In other 10 patients impairement was reported in maximal diastolic posterior wall endocardial velocity [p < 0.001] and deceleration of early diastolic filling [p < 0.05]. We concluded that ECG is a sensetive tool in detecting cardiac involvement in DMD. Both systolic and diastolic functions are affected by myocardial dystrophy, such impairement is progressive throughout the course of the disease