Ventricular arrhythmia in Duchenne muscular dystrophy: prevalence, significance and prognosis.
The prevalence and prognostic value of ventricular arrhythmias were examined in 45 Duchenne muscular dystrophy patients without congestive heart failure and followed up for 3 yr. Baseline evaluation included 24 h ECG monitoring, systolic time intervals measurement (preejection period/left ventricular ejection time PEP/LVET), echocardiogram and vital capacity tests. Fifteen patients (33%) had ventricular premature beats (VPB > or = 2 h-1). More complex ventricular ectopy (Lown grades 3, 4A, 4B) occurred in 12 patients (27%), who had abnormal ventricular contractility (PEP/LVET > 0.48) and an area of akinesia or dyskinesia. Complex VPB were present on presentation in only 3 of the 30 survivors (10%) but were detected in 6 of the 15 patients (40%) who died. Patients who died suddenly were more likely to have had documented complex ventricular arrhythmias (6 of 9; 66%). It is concluded that: (1) significant arrhythmias frequently coexist with asymptomatic left ventricular dysfunction and wall motion abnormalities; (2) complex VPB as well as left ventricular dysfunction and dilated cardiomyopathy are risk factors for sudden death.
Chenard
A A
AA
Department of Internal Medicine, Central Hospital of Meaux, France.
Becane
H M
HM
Tertrain
F
F
de Kermadec
J M
JM
Weiss
Y A
YA
D000293:Adolescent; D000328:Adult; D000375:Aging / Q000502:physiology; D001145:Arrhythmias, Cardiac / Q000453:epidemiology* / Q000209:etiology* / Q000503:physiopathology; D005117:Cardiac Complexes, Premature / Q000503:physiopathology; D002648:Child; D003645:Death, Sudden / Q000453:epidemiology / Q000209:etiology; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D005500:Follow-Up Studies; D006339:Heart Rate / Q000502:physiology; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000150:complications / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000150:complications*; D011379:Prognosis; D012131:Respiratory Insufficiency / Q000209:etiology; D012307:Risk Factors; D016277:Ventricular Function, Left / Q000502:physiology; D014797:Vital Capacity