Cardiac asynchrony in Duchenne muscular dystrophy

Abstract

Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. Heart failure is a classical complication in this disease. Little data are available about systolic dyssynchrony in DMD. We sought to assess the prevalence of left ventricular dysfunction and systolic asynchrony in DMD patients using echocardiographic parameters. We performed electrocardiography and echocardiography for adult's patients with DMD. For systolic dyssynchrony assessment, echocardiography-Doppler was performed and completed by tissular Doppler imaging. 48 DMD were included in our study. Age ranged from 20 to 37years. QRS duration >120ms was present in 10 patients/48 and 1 patient disclosed a QRS duration >150ms. Left ventricular (LV) ejection fraction (EF) ranged from 10 to 62% with a median of 43%. Inter-ventricular asynchrony was found in 11.9% of patients with EF<35% and in 2.6% of patients with EF>35%. Intra-ventricular asynchrony was present in 6% of patients with EF<35%. We found a high prevalence of LV dysfunction in DMD. Systolic ventricular asynchrony seems frequent particularly in patients with EF<35%.