Abstract
Purpose Little is known about sudden cardiac death risk and arrhythmias in boys with Duchenne muscular dystrophy(DMD). The goal of the study is to describe ECG findings and arrhythmia burden in a multi-center contemporary cohort of boys with DMD. Methods We conducted a longitudinal retrospective study of 436 boys with DMD followed at 17 centers across the US and Canada from 2005 to 2015. Results A total of 1227 ECGs and 222 holter monitor studies were reviewed during the study period. Median of 2 ECGs were obtained per patient (IQR 1-4). ECG analysis demonstrated sinus rhythm (SR) in 82.2%, sinus tachycardia (ST) in 14.8%, and sinus bradycardia in 3%. Median heart rate was 94 bpm (IQR of 84-104). Hypertrophy was noted in 30.7% of ECGs in form of right ventricular hypertrophy (40.8%), left ventricular hypertrophy (33.3%), and biventricular hypertrophy (25.8%). Ectopy (isolated premature atrial contractions or isolated premature ventricular contractions) was noted in 1.1% of ECGs. Of the holters performed - 146 boys (65.8%) had one, 53 boys (23.8%) had two, and 23 boys (10.4%) had three. Median age at first baseline holter was 14 years (IQR 11-17). Underlying rhythm was SR in most (116, 79.4%) followed by ST (20, 13.7%). Ectopy occurred in 5 boys - 4 had supraventricular tachycardia (SVT) and 1 had ventricular tachycardia (VT). For second holter, median age of boys was 18 years (IQR 15.5-19). Underlying rhythm was SR in 48 (91%) and VT in 5 (9%). Atrial ectopy occurred in 4 boys. For third holter, median age was 18 years (IQR 14.5-19). Underlying rhythm was SR in 21 (91%), ST in 1 (4.3%), and atrial fibrillation in 1 (4.3%). Ectopy occurred in 8 boys - SVT in 3 and VT in 5. Using regression analysis, incidence of significant SVT or VT increases with age (p Conclusion Data suggests that ECG & rhythm abnormalities are common in boys with DMD. Holter monitoring is not used frequently; however, screening for arrhythmias is warranted especially as boys age.
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