Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy

BACKGROUND: Patients with Duchenne Muscular Dystrophy (DMD) develop cardiac fibrosis and dilated cardiomyopathy. We described the frequency of significant Holter findings in DMD, the relationship between cardiac function and arrhythmia burden, and the impact of these findings on clinical management. METHODS AND RESULTS: A retrospective review was done of patients with DMD who received a Holter from 2010 to 2014. Clinical and arrhythmic outcomes were analyzed. Patients were classified based on left ventricular ejection fraction (LVEF): ≥55%, 35% to 54% and <35%. Significant Holter findings included atrial tachycardia, ventricular tachycardia and atrial fibrillation/flutter. Logistic regression was used to assess predictors of significant Holter findings and change in care. The study included 442 Holters in 235 patients. Mean age was 14±4 years. Patients with cardiac dysfunction were older, and had increased late gadolinium enhancement and left ventricular dilation (P<0.01). There were 3 deaths (1%), all with normal function and none cardiac. Patients with LVEF <35% had more arrhythmias including nonsustained atrial tachycardia (P=0.01), frequent premature ventricular contractions, ventricular couplets/triplets, and nonsustained ventricular tachycardia (P<0.001) compared to the other groups. LVEF <35% (P<0.001) was the only predictor of clinically significant Holter finding. Four patients (40%) had change in medication in the LVEF <35% group compared to 9 (3%) in the ≥55% and 4 (4%) in the 35% to 54% groups (P<0.001). CONCLUSIONS: Sudden cardiac events are rare in DMD patients with an LVEF >35%. Significant Holter findings are rare in patients with DMD who have an LVEF >35%, and cardiac dysfunction appears to predict significant Holter findings. Holter monitoring is highest yield among DMD patients with cardiac dysfunction.