Cardiomyopathy
Relation of Cardiac Dysfunction to Rhythm Abnormalities in Patients With Duchenne or Becker Muscular Dystrophies

https://doi.org/10.1016/j.amjcard.2016.01.031Get rights and content

The association between systolic cardiac dysfunction and arrhythmia development in patients with Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) is generally assumed but has not been extensively studied. The purpose of this study was to describe arrhythmias in patients with DMD and BMD in the present era and determine whether arrhythmia development is associated with cardiac dysfunction. This is a single-center retrospective review of 237 Holters from 91 patients with DMD (mean = 17 ± 4 years, range 3 to 27 years) and 64 Holters from 21 patients with BMD (mean = 18 ± 7 years, range 4 to 31 years) with corresponding echocardiography. Holters were stratified by age of patient at the time of study and ejection fraction: normal (≥55%), mild (<55% and ≥45%), moderate (<45% and ≥30%), and severe (<30%). Arrhythmias included frequent atrial and ventricular premature complexes (>10/hr), couplets, and runs of supraventricular and ventricular tachycardias. Arrhythmias occurred in 44% of DMD and 57% of BMD patients and were significantly associated with decrease in cardiac function. Clinically significant arrhythmias (supraventricular tachycardia and ventricular tachycardia) occurred in 10% of all Holters obtained in patients with DMD and 25% of all Holters obtained in patients with BMD. Subgroup analysis of Holters from patients with DMD demonstrated that arrhythmias increased with decreasing ejection fraction regardless of age, but that age was also a significant predictor of arrhythmia development. In conclusion, among patients with DMD or BMD, arrhythmias increase with development of cardiac dysfunction.

Section snippets

Methods

A retrospective chart review of all patients with DMD or BMD at Texas Children's Hospital (Houston, Texas) was performed with approval from the internal review board. Patients with both 24-hour Holter monitoring and echocardiography were included. Patients with DMD or BMD generally have a slow decrease in cardiac function, but can also demonstrate acute decrease with subsequent improvement in function, particularly during times of acute respiratory illness. To accommodate the fluctuating

Results

A retrospective chart review was conducted on 266 patients with DMD and 56 patients with BMD at Texas Children's Hospital based on data from 2008 to 2015. Of these patients, all of whom were males, 91 patients with DMD and 21 patients with BMD had 24-hour Holter recordings with corresponding echocardiography that met study criteria. From these patients, a total of 237 and 64 Holter studies with corresponding echocardiography were reviewed and summarized. At the time of Holter monitoring,

Discussion

The association between cardiac dysfunction and arrhythmias in the muscular dystrophies has generally been assumed; however, this is the first study to formally evaluate and demonstrate a statistically significant association between progressive cardiac dysfunction and cardiac arrhythmias in patients with DMD or BMD. Among patients with DMD, we found a statistically significant association between cardiac dysfunction and incidence of any arrhythmia as well as clinically significant arrhythmias

Acknowledgment

The authors thank Melissa Smith, RN and Melissa Domino, RN (Texas Children's Hospital, Houston, Texas) for their assistance with data collection and Shaine Morris, MD, MPH (Texas Children's Hospital, Houston, Texas) for advice on statistical analysis.

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