Abstract 13544: Differences in the Population of Muscular Dystrophy Patients With Respect to Ventricular Tachycardia, Heart Failure and Use of Implantable Cardioverters Defibrillators

Abstract

Introduction: As the treatment of the respiratory and skeletal muscle complications associated with muscular dystrophy (MD) has improved, males with MD are living longer and are more likely to develop cardiac disease. The impact of sudden cardiac death related to ventricular arrhythmias and heart failure and the indications for ICD implantation are not well established. Hypothesis: We evaluated the hypothesis that VT is a significant contributor to mortality in hospitalized patients with MD and this is improved with ICD implantation. Methods: We searched the Pediatric Health Information System (PHIS) database for males with discharge diagnosis codes of MD and different combinations of heart failure (HF), ventricular arrhythmias (VT) and ICD placement between 2003 and 2013. Results: There were significant differences in the mean length of stay and mean age between MD patients with HF and/or VT versus all hospitalized male MD patients (p<0.05). Out of a total of 76 deaths in MD patients, 32% (n=24) were related to HF. Of those, 30% (n=7) also had a diagnosis of VT. Isolated VT without HF was only 1% (n=42) of admissions and the cause of death in 2 patients. However, ICD’s were implanted in 12% (n=5) of MD/VT patients compared to 5% (n=5) of MD/HF/VT patients. No deaths were seen in patients with ICD’s. Only 1 ICD was placed prior to 2009. Conclusions: VT was a frequent cause of death in hospitalized male patients with MD and HF, however isolated VT without HF was not. No deaths were found in the small number of patients with ICD’s. Further prospective studies are needed to validate the importance of ICD use in MD patients