Preserved Subcutaneous Implantable Cardioverter-defibrillator Function Following Septal Myectomy and Coronary Artery Unroofing in a Pediatric Patient with Severe Hypertrophic Cardiomyopathy.

Abstract

Hypertrophic cardiomyopathy (HCM) is an inherited disease present in 1 in 500 individuals and is the most common cause of sudden cardiac death in children. We present the case of a 17-year-old boy with HCM and a primary prevention subcutaneous implantable cardioverter-defibrillator (S-ICD) who developed left ventricular outflow tract obstruction and a myocardial bridge of the left anterior descending coronary artery. The patient underwent a septal myectomy/myotomy and muscular bridge unroofing. The S-ICD system was undisturbed during the surgery, with no loss of function. Septal myectomy may be accomplished in pediatric HCM patients following optimal S-ICD placement with maintained S-ICD function.