Desena Holly C, Brumund Michael R, Superneau Duane, Snyder Christopher S
Department of Pediatrics, Tulane University, New Orleans, LA, USA.
Congenit Heart Dis. 2011 Jul-Aug;6(4):397-401. doi: 10.1111/j.1747-0803.2010.00471.x. Epub 2011 Mar 10.
Pompe disease is a rare genetic disorder resulting from a deficiency of the acid α-glucosidase enzyme. Although arrhythmias occur in these patients undergoing general anesthesia, they have not received sufficient emphasis in pediatric cardiology. We report a case of an infant with Pompe disease who experienced ventricular fibrillation during induction of anesthesia.