Ventricular fibrillation in a patient with Pompe disease: a cautionary tale.

Abstract

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.

• Authors: Holly C Desena, Michael R Brumund, Duane Superneau, Christopher S Snyder
• Journal: Congenital heart disease (Congenit Heart Dis) 2011 Jul-Aug Vol. 6 Issue 4 Pg. 397-401 ISSN: 1747-0803 [Electronic] United States
• PMID: 21392261 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.

Topics

• Anesthesia, General (adverse effects)
• Cardiomegaly (etiology)
• Catheterization, Central Venous
• Electrocardiography
• Enzyme Replacement Therapy
• Female
• Glycogen Storage Disease Type II (complications, diagnosis, drug therapy)
• Humans
• Infant
• Ventricular Fibrillation (diagnosis, etiology, therapy)