Cardiology in the Young

Part 1: A Holistic Approach to Hypoplastic Left Heart Syndrome

Feeding, growth, nutrition, and optimal interstage surveillance for infants with hypoplastic left heart syndrome

David A. Hehira1a2 c1, David S. Coopera3, Elizabeth M. Waltersa4 and Nancy S. Ghanayema2

a1 Division of Pediatric Cardiology, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America

a2 Division of Pediatric Critical Care Medicine, Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America

a3 The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, United States of America

a4 The Heart Center, All Children's Hospital, Miami, Florida, United States of America

Abstract

Improvement in operative survival of patients with hypoplastic left heart syndrome has led to increasing emphasis on prevention of interstage mortality. Many centres have improved interstage results through programmes of home monitoring following discharge after the Norwood (Stage 1) operation. Experience with heightened interstage surveillance has identified failure to thrive during infancy as a modifiable risk factor for this population, one that has been linked to concerning outcomes at subsequent palliative surgeries. Ensuring normal growth as an infant has thus become a priority of management of patients with functionally univentricular hearts. Herein, we review the existing evidence for best practices in interstage surveillance and optimal nutrition in infants with functionally univentricular hearts. In addition, we highlight data presented at HeartWeek 2011, from Cardiology 2011, the 15th Annual Update on Pediatric and Congenital Cardiovascular Disease, and the 11th Annual International Symposium on Congenital Heart Disease.

(Online publication December 13 2011)

Correspondence:

c1 Correspondence to: Dr D. A. Hehir, MD, 9000 West Wisconsin Avenue, Milwaukee, Wisconsin 53201, United States of America. Tel: 414-266-5711; Fax: 414-266-3563; E-mail: dhehir@mcw.edu