Cardiology in the Young

Part 1: A Holistic Approach to Hypoplastic Left Heart Syndrome

Intermediate-term outcomes after paediatric cardiac extracorporeal membrane oxygenation – what is known (and unknown)

John M. Costelloa1 c1, David S. Coopera2, Jeffrey P. Jacobsa2, Paul J. Chaia2, Roxanne Kirscha3, Tami Rosenthala3, Heidi J. Daltona4, Joseph N. Grazianoa4 and James A. Quintessenzaa2

a1 Children's Memorial Hospital, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, United States of America

a2 The Congenital Heart Institute of Florida (CHIF), All Children's Hospital, Saint Petersburg, Florida, United States of America

a3 Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America

a4 Phoenix Children's Hospital, Phoenix, Arizona, United States of America

Abstract

The use of extracorporeal membrane oxygenation in infants and children with cardiac disease who develop refractory cardiogenic shock, cyanosis, or cardiac arrest is increasing. Early mortality in children with cardiac disease who require extracorporeal membrane oxygenation remains an important issue, as only 40% of cannulated patients survive to discharge from the hospital. However, it is encouraging that 90% children who are discharged alive from the hospital after extracorporeal membrane oxygenation are still alive at intermediate-term follow-up. Surviving patients are at risk for long-term dysfunction of multiple organ systems related to their underlying cardiac disease, non-cardiac comorbidities, treatment-related complications, and exposure to extracorporeal membrane oxygenation. Among the most important acute complications related to support with extracorporeal membrane oxygenation is injury to the central nervous system, which may contribute to adverse neurodevelopmental outcomes. All of these factors, in turn, influence quality of life. Many survivors remain medically complex related to their underlying cardiac disease, comorbidities, and sequelae of complications acquired over their lifetime. Neurological morbidity clearly plays an important role in approximately one-third of survivors, with significant deficits in approximately 10%. The limited data about quality of life data that are available for survivors of cardiac extracorporeal membrane oxygenation suggests that approximately 15–30% of survivors have at least moderately decreased quality of life. Overall, published data support the ongoing use of support with extracorporeal membrane oxygenation in children with acute cardiac failure, most of whom would die without it. However, programmatic efforts to improve the selection of patients and the preservation of the function of end organs during extracorporeal membrane oxygenation are clearly needed in order to improve long-term outcomes.

(Online publication December 13 2011)

Correspondence:

c1 Correspondence to: Dr J. M. Costello, MD, MPH, Director, Regenstein Cardiac Care Unit, Division of Cardiology, Children's Memorial Hospital, 2300 Children's Plaza, Box 21, Chicago, Illinois 60614, United States of America. Tel: +1 773 880 3099; Fax: +1 773 880 8111; E-mail: jmcostello@childrensmemorial.org