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Optimal timing of the second stage of palliation for hypoplastic left heart syndrome facilitated through home monitoring, and the results of early cavopulmonary anastomosis

Published online by Cambridge University Press:  10 January 2006

Nancy S. Ghanayem ,
James S. Tweddell ,
George M. Hoffman ,
Kathleen Mussatto  and
Robert D. B. Jaquiss
Nancy S. Ghanayem
Affiliation:
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Division of Critical Care, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
James S. Tweddell
Affiliation:
Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Herma Heart Center at Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
George M. Hoffman
Affiliation:
Division of Critical Care, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Department of Anesthesia and Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Herma Heart Center at Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Kathleen Mussatto
Affiliation:
Herma Heart Center at Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
Robert D. B. Jaquiss
Affiliation:
Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America Herma Heart Center at Children's Hospital of Wisconsin and Medical College of Wisconsin, Milwaukee, Wisconsin, United States of America
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Extract

For children with hypoplastic left heart syndrome, contemporary management over three stages includes a window of high risk for sudden death between the initial stage of palliation, the Norwood operation itself, and the second stage, creation of the bidirectional superior cavopulmonary connection. The risk is highest at a time when patients have been discharged from the hospital to grow and prepare for the second stage,1–4 and has persisted despite the remarkable improvements in immediate postoperative and hospital survival after the initial surgery.5,6 Potential contributing factors to the increased vulnerability to sudden death between the stages include the limited circulatory reserve inherent in the parallel circulations supported by a functionally univentricular heart, the reliance on a prosthetic shunt which is susceptible to thrombosis, and congenital or acquired anatomical cardiovascular abnormalities such as aortic atresia, residual obstruction in the aortic arch, tricuspid valvar insufficiency, or right ventricular dysfunction.7–12

Type
Research Article
Information
Cardiology in the Young , Volume 16 , Supplement S1: Controversies and Challenges in the Management of the Functionally Univentricular Heart , February 2006 , pp. 61 - 66
Copyright
© 2006 Cambridge University Press

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