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Hypoplastic Left Heart Syndrome pp 129–148Cite as

Staged Reconstruction For HLHS

The Bidirectional Cavopulmonary Shunt

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Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 246))

Abstract

The Norwood operation (with its technical variations) is the only useful reconstructive surgical procedure for neonates with HLHS and related abnormalities [1]. While the Norwood operation allows the neonate to survive well beyond the first few weeks of life, it is not a stable solution for the long term. The postoperative Norwood physiology presents several problems, some of which center on RV function in the presence of pulmonary recirculation. The volume load that is necessarily imposed on the heart in HLHS may place the RV in an unfavorable position on the Starling curve, which typically has a leftward shift in comparison to the normal LV. The systemic tricuspid valve, which lacks annular definition and is supported by septal-derived papillary muscles, is functionally exquisitely sensitive to volume loading of the RV, especially when required to eject at systemic arterial pressure. Tricuspid insufficiency may thus be the limiting factor for future suitability for a Fontan operation. Finally, the small prosthetic (PTFE) tubes typically employed to create a controlled source of pulmonary blood flow (MBTS) are destined to become obstructed with thrombus, and/or neointima. Even a widely patent shunt may become inadequate as the baby grows. At the same time, the low diastolic arterial pressure typically associated with a MBTS may place the baby at continued risk for myocardial ischemia as activity increases. The presence of the shunt may also contribute to ongoing limitation of growth and distortion of the PAs. For survivors of the Norwood operation, an unacceptably high late mortality has been observed for infants who have not yet undergone further staging operations [2]. For all of these reasons, a further, more definitive procedure is required.

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  1. Division of Cardiothoracic Surgery, University of California-San Francisco Children’s Hospital, California, USA

    Tom R. Karl M.S., M.D.

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  1. Tom R. Karl M.S., M.D.
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Editors and Affiliations

  1. Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, USA

    Jack Rychik M.D.  & Gil Wernovsky M.D.  & 

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Karl, T.R. (2003). Staged Reconstruction For HLHS. In: Rychik, J., Wernovsky, G. (eds) Hypoplastic Left Heart Syndrome. Developments in Cardiovascular Medicine, vol 246. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-0253-1_8

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  • DOI: https://doi.org/10.1007/978-1-4615-0253-1_8

  • Publisher Name: Springer, Boston, MA

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