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Current issues and perspectives in hypoplasia of the left heart

Published online by Cambridge University Press:  14 April 2005

David Sedmera
Affiliation:
Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina, United States of America
Andrew C. Cook
Affiliation:
Cardiac Unit, Institute of Child Health, University College London, London, United Kingdom
Girish Shirali
Affiliation:
Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Tim C. McQuinn
Affiliation:
Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina, United States of America Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America

Abstract

Hypoplastic left heart syndrome is a rare but serious form of congenital cardiac disease, characterized by underdevelopment of the components of the left heart, rendering the left ventricle non-functional. Its aetiology is largely unknown, but there is certainly a genetic component. Prenatal diagnosis nowadays uncovers about half of cases. Postnatal options for treatment include comfort care, 3-stage palliative surgery, or cardiac transplantation. In this review, we discuss the morphology, possible pathogenetic mechanisms, clinical management, and perspectives of prenatal intervention based on work in animal models.

Type
Continuing Medical Education
Copyright
2005 Cambridge University Press

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