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Unnatural history of the right ventricle in patients with congenitally malformed hearts

Published online by Cambridge University Press:  01 December 2010

Leo Lopez ,
Meryl S. Cohen ,
Robert H. Anderson ,
Andrew N. Redington ,
David G. Nykanen ,
Daniel J. Penny ,
John E. Deanfield  and
Benjamin W. Eidem
Leo Lopez*
Affiliation:
Division of Pediatric Cardiology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York, United States of America
Meryl S. Cohen
Affiliation:
Division of Cardiology, Children’s Hospital of Philadelphia, The Cardiac Center, University of Philadelphia School of Medicine, Philadelphia, Pennsylvania, United States of America
Robert H. Anderson
Affiliation:
Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Andrew N. Redington
Affiliation:
Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Canada
David G. Nykanen
Affiliation:
Division of Pediatric Cardiology, Arnold Palmer Children’s Hospital, The Congenital Heart Institute, Orlando, Florida, United States of America
Daniel J. Penny
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia
John E. Deanfield
Affiliation:
Cardiothoracic Unit, Great Ormond Street Hospital for Sick Children, Institute of Child Health, London, United Kingdom
Benjamin W. Eidem
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America
*
Correspondence to: L. Lopez, Division of Pediatric Cardiology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York, United States of America. Tel: 1-718-741-2184; Fax: 1-718-741-2570; E-mail: llmd@llmd.net
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Abstract

The long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.

Keywords

Tetralogy of FallotFontan circulationEbstein’s malformationcorrected transposition
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 107 - 112
Copyright
Copyright © Cambridge University Press 2010

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