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Designing therapeutic strategies for patients with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs

Published online by Cambridge University Press:  21 January 2005

Robert M. Freedom ,
Shi-Joon Yoo ,
Jennifer Russell ,
Donald Perrin  and
William G. Williams
Robert M. Freedom
Affiliation:
Division of Cardiology of the Department of Pediatrics, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada Department of Diagnostic Imaging, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada Division of Pathology of the Department of Laboratory Medicine, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada
Shi-Joon Yoo
Affiliation:
Department of Diagnostic Imaging, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada
Jennifer Russell
Affiliation:
Division of Cardiology of the Department of Pediatrics, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada
Donald Perrin
Affiliation:
Division of Pathology of the Department of Laboratory Medicine, The Hospital for Sick Children, The University of Toronto Faculty of Medicine, Toronto, Canada
William G. Williams
Affiliation:
Division of Cardiovascular Surgery, The Hospital for Sick Children, and Departments of Pediatrics, Medical Imaging, Laboratory Medicine and Surgery, The University of Toronto Faculty of Medicine, Toronto, Canada
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Abstract

The palliation of the cyanotic child with a dominant morphologically left ventricle, discordant ventriculo-arterial connections, and obstruction to the pulmonary outflow tract has continued to evolve and mature. The evolution began in the early days of surgical palliation with the Blalock–Taussig shunt, extended to construction of cavopulmonary shunts, if required, and then to the Fontan procedure and its subsequent modifications. This journey took nearly 30 years to complete. There is increasing clinical data to document the beneficial effects of this approach, with ever-improving outcomes. Some aspects of the history of the cavopulmonary shunt have been previously reviewed in this journal and elsewhere,1–5 as have analysis of outcomes for some groups of patients considered for surgical completion of the Fontan circulation.6–12 While there has been some ongoing interest in ventricular septation since the early success of Sakakibara et al.,13 this approach has largely been abandoned. Considerably more challenges and debate resonate in the surgical algorithms defined for patients whose hearts are characterized by a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs. This latter group will be the focus of this review, as will the aetiology of the myocardial hypertrophy that is particularly frequent in this group of patients, its clinical recognition, indeed its anticipation, and the multiple surgical strategies designed to prevent or treat it. All these manoeuvres are considered to optimise suitability for, and outcome from, creation of the Fontan circulation.

Keywords

Damus–Kaye–StanselFontan outcomemyocardial hypertrophyNorwood procedurepulmonary artery bandingsystemic outflow tract obstruction
Type
Continuing Medical Education
Information
Cardiology in the Young , Volume 14 , Issue 6 , December 2004 , pp. 630 - 653
Copyright
© 2004 Cambridge University Press

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