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Surgery for the functionally univentricular heart in patients with visceral heterotaxy

Published online by Cambridge University Press:  10 January 2006

Christo I. Tchervenkov ,
Marshall L. Jacobs  and
Danny Del Duca
Christo I. Tchervenkov
Affiliation:
Division of Cardiovascular Surgery, Montreal Children's Hospital of the McGill University Health Center, McGill University, Montréal, Québec, Canada
Marshall L. Jacobs
Affiliation:
Section of Pediatric Cardiac Surgery, St Christopher's Hospital for Children, Drexel University College of Medicine, Philadelphia, Pennsylvania, United States of America
Danny Del Duca
Affiliation:
Division of Cardiovascular Surgery, Montreal Children's Hospital of the McGill University Health Center, McGill University, Montréal, Québec, Canada
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Extract

The heterotaxy syndromes are characterized by a high incidence of cardiac anomalies of extreme heterogeneity. Due to this, the surgical management is varied and challenging. Although a minority of patients can undergo biventricular repair, the complexity of the cardiac defects, and the high incidence of ventricular hypoplasia, mandate a surgical approach in the majority of patients depending on the creation of a functionally univentricular heart. Traditionally, the functionally univentricular approach was associated with a high mortality. More recently, the results have been improving as a result of better understanding of these malformations, and logical improvements in surgical technique. In this review, we will make brief comments on the nomenclature and classification of the heterotaxy syndromes, and the range of cardiac anomalies, before concentrating on the surgical treatment for those patients having functionally univentricular hearts in the setting of heterotaxy. More specifically, we will review initial palliation in early life, creation of the cavopulmonary anastomoses, including the Kawashima procedure and the Fontan circulation, and the role of transplantation.

Keywords

Right isomerismleft isomerismaspleniapolysplenia

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