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Ann Thorac Surg 2000;69:S249-S263
© 2000 The Society of Thoracic Surgeons

Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle

^a Department of Surgery, Wayne State University School of Medicine, Children’s Hospital of Michigan, Detroit, Michigan, USA
^b Department of Surgery, Northwestern University School of Medicine, Children’s Memorial Hospital, Chicago, Illinois, USA
^c Department of Surgery, McGill University, The Montreal Children’s Hospital, Montreal, Quebec, Canada
^d Department of Surgery, University of South Florida School of Medicine, All Children’s Hospital, St. Petersburg, Florida, USA
^e Department of Surgery, Marie Lannelongue Hospital, Paris, France
^f Department of Surgery, Hanneman University School of Medicine, St. Christopher’s Children’s Hospital, Philadelphia, Pennsylvania, USA

Address reprint requests to Dr Walters, Department of Cardiovascular Surgery, Children’s Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201
e-mail: halwalters{at}aol.com

Presented at the International Nomenclature Conferences for Pediatric Cardiac Surgery, 1998–1999.

Abstract

Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.

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