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Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle
H L Walters1, C Mavroudis, C I Tchervenkov
1Department of Surgery, Wayne State University School of Medicine, Children's Hospital of Michigan, Detroit 48201, USA. halwalters@aol.com
Insights
Double outlet right ventricle (DORV) is a complex congenital heart defect where both great arteries originate from the right ventricle. Surgical repair focuses on connecting the left ventricle to the systemic circulation, with alternative procedures for complex anatomies.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Anatomy
Background:
- Double outlet right ventricle (DORV) is characterized by both great arteries arising from the right ventricle.
- Diagnosis relies on detailed morphological assessment, not solely on specific descriptors like aortic-mitral discontinuity.
- Understanding DORV's varied anatomy is crucial for effective management.
Purpose of the Study:
- To define the comprehensive morphological features of DORV.
- To outline preferred and alternative surgical strategies for DORV.
- To propose a framework for a DORV module in a pediatric cardiac surgical database.
Main Methods:
- Detailed morphological description including VSD, semilunar valves, great artery relationships, coronary anatomy, and outflow tract obstruction.
- Review of surgical treatments, including intraventricular tunnel repair and alternative procedures.
- Development of a proposed framework for a standardized surgical database module.
Main Results:
- DORV morphology is complex and requires thorough evaluation beyond specific diagnostic prerequisites.
- Intraventricular tunnel repair is the preferred surgical approach when feasible.
- Alternative surgical techniques are essential for managing complex DORV anatomies.
Conclusions:
- A comprehensive approach to DORV morphology is vital for surgical planning.
- Surgical management of DORV requires tailored strategies based on individual anatomy.
- A standardized database framework can improve data collection and analysis for DORV.
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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