Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia—ventricular septal defect

Abstract

Pulmonary atresia (PA) and ventricular septal defect (VSD) is a complex and extremely heterogeneous cardiopulmonary malformation that has not been accurately defined, as evidenced by the synonymous use of the term with tetralogy of Fallot with PA. The anatomy and morphology of the pulmonary circulation to a large extent determines the surgical approach and overall outcome, with the intracardiac anatomy playing a secondary role. Based on the characterization of the pulmonary circulation a new classification of PA-VSD is proposed. In type A, there are only native pulmonary arteries (NPA). In type B, pulmonary blood flow is provided by both NPA and by major aortopulmonary collateral arteries [MAPCA(s)]. In type C, there are only MAPCA(s) and no NPA. This new classification is proposed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.