Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease

Abstract

Ebstein's anomaly is a rare congenital heart defect that is characterized by a spectrum of anatomical abnormalities of the tricuspid valve that also involve the right atrium and right ventricle. The extant nomenclature for Ebstein's anomaly and our approach to the description of the severity of Ebstein's anomaly are reviewed with the objective of establishing a unified reporting system. Although there are common features in Ebstein's anomaly, there is a wide spectrum of pathology with an infinite variety of combinations of severity of the involved structures. An effort was made to develop a classification system that would take into consideration the anatomic abnormalities that help direct the surgical management, particularly in regard to tricuspid valve repair or valve replacement. Isolated congenital tricuspid stenosis and regurgitation are also rare, and a simple classification system is presented. Acquired causes of tricuspid regurgitation and stenosis are more common and are included in the classification system. A comprehensive database set for these malformations is presented so that a comprehensive risk stratification analysis can be performed. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and risk factors are presented.