Initial, intra-operative, and post-operative evaluation of children with pulmonary atresia with intact ventricular septum with emphasis on the coronary connections to the right ventricle

Pulmonary atresia and intact ventricular septum (PAIVS), more than most congenital cardiac defects, requires detailed assessment for initial decision making and to guide intraoperative procedures. Among the important questions to answer are the degree of tricuspid valve (TV) and right ventricular (RV) hypoplasia and whether or not significant connections exist between the coronary arteries and the right ventricle (RV-CACs). When the TV and RV hypoplasia are severe and/or significant RV-CACs exist, these lesions are thought to preclude a two-ventricle repair (2VR) at most institutions. Our approach, however, is that these lesions can be defined and successfully treated making an adequate 2VR probable.We have developed and successfully employed a combined echocardiographic and angiographic approach for neonates at the severe end of the PAIVS spectrum and particularly those with RV-CACs that has guided and allowed 2VRs in these infants. Our approach includes preoperative, intraoperative and postoperative echocardiography including transthoracic, transesophageal (TEE) and cardiac surface imaging. The echocardiographic studies are used to define the TV and RV abnormalities and determine if RV-CACs are present. Significant RV-CACs are indicated by bidirectional flow in the RV-CAC with associated coronary flow reversal and dilated major coronary arteries with abrupt caliber changes at the site of the connection. When significant RV-CACs are present, cardiac catheterization with complementary right ventricular and aortic root angiography from similar projections aids localization of the connections as well as the associated coronary stenoses and interruptions which seem to result from hypertensive, hypoxic perfusion. The combined use of these techniques allows preoperative localization of significant RV-CACs while the use of intraoperative transesophageal and epicardial surface echocardiography aids in their ligation. Postoperatively, identification of any regional wall motion abnormality by TEE, should prompt a search for previously undetected or residual coronary artery connections. This detailed diagnostic approach to the evaluation and treatment of PAIVS will even allow infants at the severe end of the spectrum to be on a 2VR track.