Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum

Coronary artery pathology is a major determinant of treatment strategy and outcome in patients with pulmonary atresia and intact ventricular septum (PA/IVS). For this reason, infants with PA/IVS routinely undergo preoperative cardiac catheterization. The goal of this study was to identify echocardiographic predictors of coronary artery pathology in infants with PA/IVS. The initial preoperative echocardiograms of 30 consecutive infants with PA/IVS (median age at diagnosis 1 day) were reviewed for indexes predicting the degree of coronary pathology. The tricuspid valve (TV) annulus diameter Z- score was determined and evidence of abnormal flow in the coronary arteries by Doppler was evaluated. Coronary pathology was defined by angiography and graded as: 0 = no fistulae; 1 = fistulae/no right ventricular (RV)–dependent coronary arteries; 2 = fistulae with 1 RV-dependent coronary; 3 = fistulae with ≥2- vessel RV-dependent coronary arteries. Outcome was classified as: 2 ventricles, “1.5” ventricles, and 1 ventricle. By angiography, 30% of the patients had grade 0 coronary pathology, 30% had grade 1, 20% had grade 2, and 20% had grade 3. There was 1 death in a patient with grade 3 coronary pathology. Among the survivors (median age at follow-up 28.6 months), biventricular circulation existed in 12 patients (41%), 7 patients (24%) were 1.5, and 10 (34%) were 1 ventricle. All patients with TV Z-score ≤−2 had coronary fistulae by angiography and 35% had grade 3 coronary pathology. None of the patients with grade 2 or 3 coronary artery pathology had a TV Z-score >−2.5. The sensitivity, specificity, positive, and negative predictive values of TV Z-score ≤−2.5 in predicting RV dependent coronary arteries were 100%, 83%, 80%, and 100%, respectively. Thus, in newborns with PA/IVS the echocardiographically derived TV Z-score predicts the likelihood of coronary artery fistulae and RV-dependent coronary arteries and can be used to rationalize the need for preoperative diagnostic catheterization.