title: Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum.
abstract: 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.
authors.affiliation: Department of Cardiology, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
authors.forename: G M
authors.initials: GM
authors.lastname: Satou
authors.forename: S B
authors.initials: SB
authors.lastname: Perry
authors.forename: K
authors.initials: K
authors.lastname: Gauvreau
authors.forename: T
authors.initials: T
authors.lastname: Geva
mesh_terms: D006328:Cardiac Catheterization; D003330:Coronary Vessel Anomalies / Q000000981:diagnostic imaging* / Q000601:surgery; D004452:Echocardiography; D018618:Echocardiography, Doppler, Color; D005260:Female; D005500:Follow-Up Studies; D005583:Fourier Analysis; D006346:Heart Septum / Q000000981:diagnostic imaging*; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging* / Q000601:surgery