Prenatal Predictors of Postnatal Outcome in Pulmonary Atresia with Intact Ventricular Septum: A Multicenter Study

PI: Michael Puchalski

To identify fetal echocardiographic predictors of post-natal outcomes (type of post-natal circulation and survival) in PA/IVS
Hypothesis: Fetal echocardiographic anatomic features (partite nature of the RV, degree of tricuspid regurgitation and presence/absence of coronary sinusoids), measurements (Z-scores of right heart structures and pulmonary arteries), as well as Doppler indices will help determine the eventual post-natal circulation (biventricular, uni-ventricular and one-and-half ventricle repair) and transplant free survival up to 5-year of age in patients with PA/IVS.
Evaluate the longitudinal pre-natal growth of right heart structures and pulmonary arteries in biventricular, univentricular and one-and-half ventricle group.
Hypothesis: Longitudinal pre-natal growth of right heart structures and pulmonary arteries in the biventricular groups will be higher than the univentricular or one-and-half ventricle groups. We will use gestational age corrected Z-scores and right-left heart structure ratios to account for prenatal changes in right heart structure.