Echocardiographic Predictors of Outcome in Fetuses with Pulmonary Atresia with Intact Ventricular Septum

Objective: We sought to identify in utero predictors of postnatal outcomes in fetal patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis. Background: Although PAIVS or critical pulmonary stenosis can be diagnosed in utero by echocardiography, our ability to predict outcomes is limited. Methods: Fetal echocardiograms from 28 patients with PAIVS/critical pulmonary stenosis were retrospectively reviewed. Tricuspid valve (TV) annulus, right and left ventricular internal dimensions, and degree of tricuspid regurgitation were recorded. To establish normal fetal values, echocardiograms from healthy patients were analyzed in an identical fashion. Results: Both a fetal TV z score of -4 or less beyond 23 weeks of gestation and a fetal TV annulus of 5 mm or less beyond 30 weeks of gestation were predictive of poor postnatal outcomes. In addition, right:left ventricular length or width less than 0.5 and/or the absence of tricuspid regurgitation were predictive of poor outcome. Conclusions: TV annulus size, right:left ventricular ratios, and presence of tricuspid regurgitation on fetal echocardiograms may aid in guiding prenatal counseling regarding postnatal outcome in PAIVS.