Abstract 18401: Echocardiographic Prediction of Outcomes in Fetal Pulmonary Atresia With Intact Ventricular Septum
Background: Critical pulmonary stenosis (CPS) and pulmonary atresia with an intact ventricular septum (PA-IVS) represent a spectrum of pathology that ranges from good-sized to severely hypoplastic and dysplastic right ventricle (RV), pulmonary valve and tricuspid valve. These conditions are rarely associated with chromosomal or extracardiac anomalies, so parental decision regarding continuation of the pregnancy is strongly influenced by the cardiologist’s prediction of a univentricular or biventricular circulation in midgestation. Currently the prediction of outcome is mainly based on the results of five studies, each proposing a different predictive score based on small patient populations.
Hypothesis: We hypothesized that the previously published echocardiographic scores have limited capacities at predicting the outcome in patient with CPS or PA-IVS when using a larger patient population. Therefore, we aimed to create an improved predictive scoring system based on the published measurements.
Method: We conducted a multicentre retrospective study of all cases of PA-IVS or CPS diagnosed prior to 32 weeks of gestation using a single blinded observer.
Results: A total of 99 pregnancies were included. The termination rate was 36 %. Of 59 live-born patients with intention to treat, 25 had non-biventricular outcome and 32 had biventricular circulation. The survival at 1 year was 100% for biventricular repair, compared with 85% for non-biventricular outcome. The presence of sinusoids was a significant risk factor for mortality but screening was not performed systematically. All scoring systems had significantly lower sensitivities and specifities than previously reported. A small tricuspid valve annulus, a low right ventricle-to-left ventricle lenght ratio and low tricuspid-to-mitral valve ratio, as well as the presence of sinusoids are the most reliable predictors of a non-biventricular outcome.
Conclusions: The outcome of PA-IVS remains difficult to predict. A new scoring system combining the best predicting measurements of each scores is underway. Coronary sinusoids, when present, are associated with a single ventricle outcome with high mortality and should be screened systematically using optimal echocardiographic settings.
Author Disclosures: C. Renaud: None. B. Zoeller: None. S. Levasseur: None. T. Cavalle: None. M. Raboisson: None. L. Hornberger: None. E. Jaeggi: None.
- © 2016 by American Heart Association, Inc.