Pulmonary Atresia -Critical Stenosis with Intact Ventricular Septum: An International Survey

Pulmonary atresia/critical stenosis with intact ventricular septum is a heterogeneous disease in which perinatal management is not exempt from controversies. We aim to understand which areas are the ones with the highest and least consensus. An online questionnaire including 30 questions was administered through a REDCap link. Invitations were sent out through the Fetal Heart Society and personal emails to the heads of fetal cardiology programs. Results were analyzed in a descriptive manner and compared among the regions with most responses (North America and Europe). A total of 72 specialists in fetal and pediatric cardiology responded, most from North-America (n = 31) and Europe (n = 29). Antenatal counselling was mainly based on the tricuspid valve z-score (43.7%), although among European responders, multivariable models were preferred (44.8%). There was a wide regional variation in the nomograms used for prenatal assessment of the tricuspid valve. More than half did not consider prenatal valvuloplasty (54.3%), and those who did diverged in selection criteria. Postnatally, in mild-moderately hypoplastic right ventricle, most opted to first assess the tricuspid valve z-score (60%) whereas others would always attempt a biventricular pathway (30.8%). The majority opted to perform cardiac catheterization in all cases to assess ventriculo-coronary connections (60.6%). If right ventricle-dependent coronary circulation was confirmed, 66.7% would opt for single ventricle palliation, whereas 15.9% would directly list for transplant. There is great heterogeneity in the management of pulmonary atresia-critical stenosis with intact ventricular septum, particularly in prenatal assessment and consideration for prenatal intervention.