Prognostic Model of Postnatal Circulation in Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum

Pulmonary atresia (PA)/critical stenosis (CS) with intact ventricular septum (PA/CS-IVS) is a rare congenital heart disease (CHD), that presents heterogeneously. Prognosis is conditioned by the possibility of achieving a primary repair with biventricular circulation (BV) or a one-and-a-half ventricle solution vs. a palliative approach bound to a univentricular (UV) circulation in which both survival and quality of life are significantly impaired. Predicting UV circulation prenatally is still a challenge. The aim of this study is: 1/ to evaluate the natural history of the disease and develop a prognostic model for the prediction of transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age 2/ To develop a model to predict the risk of right ventricle dependent coronary circulation 3/ To evaluate prenatal and postnatal outcomes in non-intervened fetuses with a confirmed postnatal diagnosis of PA-CS/IVS including Intrauterine death, neonatal/Infant death, number of required postnatal procedures, need for oxygen support, need for cardiac transplantation

NCT Number	NCT07095829
Lead Sponsor	Hospital Universitario 12 de Octubre Collaborators: Instituto de Salud Carlos III, Instituto de investigación Hospital 12 de Octubre
Conditions	Pulmonary Atresia With Intact Ventricular Septum, Congenital Heart Disease, Fetal Cardiac Disorder
Enrollment	150 participants
Start Date	2024-02-01
Primary Completion	2028-02-01 (estimated)
Study Completion	2029-12-31 (estimated)
Updated on ClinicalTrials.gov	2025-09-15