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
Absence of flow at the pulmonary valve (PA) or presence of thickened and domed. pulmonary valve cusps with a pinhole jet of flow.
Doppler evidence of ductal-dependent pulmonary circulation.
Intact ventricular septum.
Poor imaging windows and incomplete/poor quality scan
Termination of pregnancy
Cases initially included that undergo prenatal pulmonary valvuloplasty later on in pregnancy.
Unconfirmed PA-CS/IVS at birth.
Hospital Universitario 12 de Octubre
Lead sponsor
Instituto de Salud Carlos III
Collaborator
Instituto de investigación Hospital 12 de Octubre
Collaborator