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.
Screening data: All pregnant women with a fetus with suspected PA-CS/IVS will be screened for eligibility. If they meet all the inclusion criteria and none of the exclusion ones, they will be offered to participate. A complete count of screened, eligible, and included participants will be detailed in the final report of the study. Eligibility: As described in the next section, cases must meet all the inclusion criteria, and none of the exclusion ones.
Cohort
Prospective
No intervention
Other interventionEvaluation of natural history of the disease
Prediction of postnatal circulation
Transplantation-free survival with a non-univentricular circulation (biventricular or a one-and-a-half repair) at 2 years postnatal age: yes/no
Prediction of coronary circulation
Prediction of right ventricle-dependent coronary circulation confirmed by cath or autopsy (yes/no)
Intrauterine death
Intrauterine death (yes/no)
Neonatal/Infant death in the first 2 years
Neonatal/Infant death in the first 2 years (yes/no)
Number of required postnatal surgical and catheter interventional procedure
Number of required postnatal surgical and catheter interventional procedures
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Hospital Universitario 12 de Octubre
Lead sponsor
Instituto de Salud Carlos III
Collaborator
Instituto de investigación Hospital 12 de Octubre
Collaborator
