An observational study in Pulmonary Atresia With Intact Ventricular Septum, Congenital Heart Disease and Fetal Cardiac Disorder, sponsored by Hospital Universitario 12 de Octubre. Recruiting at 2 sites in 2 countries. Open to participants aged 16 Weeks to 28 Weeks. Per ClinicalTrials.gov, last updated 2025-09-15.
Sponsored by Hospital Universitario 12 de Octubre · Observational
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
This is an international retrospective and prospective observational cohort study including non-intervened fetuses with a diagnosis of PA-CS/IVS between 16+0 and 28+6 weeks of gestation.
A baseline fetal ultrasound examination will be recorded for all included cases. The primary aim of the study is to develop a prognostic and validate model for the type of postnatal circulation (transplantation-free survival with a biventricular or a one-and-a-half repair at 2 years postnatal age vs univentricular palliation or transplanted).
In order to maximize the number of included cases in this rare entity, the model will be developed on retrospective data from 2013 until 2023 and validated in a prospective cohort. Cases will be recruited in referral centers regardless of their offering of fetal pulmonary valvuloplasty. However, only non-prenatally-intervened cases will be used for analysis. Fetal and postnatal echocardiographic examinations will be reviewed by a core laboratory to confirm eligibility for inclusion and identify potential measurement errors.
Data from at least one fetal echo (the first diagnostic evaluation at the referral center) and one postnatal (the first one performed after birth) will be collected, each containing a comprehensive set of two-dimensional and Doppler measurements. Additionally, one additional follow-up scan performed at least 8 weeks apart from the baseline diagnostic scan (ideally between 28 and 32 weeks) will be included in the study as well. Analysis of the prenatal change of dimensions of the right heart structures and selected hemodynamic parameters will enable comparisons between centers regardless of off-protocol center-specific postnatal treatment policies.
1,007 studies on the registry are indexed under Heart Defects, Congenital; 257 are open to participants now.
This study's planned enrollment of 150 is above the median of 121 across 442 observational studies indexed under Heart Defects, Congenital.
Browse Heart Defects, Congenital studies →Hospital Universitario 12 de Octubre is the lead sponsor of 33 studies on the registry; 15 are open to participants now.
Counted across the registry records on this site, refreshed daily.
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.
Exclusion Criteria:
Retrospective cases that meet all the inclusion criteria and none of the exclusion criteria from 2013 until 2023 will be included for model development. They should be non-intervened cases in order to assess the natural history of the disease.
Other: No intervention
Prospectively recruited cases will be included when meeting all the inclusion criteria and none of the exclusion ones. This cohort will serve as a validation cohort for the previously developed model. The nature of the study is observational. Clinicians will not be given the result of the model during the study and clinical practice will be routine.
Other: No intervention
Evaluation 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
Time frame: 3 years (1 recruitment, 2 follow-up)
Prediction of coronary circulation
Prediction of right ventricle-dependent coronary circulation confirmed by cath or autopsy (yes/no)
Time frame: 3 years (1 recruitment, 2 follow-up)
Intrauterine death
Intrauterine death (yes/no)
Time frame: 3 years (1 recruitment, 2 follow-up)
Neonatal/Infant death in the first 2 years
Neonatal/Infant death in the first 2 years (yes/no)
Time frame: 3 years (1 recruitment, 2 follow-up)
Number of required postnatal surgical and catheter interventional procedure
Number of required postnatal surgical and catheter interventional procedures
Time frame: 3 years (1 recruitment, 2 follow-up)
Type of required postnatal surgical and catheter interventional procedures
Type of required postnatal surgical and catheter interventional procedures
Time frame: 3 years (1 recruitment, 2 follow-up)
O2 saturation at 30 days (%)
O2 saturation at 30 days (%)
Time frame: 3 years (1 recruitment, 2 follow-up)
Transplantation in the first two years
Transplantation in the first two years (yes/no)
Time frame: 3 years (1 recruitment, 2 follow-up)
Documents are hosted by the registry — open the source record to download them.
Plan to share: No
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Pulmonary Atresia with Intact Ventricular Septum
Hospital Universitario 12 de Octubre