Predicting postnatal circulation in pulmonary atresia with intact ventricular septum
Development and Validation of a Prognostic Model of Postnatal Circulation in Fetuses With a Diagnosis of Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum A Prospective Observational Cohort Study
This project will try to use fetal ultrasound measurements to see if doctors can predict which babies with pulmonary atresia and an intact ventricular septum will have a biventricular/one-and-a-half repair versus need univentricular palliation by age 2.
Quick facts
| Study type | Observational |
|---|---|
| Enrollment | 150 (estimated) |
| Ages | 16 Weeks to 28 Weeks |
| Sex | All |
| Sponsor | Hospital Universitario 12 de Octubre Academic / other |
| Locations | 2 sites (Madrid and 1 other locations) |
| Trial ID | NCT07095829 on ClinicalTrials.gov |
What this trial studies
This is an international observational cohort that combines retrospective cases (2013–2023) with a prospective validation cohort to develop a prenatal prognostic model. Non-intervened fetuses diagnosed between 16+0 and 28+6 weeks with PA/CS-IVS will be included and baseline and follow-up fetal echocardiograms plus the first postnatal echo will be collected. All echocardiographic studies will be reviewed centrally by a core laboratory to confirm eligibility and standardize measurements. The primary outcome is transplantation-free survival with a biventricular or one-and-a-half repair at 2 years versus univentricular palliation or transplantation, and the study will also model risk of right-ventricle-dependent coronary circulation.
Who should consider this trial
Good fit: Ideal candidates are fetuses diagnosed at a participating referral center between 16+0 and 28+6 weeks with pulmonary atresia or critical pulmonary stenosis and an intact ventricular septum, without major extracardiac anomalies or known genetic syndromes, and who did not undergo prenatal pulmonary valvuloplasty.
Not a fit: Patients who had prenatal pulmonary valvuloplasty, terminated pregnancies, poor-quality or incomplete imaging, postnatally unconfirmed diagnoses, or significant associated cardiac or extracardiac anomalies are unlikely to benefit from or be included in the predictive model.
Why it matters
Potential benefit: If successful, the model could give families and clinicians clearer prenatal prognoses to guide counseling, delivery planning, and early postnatal management.
How similar studies have performed: There are single-center reports and surgical series describing outcomes and some predictors, but comprehensive, externally validated prenatal models for predicting univentricular versus biventricular circulation in PA/CS-IVS remain limited.
Eligibility criteria
Show full inclusion / exclusion criteria
Inclusion Criteria: * 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. Exclusion Criteria: * 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. * Functional PA-CS/IVS (Ebstein malformation, monochorionic twins) * Any associated cardiac defect except persistent left superior vena cava and aberrant right subclavian artery. * Any significant (i.e that might influence outcome) extracardiac anomaly and/or known genetic syndromes. Also, if such a condition is present at inclusion but diagnosed only after birth, the case will be retrospectively excluded.
Where this trial is running
Madrid and 1 other locations
- Hospital Universitario 12 de Octubre — Madrid, Spain (Recruiting)
- University of Gothenburg — Gothenburg, Sweden (Active_not_recruiting)
Study contacts
- Principal investigator: Cecilia Villalaín González, MD PhD — Instituto de investigación imas12
- Study coordinator: Cecilia Villalain, MD PhD
- Email: cecilia.villalain@salud.madrid.org
- Phone: +34913908310
How to participate
- Review the eligibility criteria above with your treating physician.
- Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
- Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.