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

Key facts

Where
Gothenburg, SwedenSee the site

Where in Sweden

  • University of GothenburgGothenburg, SwedenContact: Cecilia Villalain, MD PhDNo longer taking part

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Trial details

Who runs it
Hospital Universitario 12 de Octubre
Trial code
NCT07095829
Record updated
15 September 2025
Original description
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.Read full original descriptionShow less

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

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

Inclusion: 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: 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; 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).

ClinicalTrials.gov NCT07095829