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

Sponsor
Hospital Universitario 12 de Octubre
Study ID
NCT07095829
Status
Recruiting
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Conditions

  • Congenital Heart Disease
  • Fetal Cardiac Disorder
  • Pulmonary Atresia With Intact Ventricular Septum

Eligibility Criteria

Sex
ALL
Age
16 Weeks - 28 Weeks
Healthy Volunteers
Not accepted

Interventions

  • No intervention — OTHER
    Evaluation of natural history of the disease

Study Details

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

Key Dates

First listed
Jul 31, 2025
Start date
Feb 1, 2024
Status verified
Sep 2025
Primary completion
Feb 1, 2028
Completion
Dec 31, 2029

Study Design

Enrollment
150 participants (estimated)

Arms

  • Arm: Retrospective cohort
    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.
  • Arm: Prospective cohort
    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.

Primary Outcome Measure

Prediction of postnatal circulation [ Time Frame: 3 years (1 recruitment, 2 follow-up) ]

Central Contacts

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