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

NCT07095829 | Recruiting DMC

• 1 other identifier: PROGPAIVS
• Study Type: observational
• Target: 150
• Locations: 2 countries
• Sites: 2

Brief Summary

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

Conditions

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

Keywords

Prognosis; Prenatal diagnosis; Ultrasound

Outcome Measures

Primary Outcomes (1)

• 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

  3 years (1 recruitment, 2 follow-up)

Secondary Outcomes (7)

• Prediction of coronary circulation

  3 years (1 recruitment, 2 follow-up)

• Intrauterine death

  3 years (1 recruitment, 2 follow-up)

• Neonatal/Infant death in the first 2 years

  3 years (1 recruitment, 2 follow-up)

• Number of required postnatal surgical and catheter interventional procedure

  3 years (1 recruitment, 2 follow-up)

• Type of required postnatal surgical and catheter interventional procedures

  3 years (1 recruitment, 2 follow-up)

Study Arms (2)

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.

Other: No intervention

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.

Other: No intervention

Interventions

No intervention OTHER

Evaluation of natural history of the disease

Prospective cohort; Retrospective cohort

Eligibility Criteria

• Age: 16 Weeks - 28 Weeks
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)
• Sampling Method: Non-Probability Sample

Study Population

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.

You may qualify if:

• 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.

You may not qualify if:

• 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.

Sponsors & Collaborators

• Hospital Universitario 12 de Octubre: lead
• Instituto de Salud Carlos III: collaborator
• Instituto de investigación Hospital 12 de Octubre: collaborator

Study Sites (2)

Hospital Universitario 12 de Octubre

Madrid, 28041, Spain

RECRUITING

University of Gothenburg

Gothenburg, Sweden

ACTIVE NOT RECRUITING

MeSH Terms

Conditions

Pulmonary Atresia with Intact Ventricular Septum; Heart Defects, Congenital

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Cecilia Villalaín González, MD PhD

  Instituto de investigación imas12

  PRINCIPAL INVESTIGATOR

• Alberto Galindo Izquierdo, MD PhD

  Instituto de investigación imas12

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Cecilia Villalain, MD PhD

CONTACT

+34913908310; cecilia.villalain@salud.madrid.org

Alberto Galindo, MD PhD

CONTACT

+34913908310; agalindo@salud.madrid.org

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: March 18, 2024
• First Posted: July 31, 2025
• Study Start: February 1, 2024
• Primary Completion (Estimated): February 1, 2028
• Study Completion (Estimated): December 31, 2029
• Last Updated: September 15, 2025

Record last verified: 2025-09

Data Sharing

• IPD Sharing: Will not share

Locations

ES (1); SE (1)