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

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Total Enrollment

150 participants

Study Classification

OBSERVATIONAL

Study Start Date

2024-02-01

Study Completion Date

2029-12-31

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

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

Related Clinical Trials

Explore similar clinical trials based on study characteristics and research focus.

Fontan Circulation and Cardiopulmonary Function: Prognostic Factors, Hemodynamics and Long-term Effects

NCT02378857

Congenital Heart Disease

COMPLETED

Mild Valvar Pulmonary Stenosis

NCT00277927

Congenital Disorders

TERMINATED

Biomarker Study for Heart Failure in Children With Single Ventricle Physiology

NCT00571233

Tricuspid Atresia Hypoplastic Left Heart Syndrome Unbalanced AV Canal

COMPLETED

Pulmonary Resistance in Fontan Who Have Undergone Heart Transplantation

NCT00268047

Congenital Disorders

TERMINATED

Review of Descending Aortic Flow Reversal in Total Anomalous Pulmonary Venous Connection

NCT00486070

Congenital Disorders

TERMINATED

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

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.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

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

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

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.

No intervention

Intervention Type OTHER

Evaluation of natural history of the disease

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.

No intervention

Intervention Type OTHER

Evaluation of natural history of the disease

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

No intervention

Evaluation of natural history of the disease

Intervention Type OTHER

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

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.

Minimum Eligible Age

16 Weeks

Maximum Eligible Age

28 Weeks

Eligible Sex

ALL

Accepts Healthy Volunteers

No