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

Status: Recruiting
Location: See all (2) locations...
Intervention Type: Other
Study Type: Observational
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

Eligibility
Participation Requirements
Sex: All
Minimum Age: 3 months
Maximum Age: 6 months
Healthy Volunteers: f
View:

• 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.

Locations
Other Locations
Spain
Hospital Universitario 12 de Octubre
RECRUITING
Madrid
Sweden
University of Gothenburg
ACTIVE_NOT_RECRUITING
Gothenburg
Contact Information
Primary
Cecilia Villalain, MD PhD
cecilia.villalain@salud.madrid.org
+34913908310
Backup
Alberto Galindo, MD PhD
agalindo@salud.madrid.org
+34913908310
Time Frame
Start Date: 2024-02-01
Estimated Completion Date: 2029-12-31
Participants
Target number of participants: 150
Treatments
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.
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.
Sponsors
Leads: Hospital Universitario 12 de Octubre
Collaborators: Instituto de investigación Hospital 12 de Octubre, Instituto de Salud Carlos III

This content was sourced from clinicaltrials.gov