NCT07095829 Prognostic Model of Postnatal Circulation in Pulmonary Atresia-critical Stenosis With Intact Ventricular Septum
| NCT ID | NCT07095829 |
| Status | Recruiting |
| Phase | — |
| Sponsor | Hospital Universitario 12 de Octubre |
| Condition | Pulmonary Atresia With Intact Ventricular Septum |
| Study Type | OBSERVATIONAL |
| Enrollment | 150 participants |
| Start Date | 2024-02-01 |
| Primary Completion | 2028-02-01 |
Trial Parameters
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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
Eligibility Criteria
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.