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

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

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.

Conditions4

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