Single ventricle: Diagnosis and prognosis

Abstract

Between October 2011 and June 2019 we detected 2787 new heart defects in fetuses in the Referral Centre for Perinatal Cardiology in Warsaw, Poland, Agatowa US Clinic. 393 had univentricular hearts and 144 critical aortic stenosis. The term: SINGLE VENTRICLE is a very wide definition. If the heart is “functionally univentricular” one ventricle is too small or its function is too poor to maintain systemic or pulmonary cardiac output. It is already known that cardiac lesions are dynamic. It is likely that in some circumstances growth of cardiovascular structures depends on the volume of flow. There are hearts which will always be univentricular. There are: tricuspid atresia (67 cases), double inlet left ventricle (31), mitral atresia (9), hypoplastic left heart syndrome (222), and more complicated cardiac defects in which only one ventricle is developed. In such babies final treatment is to obtain Fontan circulation. During prenatal period it is important to plan the perinatal management and to decide what kind of treatment will be necessary in the newborns. Stenosis or hypoplasia of the pulmonary artery or aorta can occur, so the lesion will be ductal dependent. In other - restriction of the foramen ovale can cause the neonatal problem. In rare cases neonates will not need any treatment, if both arteries are well developed. On the other hand there are “evolving lesions”, like critical aortic stenosis (evolving HLHS) or pulmonary atresia and intact ventricular septum. In both we suspect that outflow tract obstructions caused underdevelopment of the ventricle. So fetal opening of the left or right outflow tract should be beneficial. Basing on this theory fetal cardiac interventional program developed. My team performed 90 aortic balloon valvluoplasties in 84 fetuses and 12 pulmonary artery valvuloplasties in 11 fetuses. In the recent period we achieved 48% of biventricular circulation of live born babies with critical aortic stenosis and 90% in pulmonary atresia or critical pulmonary stenosis. Criteria for prenatal intervention are still under development. The main rule is that prenatal cardiac interventions should be performed only if predictive outcome without intervention will be worse than without it. It must be remembered that fetal valvuloplasty in the aortic stenosis is the first step of long and complicated postnatal treatment. In conclusion: univentricular heart can be successfully treated for life long single ventricular physiology. In some lesions natural history can be changed by prenatal cardiac intervention.