Fetal Cardiac Procedures and Postnatal Outcomes: Experience of a Single Center

Purpose: An analysis of our initial experience in fetal cardiac intervention (FCI) to evaluate procedural Results and postnatal outcome.

Method: retrospective study of FCI. Hypoplastic left heart syndrome with intact atrial septum (HLHS/IAS), aortic valvular stenosis (AVS) with dilated left ventricle and pulmonary atresia with intact septum (PAIS) are diseases that can be treated in utero. Selection criteria for procedures are those published by the Boston group.The study was approved by the ethical committee of the hospital.

Results: Between 2012 and 2014, seven fetuses were selected (four AVS and three HLHS/IAS). Three pregnant women refused the procedure. The intrauterine aortic valvuloplasty were performed in three fetuses with AVS at 24th, 26th, 29th weeks. The procedures were technically successful in two patients. In one case we did not perform the procedure for unfavorable position of the fetus. There were no fetal and maternal complications, except for a case of iatrogenic detachment between the amniotic and the chorionic membranes and an intrauterine clot solved in the next days. After the procedure we observed the growth of aortic annulus with aortic anterograde flow but the development of left ventricle hypoplasia in all patients and a little growth of a dysplastic mitral valve in one patient. Fetuses with successful procedure were born at term, one by caesarean section because of a severe intrauterine growth retardation (1.8 kg) and maternal nephrotic syndrome and the other by induced vaginal delivery (3.8 kg); the third fetus was born preterm at 32 weeks by spontaneous vaginal delivery (1.9 kg). Postnatal outcome was good with stable hemodynamic condition. All patients were treated with univentricular palliation with hybrid procedure or pulmonary artery bibanding with early Norwood as recently introduced in our center. Suddenly death at home was observed in one case, the other two patients are alive in second stage of palliation. A procedure of atrial septoplasty and stenting was performed without fetal-maternal complication in the fetus with HLHS/IAS. There is no displacement of the stent for all the pregnancy with no restrictive flow and improvement of pulmonary vein Doppler. This fetus was delivered at 39 weeks by induced vaginal delivery (3.8 kg). The neonate was very stable at birth, with no need of intubation or further atrial septoplasty procedure.

Conclusion: Our population, nowadays the greatest in Italy, shows that both aortic valvuloplasty ad atrial stenting are procedure feasible in utero without complications and associated with vaginal delivery of a stable chiocciola term neonate. In our patients the technical success of the procedure did not allow a biventricular repair after birth. New prenatal echocardiographic criteria are needed to select patients with more probabilities of biventricular repair, but the small amount of patients did not permit us to find out these criteria. Stenting of interatrial septum in patients with HLHS/IAS has to be considered a save life procedure, improving neonatal survival. A greater amount of patients could permit us to evaluate the effects of successful atrial stenting on pulmonary vasculopathy noted in cases of intact atrial septum. A multidisciplinary approach has been used to coordinate prenatal procedures and optimize postnatal outcomes.