VP22.23: Fetal complex rare cardiac malformation

Abstract

A 27-year-old gravida 1 para 0 attended for anomaly scan with a singleton pregnancy. There were no significant events of interest nor consanguinity between both parents. Second-trimester ultrasound scan at 22+3 weeks showed a single live fetus in changing lie. Fetal Echo revealed: Situs solitus. The 4- chamber view showed univentricular heart, left handed ventricular topology, right sided main ventricle of left ventricular morphology, hypoplastic left sided (morphological right) ventricle, left sided AV valve (likely tricuspid valve) atresia, VSD (subaortic), AV & VA discordance - congenitally corrected transposition. Foramen ovale was largely restrictive with significant a-wave reversal. Aorta was located to right and anterior to pulmonary artery - D malposition. Aortic arch hypoplasia, mild preductal aortic coarctation, main pulmonary arterial dilatation, bilateral branch pulmonary artery origin stenosis was noted. No arrhythmias, no disturbances in the heart rate. Fetal cardiac function was normal. Amniocentesis and microarray offered but refused by patient. At 39+3 weeks of gestation a 3.05 kg male infant was born by spontaneous vaginal delivery, required PPV and CPAP at birth, due to poor respiratory effort then recovered. Postnatal echocardiography confirmed the diagnosis and on second day after birth the newborn underwent Norwood surgery with right modified Blalock-Taussig shunt and recovered uneventfully. The second stage surgery (Glenn superior cavo pulmonary shunt) and PA plasty was performed at 6 months of age and the patient recovered uneventfully. The patient will be followed in multidisciplinary fashion along with pediatric cardiac critical care and pediatric cardiology teams. He will proceed along the univentricular pathway, with a Fontan procedure (at a weight of 15 kg or greater) at 3 to 4 years of age, preceded by cardiac catheterisation. He should remain on aspirin (5 mg/kg once daily) until his Fontan (if feasible).