A rare case of truncus arteriosus Van Praagh type A3: Prenatal diagnosis and postnatal management

Abstract

Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C–E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks’ gestation. TrA C–E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks’ gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management.<Learning objective: Delayed diagnosis of truncus arteriosus (TrA) Van Praagh (VP) type A3 can cause unilateral pulmonary artery disconnection; thus, we should consider both Collett and Edwards and VP classifications, including a ductus arteriosus contralateral to the aortic arch, during TrA diagnosis. Furthermore, to prevent the risks associated with an open-heart surgery in the neonatal period in such cases, pulmonary blood flow can be controlled by maintaining the ductus arteriosus with prostaglandin E1.alpha-cyclodextrin and unilateral pulmonary artery banding.>