Prenatal Diagnosis and Management of Berry Syndrome, a Rare Conotruncal Anatomy.

Abstract

Berry syndrome is a rare cardiac malformation made up of aortopulmonary window, interrupted aortic arch, aortic origin of right pulmonary artery, and intact interventricular septum.1 Prenatal diagnosis has the potential to improve outcomes with prompt initiation of prostaglandin E1 infusion and early surgery, thereby avoiding pulmonary overcirculation and heart failure. A 26-year-old primigravida presented to the fetal cardiology clinic at 23 weeks’ gestation with a diagnosis of vasa previa, suspected congenital heart disease, and inability to visualize the aortic arch clearly on the obstetric ultrasound. A fetal echocardiogram at 25 weeks’ gestation demonstrated the constellation of Berry syndrome (Figure, A and Movie I in the online-only Data Supplement). The mother delivered a 2-kg female infant at 35 weeks as a result of premature rupture of membranes …