Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies

Abstract

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome.

Keywords

• aortic origin of the right pulmonary artery
• aortopulmonary window
• berry syndrome
• congenital heart disease
• interrupted aortic arch