Current outcomes of one-stage surgical correction for Berry syndrome.

OBJECTIVES: Berry syndrome is a combination of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, and interrupted aortic arch. We present here our current experience of primary repair of this syndrome with the goal of optimizing treatment for this rare condition.

METHODS: From January 2003 through December 2015, 16 infants with Berry syndrome underwent one-stage repair at Shanghai Children's Medical Center. Three different surgical correction techniques were used to repair the APW and aortic origin of the RPA, including intra-aortic baffle in 5, RPA detachment in 6, and RPA angioplasty with aortic cuff in 5 patients.

RESULTS: The median age at repair was 90.5 days (range, 8-170 days). The interrupted aortic arch morphology was type A in 14 and type B in 2 patients. The APW morphology was type IIa in 4, type IIb in 10, and type III in 2 patients. Hospital death occurred in 2 patients, and death at follow-up occurred in one other patient. Three patients who previously underwent RPA angioplasty with aortic cuff required reoperation for aortic or RPA stenosis. Freedom from reoperation was 84.8%, 75.4%, and 75.4%, respectively at 1, 5, and 10 years after surgery.

CONCLUSIONS: One-stage repair of Berry syndrome has achieved acceptable outcomes. Reoperations mainly are related to aortic or RPA stenosis, and the reoperation rate is higher when RPA arterioplasty is performed with an aortic cuff.