# Technical considerations and long-term outcomes of successful one-stage repair of Berry syndrome in a preterm neonate using deep hypothermic circulatory arrest: A case report

## TL;DR

A preterm neonate with a rare heart condition called Berry syndrome successfully underwent a complex one-stage surgical repair, offering hope for similar cases.

## Contribution

First successful single-stage surgical repair of Berry syndrome in a preterm neonate using deep hypothermic circulatory arrest.

## Key findings

- Surgical correction of Berry syndrome in a 34+4 weeks gestational age neonate was successfully performed on day 4 of life.
- Use of deep hypothermic circulatory arrest enabled precise repair of complex cardiac anomalies.
- Multidisciplinary care and delayed sternal closure were critical for a favorable outcome in this preterm patient.

## Abstract

Berry syndrome is a rare congenital cardiac anomaly characterized by aortopulmonary window, aortic origin of the right pulmonary artery, interrupted aortic arch, interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and intact ventricular septum.

We report a case of a preterm neonate with Berry syndrome who underwent successful surgical correction. The patient, a 34+4 weeks gestational age male, was diagnosed prenatally with complex congenital heart disease and genetic abnormalities. Postnatal echocardiography confirmed Berry syndrome. Surgical intervention was performed on the fourth day of life, involving deep hypothermic circulatory arrest for aortic window repair, ductal ligation, and aortic arch reconstruction.

Berry syndrome poses significant challenges due to its complex anatomy and the need for early surgical intervention. The use of deep hypothermic circulatory arrest allows for a bloodless field and precise repair of the cardiac anomalies. Prolonged mechanical ventilation and delayed sternal closure are often necessary in preterm neonates due to their compromised cardiopulmonary reserve.

Early diagnosis and timely surgical intervention are crucial for the survival of neonates with Berry syndrome.

•First report of successful Berry syndrome repair in a preterm neonate (34+4 weeks GA)•Definitive single-stage surgical correction performed on day 4 of life using deep hypothermic circulatory arrest.•Surgical techniques included aortopulmonary window patch closure, PDA ligation, and aortic arch reconstruction.•Multidisciplinary team management and delayed sternal closure were crucial for successful outcome despite prematurity.•Case adds to limited literature, demonstrating favorable results possible with early diagnosis and meticulous intervention.

First report of successful Berry syndrome repair in a preterm neonate (34+4 weeks GA)

Definitive single-stage surgical correction performed on day 4 of life using deep hypothermic circulatory arrest.

Surgical techniques included aortopulmonary window patch closure, PDA ligation, and aortic arch reconstruction.

Multidisciplinary team management and delayed sternal closure were crucial for successful outcome despite prematurity.

Case adds to limited literature, demonstrating favorable results possible with early diagnosis and meticulous intervention.