Abstract
BackgroundBerry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. Here, we summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future.MethodsFrom January 2014 to December 2019, three neonates with berry syndrome underwent one-stage surgical repair in our center. We mainly used two different surgical techniques to repair it, and collected clinical data retrospectively from hospitalization history, outpatient records, and telephone follow-up.ResultsThe age at operation was 28, 8, and 8 days and the body weight was 3.65, 3.86, and 3.0 kg, respectively. The morphology of the interrupted aortic arch (IAA) was type A in two patients and type B in one patient. The aortopulmonary window (APW) morphology was type IIa, III, and IIb, respectively. The phenotype of the IAA type B combined with APW type III in our second patient was reported for the first time so far. All patients survived and were followed up to date. The second patient using intra-aortic baffle experienced twice reoperation for right pulmonary artery (RPA) stenosis. All patients grew well so far.ConclusionOnce diagnosed in the neonatal period, berry syndrome can be safely corrected by one-stage surgical repair in experienced cardiac centers. Considering the variability of the location where the RPA arises from the posterior wall of the aorta, it is difficult to find the best surgical method for each patient.
Highlights
Berry syndrome is a rare disease and manifests complex cardiac anomalies involving interrupted aortic arch (IAA) or hypoplastic aortic arch (HAA) or coarctation of the aorta (CoA), aortopulmonary window (APW), the aortic origin of the right pulmonary artery (AORPA), and intact ventricular septum (1)
After incision of main pulmonary artery (MPA), we found that the opening of left pulmonary artery (LPA) was slightly narrowed and the opening of right pulmonary arteries (RPA) was nearly closed
Berry et al (5) described the possible embryogenesis that APW might affect the formation of AORPA, the blood flow through the aortic arch would be reduced, leading to HAA/CoA/IAA
Summary
Berry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. We summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future
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