Berry syndrome—a rare congenital cardiac anomaly

Haranal, Maruti; Srimurugan, Balaji; Dinh, Duyen Mai; Sivalingam, Sivakumar

doi:10.1007/s12055-021-01206-0

Cited by 4 publications

(3 citation statements)

References 31 publications

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“…Most centers pursued one-stage surgical correction, but staged repair should be considered in premature or small infants. [ 6 ] However, there is still a lack of data reporting outcomes of late-presenter Berry syndrome, especially in infants more than 6 months old.…”

Section: Discussionmentioning

confidence: 99%

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

Tandayu,

Kurniawati,

Atmosudigdo

et al. 2023

Annals of Pediatric Cardiology

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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.

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Section: Discussionmentioning

confidence: 99%

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

Tandayu,

Kurniawati,

Atmosudigdo

et al. 2023

Annals of Pediatric Cardiology

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“…Failure of posterior truncal septation may disrupt normal blood flow and cause the pulmonary bifurcation to attach incorrectly to the unseptated truncus arteriosus instead of connecting to the main pulmonary artery. As a result, the RPA becomes connected to the aorta, while the LPA becomes connected to the pulmonary trunk [8]. According to research by Bi et al, the majority of Berry syndrome patients have type II APW and type A IAA [2].…”

Section: -Discussionmentioning

confidence: 99%

A case of Berry syndrome: a rare congenital cardiac structural abnormality with one-stage surgical repair

Ma,

Wen,

et al. 2023

Preprint

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Berry syndrome is a complex congenital heart anomaly characterized by a combination of aortopulmonary window, interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, anomalous origin of the right pulmonary artery, patent ductus arteriosus, and intact ventricular septum. It is an extremely rare condition, with approximately 100 reported cases to date. In this article, we report a case of a 6-year-old girl with Berry syndrome who presented with significant ischemic and hypoxic symptoms. She underwent a one-stage surgical treatment and had a successful discharge.

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“…The aortic origin of the RPA results in early pulmonary overcirculation due to an obligatory left-to-right shunt. Simultaneously, the perfusion of the lower body organs is entirely dependent on a patent ductus arteriosus due to an IAA 2. Early clinical management is pivotal and includes diuretics and positive pressure ventilation to address the pulmonary congestion, as well as, when necessary, maintaining the arterial ductus patent with prostaglandin E1 infusion.…”

Section: Descriptionmentioning

confidence: 99%