Berry syndrome: a case report and literature review

Abstract

Background Berry syndrome, a rare combination of cardiac anomalies, consists of aortopulmonary window (APW); aortic origin of the right pulmonary artery; interrupted aortic arch (IAA) or hypoplastic aortic arch or coarctation of the aorta; and an intact ventricular septum. There is lack of review articles that elucidate the clinical features, diagnosis, treatment, and outcomes of Berry syndrome. This publication systematically reviews the 89 cases published since 1982 on Berry syndrome. Case presentation A 38-year-old woman presented with a loud murmur and cyanosis. Transthoracic echocardiography demonstrated a severely dilated aorta and main pulmonary artery with a large intervening defect. Distal to the APW, the ascending aorta gave rise to the right pulmonary artery. Additionally, a type A IAA, an intact ventricular septum, and a large patent ductus arteriosus were revealed. Computed tomography angiography with 3-dimensional reconstruction confirmed above findings. This is the first report of a patient of this age with Berry syndrome who did not undergo surgery. Conclusions Berry syndrome is a rare but well-identified and surgically correctable anomaly. Patients with Berry syndrome should be followed up for longer periods to better characterize long-term outcomes.

Outcomes of One-Stage Surgical Repair for Berry Syndrome in Neonates

“…Timely treatment after birth is very important, because most of the patients die soon after birth while the surviving patients will develop pulmonary hypertension quickly. This complex deformity has been reported in about 100 patients in the English literature since 1982 Berry first described ( 1 ). For unoperated patients, the mortality rate was 100% and the median age at death was 1 month ( 1 ).…”

“…This complex deformity has been reported in about 100 patients in the English literature since 1982 Berry first described ( 1 ). For unoperated patients, the mortality rate was 100% and the median age at death was 1 month ( 1 ). Only a small part of patients (31 cases) was operated at the neonatal period with 6 neonates (6/31, 20%) who died post-operatively ( 1 ).…”

“…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 ). The incidence of this syndrome among patients with congenital heart diseases is 0.046%, according to literature reports ( 2 ).…”

“…For unoperated patients, the mortality rate was 100% and the median age at death was 1 month ( 1 ). Only a small part of patients (31 cases) was operated at the neonatal period with 6 neonates (6/31, 20%) who died post-operatively ( 1 ). It is a great challenge for surgeons to make early diagnosis and successful surgical correction in the neonatal period and to make sure of the long-term survival of the patients.…”

Berry syndrome with bovine aortic arch

“…3 It is more common in males (67.1%), presents commonly before 3 months with one or more clinical symptoms, including cyanosis, respiratory distress, cardiac murmur, symptoms of congestive cardiac failure, and other systemic symptoms. 4 The occurrence of an atrial septal defect in a patient with berry syndrome is extremely rare with only two patients reported out of 68 patients for whom details are available in the literature. 4 There is no prior reported case of the bovine aortic arch with berry syndrome.…”