Type A interrupted aortic arch and type III aortopulmonary window with anomalous origin of the right pulmonary artery from the aorta

Binsalamah, Ziyad M.; Greenleaf, Christopher E.; Heinle, Jeffrey S.

doi:10.1111/jocs.13717

Cited by 8 publications

(5 citation statements)

References 10 publications

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“…Complications mainly presented as great vessel narrowing and residual defects. Most patients (58.6%) suffered from RPA stenosis as a result of either compression by the aorta or excessive tension on the pulmonary anastomosis [53]. In the reports we reviewed, 44.8% and 3.4% of the patients had stenosis at the aortic arch and residual APW, respectively, which accords with the findings of a previous study [6].…”

Section: Discussionsupporting

confidence: 88%

“…A closed ductus arteriosus or a constricting PDA in a patient with Berry syndrome may predict a poor prognosis. Thus, many patients receive prostaglandin E2 to maintain an open ductus arteriosus until further treatment [8,47,51,53]. On review, more than half of patients diagnosed within 3 months present with a large PDA, while patients diagnosed after 3 months all present with a large PDA.…”

Section: Discussionmentioning

confidence: 99%

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Berry syndrome: a case report and literature review

Liu

et al. 2021

BMC Cardiovasc Disord

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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.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Berry syndrome: a case report and literature review

Liu

et al. 2021

BMC Cardiovasc Disord

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“…Ideally, the reconstructed aorta and the PA can both keep growing. However, as seen in our center together with the long-term follow-up in other literature ( 10 ), post-operative RPA stenosis is still the most common complication, owing to the difficulty in reconstruction and the compression due to a limited space next to the AAO. Bi et al ( 1 ) found that many patients (58.6%, 17/29) suffered from post-operative RPA stenosis, and seven patients (41%, 7/17) underwent reinterventions, including five patients were performed surgical repair and three patients were performed balloon angioplasty.…”

Section: Discussionmentioning

confidence: 55%

“…Hemodynamically, the intra-aortic patch was pushed toward the PA owing to the pressure gradient between AAO and PA, which might cause RPA stenosis or even LPA stenosis and the potential problem of the left ventricular outflow tract. Binsalamah et al ( 10 ) reported a case with type A of IAA and type III of APW showing a mild post-operative RPA stenosis by the technique similar to Park et al ( 15 ). Ando et al ( 9 ) also reported a similar case like Binsalamah but underwent two-stage surgical repair, and manifested that the maximal flow velocity of the aorta was 1.1 m/s and both RPA and LPA were 2.0 m/s.…”

Section: Discussionmentioning

confidence: 88%

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

Shi

Weng

Jin

et al. 2022

Front. Cardiovasc. Med.

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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.

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“…The anomalous origin of the RPA is often combined with aortopulmonary window (AP window), aortic arch dissection [6,7], pulmonary aneurysm, pulmonary hypertension or heart failure [8]. In this study, for case with RPA originating from the ascending aorta, one case was combined with coarctation of the aorta, another one combined with VSD, tricuspid atresia, pulmonary valve atresia, visceral inversion and right isomerism, and the rest were not found to have been combined with other malformations.…”

Section: Anomalous Origin Of Unilateral Pulmonary Arterymentioning

confidence: 79%