Kevin Moses Hanky Jr Tandayu, Yovi Kurniawati, Indriwanto Sakidjan Atmosudigdo, Oktavia Lilyasari
{"title":"贝里综合征病例系列:一种罕见的致命性心脏畸形。","authors":"Kevin Moses Hanky Jr Tandayu, Yovi Kurniawati, Indriwanto Sakidjan Atmosudigdo, Oktavia Lilyasari","doi":"10.4103/apc.apc_109_23","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":8026,"journal":{"name":"Annals of Pediatric Cardiology","volume":"16 5","pages":"374-377"},"PeriodicalIF":0.9000,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11098299/pdf/","citationCount":"0","resultStr":"{\"title\":\"Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies.\",\"authors\":\"Kevin Moses Hanky Jr Tandayu, Yovi Kurniawati, Indriwanto Sakidjan Atmosudigdo, Oktavia Lilyasari\",\"doi\":\"10.4103/apc.apc_109_23\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":8026,\"journal\":{\"name\":\"Annals of Pediatric Cardiology\",\"volume\":\"16 5\",\"pages\":\"374-377\"},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2023-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11098299/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Annals of Pediatric Cardiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/apc.apc_109_23\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/4/1 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Annals of Pediatric Cardiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/apc.apc_109_23","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/4/1 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
Case Series of Berry syndrome: A rare constellation of fatal cardiac anomalies.
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.