贝里综合征病例系列:一种罕见的致命性心脏畸形。

IF 0.9 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS Annals of Pediatric Cardiology Pub Date : 2023-09-01 Epub Date: 2024-04-01 DOI:10.4103/apc.apc_109_23
Kevin Moses Hanky Jr Tandayu, Yovi Kurniawati, Indriwanto Sakidjan Atmosudigdo, Oktavia Lilyasari
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引用次数: 0

摘要

贝里综合征是一种极为罕见的先天性心脏畸形,包括主动脉肺窗、右肺动脉主动脉起源(AORPA)、主动脉弓中断或主动脉弓发育不良或主动脉共动脉瘤,以及完整的室间隔,新生儿死亡率很高。这种疾病是致命的,新生儿期死亡率很高(90%),存活的患者大多会出现肺动脉高压。我们描述了两名贝里综合征患者的临床表现和诊断线索。
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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.

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来源期刊
Annals of Pediatric Cardiology
Annals of Pediatric Cardiology CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
1.40
自引率
14.30%
发文量
51
审稿时长
23 weeks
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