Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries,Pediatric Cardiology

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Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries
Pediatric Cardiology ( IF 1.5 ) Pub Date : 2024-02-01 , DOI: 10.1007/s00246-023-03331-6
Justin B Jin ₁ , Joshua D Robinson ₁ , Joseph A Camarda ₁ , Michael B Satzer ₁ , Michael R Carr ₁ , Michael Monge ₁ , Angira Patel ₁

Affiliation

Abstract

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.

中文翻译：

三尖瓣闭锁伴肺动脉瓣缺失且肺动脉分支几乎不连续

抽象的

肺动脉瓣缺失伴三尖瓣闭锁或三尖瓣狭窄 (APV-TA/TS) 是一种极其罕见的先天性心脏病，具有显着的发病率和死亡率。与肺动脉瓣缺失综合征的法洛四联症相比，肺动脉分支通常不显着扩张。我们介绍了一名新生儿男性产前诊断为 APV-TA 的病例，该病例具有完整的室间隔 (IVS) 和几乎不连续的肺动脉分支、所采用的手术策略以及医疗决策中考虑的显着血流动力学因素。

更新日期：2024-01-27

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