Lurie Children's Hospital, Chicago, USA.
Pediatr Cardiol. 2024 Feb;45(2):446-451. doi: 10.1007/s00246-023-03331-6. Epub 2023 Nov 13.
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.
三尖瓣闭锁伴或不伴肺动脉瓣缺如（APV-TA/TS）是一种极其罕见的先天性心脏病，与较高的发病率和死亡率相关。与法洛四联症伴肺动脉瓣缺如综合征相比，通常不存在显著扩张的分支肺动脉。我们报告了一例新生儿男性产前诊断为 APV-TA 伴完整室间隔（IVS）和近乎不连续的分支肺动脉的病例，介绍了所采用的手术策略，以及在医疗决策中考虑的主要血流动力学因素。
