Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.
Department of Perinatology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.
Echocardiography. 2022 Oct;39(10):1359-1362. doi: 10.1111/echo.15446. Epub 2022 Sep 20.
Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20-week-old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20-week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS.
肺动脉瓣缺如综合征（APVS）是一种罕见的先天性心脏畸形。它常与法洛四联症相关，但也可能与完整的室间隔相关，或更罕见地与三尖瓣闭锁相关。它与完全性房室间隔缺损和右心室双出口的联合非常罕见，即使在出生后也是如此。在此，我们报告一例 20 周胎儿，存在右位主动脉弓和左锁骨下动脉异常，这种罕见的组合。我们报告一例 20 周胎儿，诊断为右位主动脉弓和左锁骨下动脉异常的罕见组合。伴有完全性房室间隔缺损的 APVS 可能代表另一种类型的 APVS。
