Altun Gürkan, Babaoğlu Kadir, Binnetoğlu Köksal, Kavas Nazan, Arısoy Ayşe Engin
Department of Pediatric Cardiology, Kocaeli University, Medical Faculty, Kocaeli, Turkey.
Ann Pediatr Cardiol. 2013 Jan;6(1):83-6. doi: 10.4103/0974-2069.107243.
Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.
功能性肺动脉闭锁的特征是肺动脉瓣结构正常，但在右心室射血时不开放。它通常与埃布斯坦畸形、乌尔畸形、新生儿马方综合征和三尖瓣发育异常相关。然而，解剖结构正常的新生儿中功能性肺动脉闭锁很少见。我们报告一例功能性肺动脉闭锁的新生儿，其心脏内解剖结构正常，对一氧化氮和其他血管扩张剂治疗反应良好。
