Andelfinger G, Shirali G S, Raunikar R A, Atz A M
Medical University of South Carolina, 165 Ashley Avenue, Post Office Box 250915, Charleston, SC 29425, USA.
Pediatr Cardiol. 2001 Nov-Dec;22(6):525-6. doi: 10.1007/s002460010290. Epub 2001 Dec 4.
Functional pulmonary atresia is characterized by a structurally normal pulmonary valve not opening during right ventricular ejection. We report this rare condition in a premature newborn of a twin pregnancy, in which fetal echocardiography findings were consistent with critical pulmonary stenosis. After birth, features of neonatal Marfan's syndrome were noted. Echocardiography showed a morphologically normal but immobile pulmonary valve with continuous regurgitation. Right ventricular pressure was subsystemic. In this case, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pulmonary valve atresia may be difficult. The echocardiographic criteria are discussed.
功能性肺动脉闭锁的特征是在右心室射血期间结构正常的肺动脉瓣未开放。我们报告了一例双胎妊娠早产新生儿中的这种罕见情况，其胎儿超声心动图结果与严重肺动脉狭窄一致。出生后，发现有新生儿马方综合征的特征。超声心动图显示形态正常但固定不动的肺动脉瓣伴有持续性反流。右心室压力低于体循环压力。在这种情况下，一氧化氮初始治疗，随后药物性导管闭合治疗取得成功。区分解剖性和功能性肺动脉瓣闭锁可能困难。本文讨论了超声心动图标准。
