Dunnigan A, Oldham H N, Benson D W
Am J Cardiol. 1981 Jul;48(1):117-22. doi: 10.1016/0002-9149(81)90580-4.
Congenital absence of the pulmonary valve leaflets is generally associated with ventricular septal defect, anular pulmonary stenosis and aneurysmal dilatation of the pulmonary arteries: the absent pulmonary valve syndrome. Symptomatic infants with this syndrome suffer primarily from respiratory insufficiency caused by bronchial compression by the dilatated pulmonary arteries, and have a high mortality rate. Asymptomatic infants are thought to do well, and may have elective surgery late in childhood. Findings in four infants with absent pulmonary valve syndrome are reviewed. One symptomatic infant was successfully treated with closure of a ventricular septal defect and pulmonary arterial plication. One previously asymptomatic infant had a fatal respiratory arrest revealing the complexity of management of infants with absent pulmonary valve syndrome.
肺动脉瓣叶先天性缺如通常与室间隔缺损、肺动脉瓣环狭窄及肺动脉瘤样扩张相关，即无肺动脉瓣综合征。患有该综合征的有症状婴儿主要因扩张的肺动脉压迫支气管而出现呼吸功能不全，且死亡率很高。无症状婴儿一般情况良好，可能在儿童期晚期接受择期手术。本文回顾了4例无肺动脉瓣综合征婴儿的病例。1例有症状婴儿通过室间隔缺损修补及肺动脉折叠术成功治愈。1例之前无症状的婴儿发生致命性呼吸骤停，揭示了无肺动脉瓣综合征婴儿治疗的复杂性。
