Pinsky W W, Nihill M R, Mullins C E, Harrison G, McNamara D G
Circulation. 1978 Jan;57(1):159-62. doi: 10.1161/01.cir.57.1.159.
The absent pulmonary valve syndrome consists of a severely hypoplastic pulmonary valve with anular stenosis, aneurysmal dilatation of the main pulmonary artery with one or both pulmonary branches also dilated, and a ventricular septal defect. From 1955 to 1975, 15 patients were seen at Texas Children's Hospital with this syndrome. Although anatomically similar to tetralogy of Fallot, the most significant symptoms during early infancy were secondary to bronchial compression resulting from the dilated pulmonary arteries and enlarged left atrium. Intracardiac repair performed on two infants at four months of age failed to ameliorate the respiratory symptoms and both died. Seven patients from 2.3 to 16 years of age were operated upon, with one late postoperative death. Five other patients have not yet received surgical intervention. We recommend vigorous continual respiratory therapy for for infants and small children and intracardiac repair in older children since repair in infancy does not relieve respiratory symptoms. We do not recommend a prosthetic pulmonary valve unless there is persistently elevated main pulmonary artery pressure.
肺动脉瓣缺如综合征包括严重发育不全的肺动脉瓣伴瓣环狭窄、主肺动脉瘤样扩张且一支或两支肺分支也扩张，以及室间隔缺损。1955年至1975年期间，德克萨斯儿童医院共诊治了15例患有该综合征的患者。尽管在解剖结构上与法洛四联症相似，但婴儿早期最显著的症状继发于扩张的肺动脉和增大的左心房对支气管的压迫。对两名4个月大的婴儿进行的心内修复未能改善呼吸症状，两人均死亡。对7名年龄在2.3岁至16岁的患者进行了手术，术后有1例晚期死亡。另外5名患者尚未接受手术干预。我们建议对婴幼儿进行积极持续的呼吸治疗，对大龄儿童进行心内修复，因为婴儿期修复并不能缓解呼吸症状。除非主肺动脉压力持续升高，否则我们不建议使用人工肺动脉瓣。
