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肺动脉瓣缺如综合征包括肺动脉瓣发育不良、瓣环狭窄和肺动脉瓣关闭不全、肺动脉及其分支呈瘤状扩张.此综合征常伴膜部室间隔缺损和右心室肥厚.本文报道1976～1983年间19例手术治疗结果.病儿年龄5天到11岁,其中9例婴儿<1岁,根据临床症状和采用非侵入性和侵入性检查进行诊断.婴儿主要为呼吸道症状,如肺部感染、支气管痉挛或呼吸衰竭,均需用狄戈辛和利尿剂,但内科治疗效果不好.>1岁组经常患呼吸道感染(7/10),运动耐量限止(5/10),生长发育迟缓(4/10).7例术前采用两维超声心动图,18例术前进行心导管检查,发现有室间隔缺损、肺动脉瓣缺如、肺动脉瓣关闭不全、主肺动脉和一侧或两侧肺动脉分支呈动脉瘤样
Pulmonary valve disease syndrome, including pulmonary dysplasia, valvular stenosis and pulmonary insufficiency, pulmonary artery and its branches were tumor expansion.This syndrome is often associated with membranous ventricular septal defect and right ventricular hypertrophy.This article reports from 1976 to 1983 Nineteen children under the age of 5 to 11 years, including 9 infants <1 year old, were diagnosed on the basis of clinical symptoms and using noninvasive and invasive tests.The infants were mainly respiratory symptoms such as pulmonary infections, bronchus Spasms or respiratory failure require both Digoxin and diuretics, but medical therapy is not effective.> 1-year-old patients often suffer from respiratory tract infections (7/10), exercise tolerance (5/10), growth retardation / 10). Seven cases were preoperatively treated with two-dimensional echocardiography. Eighteen patients underwent cardiac catheterization preoperatively and found ventricular septal defect, pulmonary valve insufficiency, pulmonary insufficiency, main pulmonary artery and pulmonary artery branches on one or both sides Was aneurysm-like