摘要
目的提高对单纯性单侧肺动脉缺如（uAPA）的临床表现、病理生理、诊断和治疗的认识。方法报道上海市静安区中心医院呼吸科诊治的1例单纯性左侧肺动脉缺如患者的相关资料，并结合从多个中文期刊数据库检索到的关于单纯性UAPA的病例资料进行回顾性研究。结果44例患者平均31．5岁（3个月～69岁），主要症状有反复肺部感染（47．4％）、咯血（52．6％）、气喘或活动后气促（57．9％）、胸闷（31．6％）、心悸（18．4％）、胸痛（7．9％）等，有1例患者没有症状。43．2％（19／44）的患者存在肺动脉高压。CT肺动脉造影（CTPA）、MRI、电子束CT（EBCT）、血管造影（DSA）均有确诊价值。20．5％的患者施行了手术治疗。结论单纯性UAPA是一种非常罕见的先天性畸形，其症状无明显特异性，易漏诊、误诊。可通过CTPA、MRI、EBCT、DSA等确诊。最理想的手术方法是患侧肺动脉重建术，但在我国还无法实行。
Objective To highlight the clinical manifestation, pathophysiology, diagnostic and therapeutic method of isolated unilateral absence of pulmonary artery（UAPA）. Methods The clinical, auxiliary examinational and pathological data of a patient with isolated UAPA from our hospital was presented, and relevant literatures from multiple databases of Chinese Jorunals were retrospectively reviewed. Results The median age of the 44 patients identified was 31.5 years （range,0.25 to 69 years）. The main symptoms were frequent pulmonary infections （47.7%）, hemoptisis （ 52.6%）, dyspnea or shortness of breath after the event （57.9%）, chest tightness （31.6%）, palpitations （18.4%）, chest pain （7.9%）, and,1 was asymptomatic. Pulmonary hypertension was present in 43.2%（19/44） of the patients. CTPA, MRI, EBCT and DSA had diagnostic value. Surgical procedures were performed in 20.5% of the patients. Conclusions Isolated UAPA is a rare congenital pulmonary vessel malformation, the symptoms no specific, and was easy to be misdiagnosed. The disease can be diagnosed by CTPA, MRI, EBCT and DSA, and the best surgical approach is revascularization which can not be implemented in our country.
出处
《国际呼吸杂志》
2012年第5期356-361,共6页
International Journal of Respiration
关键词
单侧肺动脉缺如
肺动脉发育不全
肺动脉不发育
肺血管畸形
肺动脉高压
治疗
Unilateral absence of pulmonary artery
Pulmonary artery hypoplasia
Pulmonary artery agenesis
Pulmonary artery malformation
Pulmonary hypertension
Treatment
