单纯性单侧肺动脉缺如的临床特点及回顾性分析

目的:提高对单纯性单侧肺动脉缺如(UAPA)的认识和早期诊治水平。方法:结合我院2009年经肺血管造影检查明确诊断的1例单纯性单侧肺动脉缺如的临床资料,和国内1999年至2009年文献检索到的13例单纯性单侧肺动脉缺如病例的详细资料,对UAPA的临床特点进行回顾性总结分析。结果:我院患者以胸闷憋气症状就诊,曾于外院误诊为"肺栓塞",行多排CT肺动脉造影(CTPA)、X线肺血管造影(CPA)最终确诊单纯性UAPA。总结14例病例后发现单纯性UAPA病例以成年人多见(10/14),女性8例,男性6例,左侧缺如6例,右侧缺如8例,主要症状为:咳嗽、咳痰、反复肺部感染(9/14),活动后胸闷、气促、喘息(9/14),咯血(3/14),最终以选择性肺血管造影检查和多排螺旋CT肺血管造影确诊病例为最多,分别为6例,5例。在8例有治疗资料中,对症治疗4例(4/14),手术治疗4例(4/14)。结论:UAPA是一种罕见的先天性肺血管畸形,单纯性UAPA更为少见,症状以咳嗽、咳痰、咯血、反复肺部感染、胸闷及气促为主。目前X线肺血管造影仍然是诊断UAPA的金标准。近几年多排螺旋CT肺血管造影、核磁共振(MRI)肺血管造影正逐渐成为诊断UAPA的重要手段之一。目前单纯UAPA的治疗方法主要以对症治疗为主,外科治疗可根据病情选择患侧肺动脉重建、选择性肺侧支血管栓塞和结扎、患侧全肺或肺叶切除术。

Clinical features of isolated unilateral absence of pulmonary artery and retrosptive analysis

Objective：To improve the understanding of isolated unilateral absence of pulmonary artery （UAPA） and its level of early diagnosis and treatment. Methods： The clinical data of a 46-year-old female patient confirmed by pulmonary angiography （computed tomography pulmonary angiography and conventional pulmonary angiography） in Beijing Anzhen Hospital in 2009 and 13 cases of isolated unilateral absence of pulmonary artery patients with details in the domestic literature between 1999-2009 were retrospectively analysed. Results： The patients presented with chest suffocation and dyspnea over 1 year in Beijing Anzhen Hospital,and was misdiagnosed as＂Pulmonary Embolism＂by other hospital,and final diagnosis was isolated unilateral absence of pulmonary artery by means of multi-slice spiral CT pulmonary angiography（CTPA） and traditional pulmonary angiography（CPA）. After summarizing the data of 14 cases,It can be found that isolated UAPA were more common in adults （10 /14）,there were 8 females cases,6 males cases,6 cases of absence of the left and 8 cases of absence of the right,the course of 4 days to 30 years,the main symptoms were cough,sputum,repeated lung infections （9 /14）,chest tightness or shortness of breath in exercise,wheezing （9 /14）,hemoptysis （3 /14）,and the relevant investigation included X-ray chest film （13 cases）,echocardiography （10 ca-ses）,contrast-enhanced CT （7 cases）,multi-slice CT pulmonary angiography （5 cases）,cardio-pulmonary vascular MRI （3 cases）,ventilation/perfusion lung imaging （3 cases）,selective cardio-pulmonary angiography （6 cases）. The selective pulmonary blood vessels angiography and multi-slice spiral CT pulmonary angiography were most used in confirmed cases,which were 6 cases and 5 cases separately. In 8 cases of treatment information,symptomatic treatment was 4 例（4 /14）,surgery was 4 例（4 /14）,which recovered with good results. Conclusion： Unilateral absence of pulmonary artery is an rare congenital pulmonary vessel malformation,isolated UAPA is more uncommon. The main symptoms of the disease are cough,sputum,hemoptysis,repeated lung infections,shortness of breath. Conventional pulmonary angiography remains the gold standard for diagnosis of UAPA currently. However multi-slice spiral CT pulmonary angiography and MRI angiography become an important means in diagnosing UAPA in recent years. At present,the treatment of UAPA is mainly symptomatic management. Ipsilateral pulmonary reconstruction,selective pulmonary collateral vascular thrombosis and occlusion and ipsilateral pneumonectomy or lobectomy can be used in surgical treatment according to the disease situation.