单纯性单侧肺动脉缺如患者临床特点分析  

Clinical features of isolated unilateral absence of pulmonary artery

作　　者：王新霞[1] 朱光发[1] 高元明[1] 朱晨曦[1] Wang Xinxia Zhu Guangfa Gao Yuanming Zhu Chenxi(Department of Respiratory Medicine, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China)

机构地区：[1]首都医科大学附属北京安贞医院呼吸科,100029

出　　处：《中国医药》2017年第7期1001-1004,共4页China Medicine

摘　　要：目的 分析单纯性单侧肺动脉缺如（UAPA）患者的临床特点，以提高对该病的认识和早期诊治水平。方法 回顾性分析首都医科大学附属北京安贞医院2001年4月至2016年9月明确诊断的6例UAPA患者的临床资料，对其一般资料、临床表现、实验室检查、胸部影像学检查、诊治情况及转归等进行研究和分析。结果 6例UAPA患者中，男2例、女4例。平均年龄（53±9）岁。其中，左侧肺动脉缺如1例，右侧肺动脉缺如5例。6例患者早期均无特殊表现，出现症状的平均年龄为（46±10）岁。临床表现主要包括胸闷、气短，心悸，咳嗽咳痰，咯血，间断性晕厥，以及双下肢水肿。均有不同程度的低氧血症，部分出现肺动脉高压、Ⅱ型呼吸衰竭、肺源性心脏病等。最终以选择性肺血管造影和多排螺旋CT肺血管造影确诊。上述患者就诊后均有误诊过程，分别被误诊为支气管扩张、肺栓塞、冠状动脉粥样硬化性心脏病等。中位误诊时间为4.5（2.5，12.5）年。6例患者中1例行支气管动脉近端弹簧圈栓塞术止血，出院后偶有少量咯血；1例无特殊处理；余4例经内科对症治疗好转后出院。结论 UAPA是一种临床罕见的先天性肺血管畸形，单纯性UAPA更为少见，早期多无症状，出现症状后多因无特异性而被长期误诊、漏诊。临床医师应提高对该病的认识，以早期诊断和治疗，延长生存期。Objective To analyze clinical features of isolated unilateral absence of pulmonary artery（UAPA）. Methods Six patients diagnosed of UAPA from April 2001 to September 2016 in Beijing Anzhen Hospital, Capital Medical University were retrospectively analyzed; clinical data included general information, clinical manifestation, laboratory examination, chest imaging examination, diagnosis, treatment and prognosis. Results There were 2 males and 4 females, with a mean age of （53±9）years. There was 1 case of left pulmonary artery absence and 5 cases of right pulmonary artery absence. Unilateral absence of pulmonary artery had no early clinical manifestation and the symptoms appeared at age of （46±10）years. The main manifestations were chest tightness, shortness of breath, palpitation, cough and expectoration, hemoptysis, intermittent syncope and edam of lower extremities; all patients had hyoxemia; some had pulmonary artery hypertension, type Ⅱ respiratory failure and pulmonary heart disease. All patients were diagnosed by selective pulmonary angiography and multi-slice spiral CT pulmonary angiography. Patients with pulmonary artery absence were usually misdiagnosed of bronchiectasis, pulmonary embolism and coronary heart disease; the mean misdiagnosis time was 4.5（2.5, 12.5）years. One case had proximal bronchial artery coil embolization; 1 case had no special treatment; 4 cases had medical symptomatic treatments and discharged after recovery. Conclusion UAPA is a rare congenital pulmonary vascular malformation with no specific clinical manifestations.

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 

分 类 号：R563[医药卫生—呼吸系统]