单侧肺动脉缺如1例报道和中文文献分析

Unilateral absence of a pulmonary artery:a case report and document analysis

目的分析中国单侧肺动脉缺如（unilateral absence of a pulmonary artery,UAPA）患者的临床特点、诊断方法和治疗策略。方法检索清华同方和万方这两个汉语数据库,查得1988~2009年发表的关于UAPA的中文文章52篇,从临床表现、各种检查、诊断、误诊、治疗和随访等方面分析这些文章。我们报道1例UAPA患者。结果 52篇中文文献报道234例UAPU患者,加上我们报道的1例共235例,其中单纯性UAPA患者29例;合并其他心血管畸形的UAPA患者201例;5例患者没有注明是否合并其他心血管畸形。单纯性UAPA患者年龄3~61岁,平均（29.2±14.3）岁,前3个主要症状：反复呼吸道感染史（48.3%）、呼吸困难（41.4%）和咯血（34.5%）。确诊UAPA的主要方法是右心室和（或）肺动脉造影,其次还有增强电子束CT、多排CT肺动脉造影、超声心动图（UCG）、MRI和核素肺通气-灌注扫描。手术治疗者较少,主要针对咯血。合并其他心血管畸形的UAPA患者年龄2~30岁,平均（9.4±8.2）岁,主要合并法洛四联征143例（71.1%）。前3个主要症状：呼吸困难、活动受限和蹲踞。确诊方法同单纯性UAPA患者。手术主要针对合并的心血管疾病。结论中文文献报道的UAPA有一定的特点。患者的临床表现缺乏特异性,但如果出现胸部畸形、患侧呼吸音低、心脏杂音和肺动脉高压,应考虑UAPA。用右心室和（或）肺动脉造影等方法确诊。UAPA患者容易误诊,特别是单纯性UAPA患者。在我国,目前手术治疗UAPA主要针对合并的其他心血管病畸形或咯血。

Objective To analyze clinic characteristics,diagnostic method and treatment in Chinese patients with unilateral absence of a pulmonary artery（UAPA）.Methods We indexed two Chinese medical databases： Qinghua Tongfang and Wanfang,we found 52 papers about UAPA in 1988-2009 published literature.The papers were analyzed in the aspect of clinic manifestation,diagnostic method,diagnosis,diagnostic errors,treatment and follow-up.Then we reported one case of a forty-year-old man with UAPA.Results Among 235 patients with UAPA（234 from database,one from our case report）,there were 29 patients with simple UAPA,201 patients with cardiovascular abnormalities.The diagnosis was confirmed by ventriculography of right ventricle,pulmonary arteriography,CT scan,echocardiography,MRI and ventilation-perfusion lung scanning.The average age of the patients with simple UAPA was（29.2±14.3） years old（range 3 to 61 years old）.The main symptoms were recurrent respiratory tract infection（48.3%）,dyspnea（41.4%） and hemoptysis（34.5%）.21% of the patients accepted operation for hemoptysis.The median age of the UAPA patients with cardiovascular abnormalities was（9.4±8.2） years old（range 2 months to 30 years old）.Fallot tetralogy was present in 71.1% of the patients.Most patients had symptoms such as dyspnea,limited exercise tolerance and squatting.Operations were practised for cardiovascular abnormalities.Conclusion Symptom of UAPA has no specificity.When frequent pulmonary infection,dyspnea,hemoptysis and limited exercise tolerance are present,UAPA should be considered.The diagnosis can be made by cardiac catheterization and pulmonary venous wedge angiography.In China,surgery treatment is mainly in choice for cardiovascular abnormalities and hemoptysis.