儿童单侧肺静脉闭锁4例并文献复习

Unilateral pulmonary vein atresia of children：4 cases report and literature review

目的总结儿童单侧肺静脉闭锁（UPVA）的临床特征、影像学特点、治疗及预后，以提高临床医师对该病的认识。方法回顾性分析广州市妇女儿童医疗中心心内科2014年1月至2016年12月收治的4例UPVA患儿的临床资料，结合复习PubMed、OVID及Elsevier医学文献数据库国际报道的50例，万方数据库国内报道4例，共58例，分析其临床特点、诊断、治疗及预后。结果本研究报道4例，平均年龄1．8岁（1．5—2．7岁），其中先天性UPVA3例，继发性UPVA1例。左上肺静脉闭锁1例，左肺静脉闭锁2例，右肺静脉闭锁1例。表现为反复咯血3例，反复咳嗽1例。3例并先天性心脏病。1例行支气管动脉栓塞术，1例行肺静脉．左心房连接术，2例保守治疗。所有病例已随访1～3年，行肺静脉-左心房连接术患儿已全愈，行支气管动脉栓塞术患儿未再出现咯血但仍有反复咳嗽，继发性UPVA患儿无明显临床症状，另1例拒绝手术后口服药物保守治疗的患儿仍反复肺部感染，间断咯血。文献报道共54例（≤18岁的患儿40例），平均年龄13．76岁（8d-43岁）。其中先天性UPVA52例，继发性UPVA2例。右侧肺静脉闭锁27例，左侧肺静脉闭锁22例，其他5例。94．4％（51／54例）的患者表现为反复咳嗽、肺部感染，92．6％（50／54例）的患者表现为气促、劳力性呼吸困难，68．5％（37／54例）的患者表现为咯血或呕血。50．0％（27／54例）的患者合并心内畸形。75．9％（41／54例）的患者合并有不同程度的肺动脉高压，35．2％（19／54例）的患者有肺淋巴管扩张。25例行肺切除术，11例行肺静脉一左心房连接术，7例行支气管动脉栓塞术，11例保守治疗。术后大多预后良好，无明显临床症状或症状轻微。结论临床上遇到不明原因的咯血、反复下呼吸道感染、肺实变或肺发育不良、肺动脉高压时，应考虑UPVA。尽早诊断，尽早行支气管动脉栓塞术或外科重建肺静脉与心房连接，同时修补心内缺损，可明显改善患儿症状或治愈患儿。

Objective To summarize the clinical characteristics,imaging characteristics,treatment and progno- sis of unilateral pulmonary vein atresia （UPVA） in children and to improve the clinician＇ s understanding of this disease. Methods The clinical data of 4 cases of UPYA from January 2014 to December 2016 in Department of Cardiology, Guangzhou Women and Children＇s Medical Center were retrospectively analyzed, and 50 cases from reviews of PubMed, OVID and Elsevier in the international medical literature database and 4 cases in Wanfang database for the domestic report were reviewed. The clinical characteristics, diagnosis, treatment and prognosis of total of 58 cases were analyzed. Results Four patients, with an average age of 1.8 years （ 1.5 - 2.7 years）, showing congenital UPVA in 3 patients and secondary UPVA in 1 patient. There was 1 case of left upper pulmonary vein atresia,2 cases of left pulmo- nary vein atresia and 1 case of right pulmonary vein atresia. Three cases showed recurrent hemoptysis and recurrent cough occurred in 1 case. Three cases were complicated with congenital heart disease. There was one case underwent bronchial artery embolization, 1 case received pulmonary vein left atrium connection, and 2 cases received conservative treatment. All patients had been followed up for 1 - 3 years so far. The patient receiving pulmonary vein [eft atrium had been completely cured, and the patient receiving bronchial artery embolization showed no occurrence of hemoptysis but still showed recurrent cough hemoptysis. The patient with secondary UPVA had no obvious clinical symptoms, the other 1 case who rejected operation and received conservative treatment still had recurrent pulmonary infection, intermittent hemoptysis. The average age of 54 cases（40 cases with age ≤ 18 years old） from the literature reports was 13.76 years （8 days -43 years） in which 52 cases were diagnosed as congenital UPVA, while 2 cases were secondary UPVA. Twenty- seven cases were right pulmonary vein atresia, 22 cases were left pulmonary vein atresia, and 5 cases were other types. There were 94.4% （51/54 cases） of the patients having recurrent cough, pulmonary infection ,92.6% （50/ 54 cases） of the patients with exertional dyspnea and polypnea,68.5% （37/54 cases） of the patients with hemoptysis and hematemesis. There were 50.0% （27/54 cases） of UPVA patients who were complicated with heart malformation. Different degrees of pulmonary hypertension were observed in 75.9% （41/54 cases） of children, and 35.2% （ 19/54 eases） of patients had pulmonary lymphatie dilatation. Pulmonary. resection was performed in 25 cases,pulmonary vein left atrium eonneetion was performed in 11 eases,bronchial artery emholization was performed in 7 cases,and conserva- tive treatment was performed in 11 cases. Alter operation,most of the patients had good prognosis without obvious clini- cal symptoms or mild symptoms. Conclusions In clinical practice,if unexplained hemoptysis, recurrent lower respira- tory tract infection,pulmonary consolidation, pulmonary dysplasia or puhnonary hypertensinn present, the possibility of UPVA should be considered. Early diagnosis and early bronchial artery embolization, reconstruction of the pulmonary vein and atrial connection and repair of the detbct of heart,can improve the symptoms or cure the children and reduce the morta-lity significantly.