儿童肺静脉狭窄临床特征分析

Clinical characteristics of pulmonary vein stenosis in children

目的分析肺静脉狭窄(PVS)患儿的临床特征、治疗方案、预后和影响因素。方法回顾性分析2016年10月至2022年3月首都医科大学附属北京儿童医院收治的19例PVS患儿的临床资料。19例患儿中男16例,女3例;诊断年龄(2.81±1.95)岁。患儿临床特征采用描述性分析。结果19例患儿中,原发性PVS 14例(73.7%),肺静脉异位引流(APVC)矫治术后继发性PVS 5例(26.3%)。13例(68.4%)患儿有咯血,其中5例病史中有危及生命的大咯血,11例(57.9%)有反复呼吸道感染/肺炎病史,其他表现包括:生长发育迟缓(6例)、发绀(5例)、呼吸困难(3例)。6例患儿合并肺动脉高压,3例患儿临床诊断右心衰竭。16例(84.2%)患儿为单侧PVS,3例为双侧PVS;全部患儿肺部CT有小叶间隔增厚、网格影及磨玻璃影表现。手术治疗10例,其中2例行肺静脉成形术,但术后均发生再狭窄;8例行单侧肺叶切除术,术后临床症状均消失。9例保守治疗:3例死亡(均为APVC矫治术后继发性双侧肺静脉狭窄患儿),6例随访中,仍间断有临床症状。结论儿童PVS临床以咯血、反复呼吸道感染为主要表现,可出现危及生命的大咯血。对于单侧PVS,肺叶切除术是有效的治疗方法。APVC术后继发性PVS较原发性PVS的预后差、病死率高。

Objective To analyze the clinical characteristics of pulmonary vein stenosis(PVS)in children,and to explore its treatment and prognostic factors.Methods The clinical data of 19 children with PVS treated in Beijing Children′s Hospital,Capital Medical University from October 2016 to March 2022 were analyzed retrospectively.There were 16 males and 3 females.The median age at diagnosis was(2.81±1.95)years.A descriptive analysis of clinical characteristics of children was made.Results Of the 19 children,14 cases(73.7%)had primary PVS and 5 cases(26.3%)had secondary PVS after surgery of anomalous pulmonary venous connection(APVC).Thirteen children(68.4%)had hemoptysis.In the hemoptysis children,5 cases had life-threatening massive hemoptysis,and 11 cases(57.9%)had a history of recurrent respiratory tract infection or pneumonia.Other manifestations of hemoptysis included failure to thrive(6 cases),cyanosis(5 cases),and dyspnea(3 cases).Complications were pulmonary hypertension(6 cases)and right heart failure(3 cases).There were 16 cases(84.2%)of unilateral PVS and 3 cases of bilateral PVS.Interlobular septal thickening,grid shadow and ground glass opacities were found on CT of all PVS cases.Ten cases underwent surgery,and 2 cases of them received angioplasty,but restenosis occurred in both of them.Eight children underwent pulmonary lobectomy,and their clinical symptoms were all relieved after operation.Nine patients were treated conservatively,and 3 cases of them died of bilateral PVS secondary to APVC.The remaining 6 alive cases still had intermittent clinical symptoms during follow-up.Conclusions Hemoptysis and recurrent respiratory tract infection are the main clinical manifestations of PVS in children,and life-threatening massive hemoptysis can occur.Lobectomy is an effective treatment for unilateral PVS.The prognosis of secondary PVS after APVC is poorer and its mortality is higher,compared with primary PVS.