儿童先天性肺动脉吊带临床特征及预后危险因素

Clinical characteristics and prognostic risk factors of congenital pulmonary artery sling in children

目的分析儿童先天性肺动脉吊带(PAS)的临床和影像学特征,探讨其诊治策略及影响预后的危险因素。方法对2011年1月至2018年10月广州医科大学附属广州市妇女儿童医疗中心收治的98例PAS患儿的临床表现、影像学特征、治疗方式及预后进行回顾性总结和分析。结果1.共纳入98例患儿,其中男56例,女42例;起病年龄0~19个月,中位数2个月;诊断年龄0~84个月,中位数4个月。2.临床表现主要为咳嗽(61/98例,62.24%),喘息(47/98例,47.96%),气促(43/98例,43.88%),发绀(19/98例,19.39%),喉鸣(8/98例,8.16%)等。3.共96例行心脏超声检查,诊断阳性84例(87.50%),合并其他先天性心血管畸形62例(62/96例,64.58%),其中以房间隔缺损最为常见(32/96例,33.33%)。4.共92例患儿行胸部CT及三维重建检查,诊断率为100.00%,呼吸道狭窄以气管中下段为主(76/92例,82.61%),支气管桥13例(13/92例,39.13%)。77例患儿行支气管镜检查,发现完全性气管软骨环43例(43/77例,55.84%),气管支气管软化13例(13/77例,16.88%)。5.其中67例行左肺动脉(LPA)重建术,术后死亡5例,均死于术后并发症难以撤机(1例单纯行LPA重建术,余4例均有气管手术干预),失访24例,存活38例,随访2~96个月,呼吸道症状均较术前改善;31例非手术治疗,死亡18例,失访7例,余6例随访中仍有不同程度的呼吸道症状。6.多因素回归分析显示,保守治疗是影响PAS患儿预后的独立危险因素(OR=7.45,95%CI:1.23~48.68)。结论心脏超声、胸部CT及支气管镜检查三者结合更有利于PAS的诊断。LPA重建术是治疗PAS的主要手段,可改善多数患儿呼吸道症状,并气管干预术者死亡风险大。保守治疗是影响PAS患儿预后的独立危险因素。

Objective To analyze the clinical and imaging characteristics of pulmonary artery sling(PAS)in children and to explore its diagnosis and treatment strategies and risk factors.Method s A retrospective study was performed in 98 pediatric patients with PAS who were admitted to Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,from January 2011 to October 2018.All their clinical features,imaging examination,treatment and prognosis were reviewed and analyzed.Result s(1)Among the 98 patients with PAS,56 cases were male and 42 cases were female.The age range of onset was 0-19 months with the median age of 2 months,and the age range of diagnosis was 0-84 months with the median age of 4 months.(2)The main clinical manifestations were cough(61/98 cases,62.24%),recurrent wheezing(47/98 cases,47.96%),shortness of breath(43/98 cases,43.88%),cyanosis(19/98 cases,19.39%),and laryngeal stridor(8/98 cases,8.16%)and so on.(3)Ninety-six patients underwent cardiac ultrasonography,and the diagnostic rate was 87.50%(84/96 cases),and 62 cases(62/96 cases,64.58%)of them were found to be associated with other congenital cardiovascular anomalies,among which atrial septal defect was the most common(32/96 cases,33.33%).(4)Chest CT and three-dimensional reconstruction were performed in 92 PAS children,and the diagnostic rate was 100.00%.Airway stenosis was mainly in the middle and lower sections of the main bronchus(76/92 cases,82.61%),and bronchobridge was found in 13 cases(13/92 cases,39.13%).Bronchoscopy was performed in 77 children with PAS,showing complete tracheal cartilage ring in 43 cases(43/77 cases,55.84%),and tracheobronchomalacia in 13 cases(13/77 cases,16.88%).(5)Among 67 cases who had received left pulmonary artery(LPA)reimplantation,5 cases died after operation because of airway stenosis and weaning failure(LPA reconstruction was performed in 1 case,and both LPA reconstruction and tracheal intervention were performed in the other 4 cases),24 cases lost to follow-up,38 cases survived,and the respiratory symptoms were improved in 2 to 96 months of follow-up.There were 31 cases of non-surgical treatment,of which 18 cases died of respiratory failure,7 cases lost to follow-up,and the rest 6 cases showed respiratory symptoms of different degrees in the follow-up period.(6)Multivariate regression analysis showed that conservative treatment was an independent risk factor affecting the prognosis of PAS(OR=7.45,95%CI:1.23-48.68).Conclusions The combination of cardiac ultrasound,chest CT and bronchoscopy is important to the diagnosis of PAS.LPA reconstruction is the main method to treat PAS,which can improve respiratory symptoms,but its fusion with tracheal intervention poses a high risk of death.Conservative treatment is an independent risk factor that affects the prognosis of PAS children.