先天性肺动脉吊带38例临床特点及预后分析

目的探讨儿童先天性肺动脉吊带(PAS)的临床特点及预后。方法回顾性分析2009年6月—2015年2月确诊的38例PAS患儿临床及随访资料。结果 38例PAS患儿中,35例(89.47%)因不同程度呼吸道症状入院,其中以反复咳嗽(89.47%)及喘息(84.21%)最为常见;余3例行常规术前检查发现异常进一步检查明确。38例PAS患儿均行多层螺旋CT血管重建(CTA)检查,37例诊断PAS,诊断率97.37%,1例提示肺动脉发育异常,后经手术证实为PAS。26例行手术治疗,其中25例行左肺动脉(LPA)重建术,死亡7例;12例非手术治疗,死亡9例,存活3例。结论心脏超声可早期提示PAS,CTA是最佳的确诊手段。LPA重建术是解除左肺动脉压迫的重要手段。

Clinical diagnosis,treatment, and prognosis of pulmonary artery sling in 38 children

Objective To explore the clinical characteristics and prognosis of congenital pulmonary artery sling (PAS) in children. MethodsThe clinical data of 38 children diagnosed with PAS during June 2009 and February 2015 were retrospectively analyzed. ResultsIn 38 PAS children, 35 cases (89.47%) were hospitalized for varying degrees of respiratory manifestations with recurrent cough (89.47%) and wheezing (84.21%) being the most common. The remaining 3 cases were found abnormal in routine preoperative examination and the diagnosis was confirmed after further examination. All 38 children were performed computer tomography angiography (CTA). Thirty-seven cases were diagnosed of PAS and diagnostic rate was 97.37%. One case was suspected of pulmonary dysplasia and diagnosed of PAS after operation. Twenty-six children received surgical treatment, of whom 25 children had pulmonary artery reconstruction (LPA). Seven children died during/after operation and 18 survived. The remaining 12 children received non-surgical treatment, of whom 9 died and 3 survived.ConclusionCardiac uhrasonography may reveal PAS in the early stage, while CTA is the best method for conifrmed diagnosis. LPA reconstruction is an important means of relieving left pulmonary artery oppression.