主肺动脉窗合并重度肺动脉高压的诊疗

Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension

目的通过本中心近年来主肺动脉窗(APW)的外科治疗经验,总结APW合并重度肺动脉高压的外科治疗及手术效果。方法回顾性分析西京医院心血管外科2010~2018年收治的23例APW患者的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。其中男9例、女14例,体重3.3~35.0(17.3±3.6)kg,年龄3个月至11岁,平均年龄(4.63±2.14)个月。结果其中APWⅠ型8例,Ⅱ型14例,Ⅲ型1例。单纯APW 11例,合并其它心脏畸形的12例,平均肺动脉压力(68.4±7.5)mm Hg。所有患者均在全身麻醉低温体外循环下行外科矫治手术。所有患者均顺利出院,平均随访时间4年,患者术后心功能良好,缺损修补无残余分流,肺动脉压力逐渐下降,平均肺动脉压30 mm Hg。结论APW发病几率较低,但进展迅速,往往短期内发生肺动脉高压,甚至导致死亡。早期准确的诊断及手术治疗、合理有效地控制肺动脉压力是治疗的关键,远期疗效较好。

Objective To study the diagnosis and treatment of aortopulmonary window(APW)associated with severe pulmonary hypertension.Methods The clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed.There were9 male and 14 female patients.The age was 3-132(4.63±2.14)months.The weight was 3.3-35.0(17.3±3.6)kg.Results Windows were situated in the proximal of semilunar valve(typeⅠ)in 8 patients,and distal of the aorta(typeⅡ)in 14 patients,from proximal to distal(typeⅢ)in only 1 patient.Eleven patients were isolated APW,the others were combined with cardiac defects.The mean pulmonary artery pressure was 68.4±7.5 mm Hg.All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass.All patients were discharged uneventfully,with an average follow-up time of 4 years.The patients showed good outcomes and no residual shunt after surgery,and the pulmonary artery pressure decreased to normal.Conclusion APW is an uncommon congenital cardiac anomaly.The clinical presentation is an excessive left-to-right shunt,and most patients present early in life.Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid.Operative treatment is indicated as soon as the diagnosis is established,regardless of the patient’s age,and most patients after surgery have a good long-term outcome.