主肺动脉窗合并重度肺动脉高压的诊疗

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1...

Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension

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.