目的探讨罕见肺动脉瓣缺如综合征（absent pulmonary valve syndrome，APVS）的外科治疗效果。 方法回顾性分析3例APVS患者在低温体外循环下行根治手术，其中女2例、男1例，年龄分别为9个月、14岁、10岁，其中2例合并右室双出口（double outlet right ventricle，DORV）。3例均采用自体心包片重建肺动脉瓣，1例行肺动脉成形术，2例未处理肺动脉。 结果3例均存活，术中食管超声及术后超声复查肺动脉瓣功能良好，随访8～24个月，均恢复良好、生活质量明显改善，复查心脏彩色超声心动图肺动脉瓣功能良好，无明显反流。 结论APVS是一种少见的先天性心脏病，合并DORV或心内其他结构正常的病例非常罕见，手术治疗可获得良好效果。
ObjectiveTo summarize the indication and surgical experiences of valve replacement, high-risk factors and long-term results for aortic regurgitation and large left ventricle. MethodsWe retrospectively analyzed the clinical data of 42 patients with aortic regurgitation and left ventricle end-diastolic diameter (LVEDD) ≥70 mm and left ventricle end-systolic diameter (LVESD) ≥ 50 mm in our hospital from March 2004 through December 2012. There were 38 males and 4 females,aged 16-73 (45.86±14.99) years. The patients underwent aortic valve replacement, who were evaluated by echocardiography at pre-operation, pre-discharge and early follow-up. The follow up period was 12-132 months. ResultsEarly death occurred in one patient. And five patients died during the follow-up. One week after surgery in 41 patients, LVEDD (62.00±13.21 mm), LVESD (50.71±14.02 mm), indexed LVEDD (35.23±8.58 mm/m2), indexed LVESD (28.92±9.08 mm/m2), LVEF (46.41%±12.49%), were significantly smaller than those before the operation (P<0.01). Heart function grades, preoperative EF, LVEDD and indexed LVEDD were the predictors for left ventricular function recovery. One-year, 5-year, 10-year survival rate was 92.9%, 90.2%, 83.8%, respectively. ConclusionMost of patients with aortic regurgitation and large left ventricle still have indications for surgical treatment, but severe left ventricular dysfunction and ventricular arrhythmia are high risk factors for long-term survival.