Williams综合征患者的心血管手术治疗及效果

Treatment and effect of cardiovascular surgery in patients with Williams syndrome

目的分析Williams综合征(WS)心血管外科手术治疗效果。方法回顾性分析2010年1月至2020年1月在广东省人民医院心外科接受心血管手术68例WS患者的临床资料,其中男48例、女20例,中位年龄2.8岁(3个月~33岁)。除1例行主动脉缩窄矫治术之外,其余67例患者均为胸部正中切口低温体外循环下实施外科手术矫治主动脉瓣上狭窄(SAVS)和肺动脉狭窄,同期行左室流出道疏通3例、右室流出道疏通2例、二尖瓣成形2例、室间隔缺损修补3例、动脉导管结扎1例、双主动脉弓矫治1例。结果2例(2.9%)患者术后第2d突发心脏骤停死亡,1例(1.5%)患者在术后3年因弓部严重狭窄导致心功能不全死亡。SAVS矫治效果满意,2例(2.9%)在术后随访期间进展至中度主动脉瓣反流。共有5例(7.4%)患者术后再次干预。结论WS患者应早期诊断并随访、评估心血管系统病变,及时外科手术治疗临床效果良好。

Objective To analyze the effects of cardiovascular surgery on Williams syndrome(WS).Methods The clinical data of 68 WS patients undergoing cardiovascular surgery in the Department of Cardiac Surgery,Guangdong Provincial People’s Hospital from January 2010 to January 2020 were retrospectively analyzed.There were 48 males and 20 females with a median age of 2.8 years ranging from 3 months to 33 years.Except one patient undergoing the coarctation repair,the rest 67 patients underwent surgical interventions to correct supravalvular aortic stenosis(SAVS)and pulmonary artery stenosis with hypothermic cardiopulmonary bypass,concommitant with 3 patients of relief of left ventricular outflow tract obstruction,2 patients of relief of right ventricular outflow tract obstruction,2 patients of mitral valvuloplasty,3 patients of ventricular septal defect repair and 1 patient of arterial catheter ligation.Results Two(2.9%)patients died of sudden cardiac arrest on the next day after surgery.One(1.5%)patient died of cardiac insufficiency due to severe aortic arch stenosis 3 years after surgery.The effect of SAVS was satisfactory.Two(2.9%)patients progressed to moderate aortic valvular regurgitation during postoperative follow-up.A total of 5(7.4%)patients were re-intervened after operation for arch stenosis or pulmonary stenosis.Conclusion WS patients should be diagnosed early,followed up and assessed for cardiovascular system diseases,and timely surgical treatment has a good clinical effect.