手术治疗先天性主动脉瓣上狭窄42例疗效观察

Surgical treatment of congenital supravalvular aortic stenosis in 42 cases

目的探讨先天性主动脉瓣上狭窄(supravalvular aortic stenosis,SVAS)患儿行手术治疗的安全性及近中期疗效。方法先天性SVAS患儿42例,36例在体外循环下行主动脉瓣窦-升主动脉补片扩大成形术,6例在体外循环下行升主动脉-主动脉弓延长补片成形术。记录体外循环时间、主动脉阻断时间、呼吸机辅助时间、术后ICU停留时间及住院时间;术后7 d复查心脏CT造影,检测主动脉瓣上及合并病变是否有残余梗阻;分别于术前及术后7 d行超声心动图检查,测定主动脉瓣上流速、主动脉跨瓣压差及左室射血分数;随访观察近中期预后。结果42例均顺利完成手术,体外循环时间(64.7±24.6)min,主动脉阻断时间(51.8±22.3)min,呼吸机辅助时间(25.8±26.4)h,术后ICU停留时间(12.3±6.2)d,住院时间(16.3±6.2)d;术后7 d心脏CT造影示主动脉瓣上及合并病变无残余梗阻;术后7 d主动脉跨瓣处压差[(20.34±8.56)mm Hg]较术前[(67.46±26.16)mm Hg]降低(P<0.05),主动脉瓣上流速[(1.63±0.68)m/s]较术前[(4.13±1.04)m/s]减慢(P<0.05),左室射血分数[(67.14±6.03)%]较术前[(61.35±5.12)%]增高(P<0.05);住院期间死亡2例,余患儿均顺利出院;随访至1个月~5年6个月,失访4例,余患儿心脏CT造影示均无残余梗阻。结论先天性SVAS患儿行手术治疗可解除主动脉瓣上及合并病变梗阻,改善心功能,安全性高,近中期效果满意。

Objective To investigate the safety as well as the short-and mid-term efficacy of surgical treatment on congenital supravalvular aortic stenosis(SVAS)in children.Methods In 42 children with congenital SVAS,36 underwent aortic valve sinus-ascending aortic patch augmentation under cardiopulmonary bypass(CPB)and 6 underwent ascending aorta-aortic arch extension under CPB.The time of CPB,time of aortic occlusion,time of ventilator assistance,ICU stay and hospitalization stay were recorded.The residual obstruction was detected by CT angiography.The supravalvular aortic velocity,aortic valve transmitral pressure gradient and left ventricular ejection fraction were detected by echocardiography before and on the 7 th postoperative day.The short-and mid-term prognosis was followed up.Results All these 42 patients completed operation successfully,with the CPB time of(64.7±24.6)min,aortic occlusion time of(51.8±22.3)min,ventilator assistance time of(25.8±26.4)h,ICU stay of(12.3±6.2)d,and hospitalization stay of(16.3±6.2)d.No residual obstruction was found on the 7 th postoperative day.The aortic valve transmitral pressure gradient and supravalvular aortic velocity decreased and left ventricular ejection fraction increased on the 7 th postoperative day((20.34±8.56)mm Hg,(1.63±0.68)m/s,(67.14±6.03)%)compared with those before operation((67.46±26.16)mm Hg,(4.13±1.04)m/s,(61.35±5.12)%)(P<0.05).All children were discharged except 2 died.The 1-month to 5.5-year follow-up showed no residual obstruction on cardiac CT angiography,and 4 patients were lost.Conclusion Surgical treatment of congenital SVAS can relieve supravalvular aortic stenosis and improve cardiac function,with a high safety and satisfactory short-and mid-term efficacy.