摘要目的 探讨右心室双出口外科手术的临床疗效和STS-EACTS分类方法的临床价值.方法 2001年1月至2007年9月期间外科手术的87例右心室双出口的患儿,男65例,女22例;年龄:12d～14岁;体重:3.1～40kg,平均(10.4±7.1)kg;术前SPO2 60%～98%,平均(81±9)%;心功能Ⅰ级5例,心功能Ⅱ级23例,心功能Ⅲ级49例,心功能Ⅳ级10例;按照STS-EACTS分类法,室间隔缺损型26例,法洛四联症型48例,完全性大动脉转位型10例,远离大动脉型3例.进行单心室修补9例,双心室修补74例.包括单纯心内隧道23例,同时补片扩大右室流出道31例,Rastelli 7例,REV 8例,大动脉调转术4例,Kawashima1例,其余为姑息手术4例.同时纠治合并肺动脉分支狭窄15例,主动脉弓病变5例,二尖瓣狭窄1例,主动脉瓣下狭窄伴右乏式窦瘤1例,胸骨裂1例.结果 全组围术期死亡2例,术后3个月死亡1例,术后随访1个月～5年,2例存在轻度左室流出道狭窄,6例存在右室流出道残余梗阻,压差15～75 mm Hg.术后心功能多可恢复至Ⅰ～Ⅱ级,但有7例心功能Ⅲ级,其中5例与右室流出道残余梗阻有关.结论 STS-EACTS分类法对于右心室双出口的个体化治疗具有更好的临床指导意义,而右心室流出道残余梗阻是影响预后的重要因素,值得进一步关注.

abstractsObjective To evaluate the clinical effects of treatment of double outlet of right ventricle (DORV) and clinical values of STS-EACTS International Nomenclature. Methods Eighty-seven DORV patients underwent operation from Jan 2001 to Sep 2007, aged from 12 days to 14 years (mean 2.8 ± 3.2 years) and weighted from 3.1 to 40 kg (mean 10.4 ± 7.1 kg). Preoperative SpO2 was from 60% to 98% (mean 81±9%). NYHA class Ⅰ was in 5 patients, NYHA class Ⅱ in 23, NYHA class Ⅲ in 49, NYHA class Ⅳ in 10. Applied with STS-EACTS International Nomenclature, 26 patients were DORV-VSD, 48 patients were DORV-Fallot, 10 were DORV-TGA (Taussig-Bing), and 3 were DORV non-committed VSD. Nine patients underwent single ventricular repair, and 74 patients underwent biventricular repair, including 23 patients with only VSD-aorta baffle procedure, 31 patients with additional right ventricle outlet enlargement with pericardiac patch, 7 with Rastelli procedure, 8 with REV procedure, 4 with arterial switch, and 1 with Kawashima procedure, the other 4 underwent palliated procedure. Associated anomalies were corrected simultaneously, including pulnomary artery stenosis (15 patients), aortic arch coactation or interrupt (5 patients), mitral valve stenosis (1 patient), subaortic valvular stenosis with right aortic sinus cystoma (1 patient). Results There were 2 early deaths and 1 death after 3 months postoperatively. All patients were followed up from one month to five years. Mild left ventricular outlet obstruction (LVOTO) existed in 2 patients. Right ventricular outlet obstruction (RVOTO) with gradient pressure from 15 to 75 mmHg existed in 6 patients. Can-clusions The STS-EACTS International Nomenclature provides more help in the treatment of DORV, and postoperative RVOTO is more important factor affecting prognosis.