摘要目的:探讨Ross Ⅱ技术治疗婴幼儿难治性先天性二尖瓣病变的早期效果。方法:回顾性分析2018年8月至2019年9月在广州市妇女儿童医疗中心心血管外科接受Ross Ⅱ技术治疗的6例婴幼儿难治性先天性二尖瓣病变患者的临床资料。男性2例，女性4例，手术年龄50 d至1岁，其中2月龄3例，4、11、12月龄各1例。重度二尖瓣关闭不全6例，合并中度二尖瓣狭窄3例，合并二尖瓣脱垂1例。术中完整切取自体肺动脉带瓣管道，采用牛心包或自体心包在肺动脉管道肌性边缘缝合一"大礼帽"结构，翻转后将肺动脉管壁与二尖瓣瓣环间断褥式缝合，并将牛心包边缘固定在左心房壁，保持肺动脉瓣环充分展开，并避开肺静脉开口。采用牛颈静脉带瓣管道重建右心室流出道。结果:手术死亡1例，为2月龄患者，术后当晚因肺高压危象导致心脏骤停死亡。5例存活患者术后均恢复顺利，痊愈出院。术后随访3~13个月，超声心动图提示二尖瓣前向流速1.5~2.3 m/s，二尖瓣轻度关闭不全4例，左心房及左心室大小均恢复至同龄儿童正常水平。二尖瓣中度关闭不全1例，伴左心室扩大及射血分数降低，且肺动脉瓣叶有小赘生物形成，伴中度狭窄。余患者重建的肺动脉前向血流通畅，瓣叶功能良好。所有患者临床症状明显改善，体重增长满意。结论:Ross Ⅱ技术作为婴幼儿难治性二尖瓣病变的补救性治疗方案，早期效果满意，中远期效果需要进一步随访。此术式手术适应证的选择应慎重。

abstractsObjective:To evaluate the early results of pulmonary autograft mitral valve replacement (Ross Ⅱ procedure) in infants with intractable congenital mitral valve lesions.Methods:Between August 2018 and September 2019, 6 infants underwent mitral valve replacement with a pulmonary autograft in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center. There were 2 males and 4 females.The age at surgery ranged from 50 days to 1 year old.Preoperative diagnoses included severe to critic mitral valve insuffiency in all patients, moderate mitral valve stenosis in 3 patients, and mitral valve prolapse in one. When the pulmonary autograft was harvested, a cuff of bovine or autologous pericardium was sewn onto the proximal (infundibular) end of the autograft ( "top-hat" configuration). The distal (pulmonary) end of the autograft was secured to the mitral annulus.At the level of the left atrial free wall, the pericardial cuff was progressively tailored and sewn onto the atrial wall to remain away from the ostia of the pulmonary veins and to maintain normal morphology of the autograft. The bovine jugular valved conduit was used to reconstruct the right ventricular outflow tract.Results:There was one early death due to sudden cardiac arrest at the night of surgery day. The remaining 5 patients were successfully recovered and discharged. Follow-up of survivors ranged from 3 to 13 months. Echocardiographic follow-up demonstrated the flow velocity across the mitral valve position was 1.5 to 2.3 m/s, with a means gradient of 4 to 6 mmHg (1 mmHg=0.133 kPa). Four patients showed mild mitral insuffiency, normal left atrium and ventricle size and left ventricle ejection fraction.One patient had moderate mitral insuffiency, pulmonary valve endocarditis, and reduced left ventricle ejection fraction. The clinical symptoms of all survivals improved significantly and the weight gain were satisfactory.Conclusion:Pulmonary autograft mitral valve replacement may be a feasible and effective remedial surgical strategy for young infants with intractable congenital mitral valve lesions.