Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany.
Eur J Cardiothorac Surg. 2013 Mar;43(3):473-81; discussion 481-2. doi: 10.1093/ejcts/ezs325. Epub 2012 Aug 23.
Having assembled information on a large series of patients with Shone's anomaly with the longest follow-up, we studied the operative results and long-term outcome of mitral valve (MV) repair techniques performed to correct the left ventricular inflow tract lesions of this congenital anomaly.
Between 1986 and 2011, 45 infants and children [mean age 5.16 ± 5.0 (median 3.9; range 2 months to 16.8 years] underwent surgical correction of Shone's anomaly. Left ventricular outflow tract obstructive lesions consisted of coarctation of the aorta, found in 89%, subaortic stenosis due to fibromuscular hypertrophy in 71% and subvalvular membrane in 51%. Left ventricular inflow tract obstructions concentrated on the mitral valve included mitral ring in all cases and the gamut of small and narrowed mitral orifice, commissural fusion, dysplastic or thickened leaflets, shortened and fused chordae, fibrous obliteration of interchordal spaces, underdeveloped papillary muscles, parachute valve, and supravalvular mitral ring-all contributing to mitral stenosis.
MV repair was performed using commissurotomy, division of chordae tendinae, papillary muscle splitting and fenestration, and resection of mitral ring, applied according to the presenting morphology in patients with either previously-corrected or concomitant correction of the left-sided obstructive lesions. Postoperative echocardiography showed absence of MV stenosis and immediate improvement of symptoms, except in a 3-month-old infant who died 18 days postoperatively due to myocardial failure. During the 23-year follow-up, 23 patients underwent repeat MV repair and one underwent MV replacement after failed attempts at repair. Mean duration of follow-up was 17.5 ± 1.5 years (range 6.4-22.7 years). Freedom from reoperation was 97.6 ± 2.4%, 89.3 ± 5.1%, 77.1 ± 7.2%, 72.0 ± 8.3% and 52.8 ± 11.8%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively Cumulative survival rate was 97.6 ± 2.4%, 92.3 ± 4.3%, 83.8 ± 6.1%, 75.7 ± 7.8% and 70.3 ± 8.9%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively. Mortality unrelated to valve repair accounted for nine deaths (20%). Age at operation, severity of mitral abnormalities and concomitant left ventricular outflow tract lesions proved to be independent risk factors for reoperation and mortality (P < 0.05).
Long-term functional outcome of mitral valve repair in children with Shone's anomaly is satisfactory. Repeat MV repair and/or replacement may be deemed necessary during the course of follow-up.
通过对一组患有 Shone 异常的大量患者的信息进行汇总，我们研究了对这种先天性异常的左心室流入道病变进行二尖瓣（MV）修复技术的手术结果和长期结果。
1986 年至 2011 年间，45 名婴儿和儿童（平均年龄 5.16 ± 5.0[中位数 3.9；范围 2 个月至 16.8 岁]）接受了 Shone 异常的手术矫正。左心室流出道阻塞性病变包括主动脉缩窄，占 89%，因纤维肌肉肥厚导致的主动脉瓣下狭窄占 71%，瓣下膜占 51%。左心室流入道阻塞主要集中在二尖瓣上，包括所有病例的二尖瓣环和各种小而狭窄的二尖瓣瓣口、瓣叶融合、发育不良或增厚、缩短和融合的腱索、腱索间空间的纤维性闭塞、发育不良的乳头肌、降落伞瓣和瓣上二尖瓣环——所有这些都导致二尖瓣狭窄。
MV 修复采用瓣交界切开术、腱索切断术、乳头肌切开术和开窗术以及二尖瓣环切除术，根据患者的表现形态进行应用，这些患者要么是先前已矫正，要么是同时矫正左侧阻塞性病变。术后超声心动图显示 MV 无狭窄，症状立即改善，除了一名 3 个月大的婴儿在术后 18 天因心肌衰竭死亡。在 23 年的随访中，23 名患者再次接受 MV 修复，1 名患者在修复失败后接受 MV 置换。平均随访时间为 17.5 ± 1.5 年（6.4-22.7 年）。术后 30 天、1 年、5 年、10 年和 15 年的无再手术生存率分别为 97.6 ± 2.4%、89.3 ± 5.1%、77.1 ± 7.2%、72.0 ± 8.3%和 52.8 ± 11.8%。累积生存率分别为 97.6 ± 2.4%、92.3 ± 4.3%、83.8 ± 6.1%、75.7 ± 7.8%和 70.3 ± 8.9%。手术时的年龄、二尖瓣异常的严重程度和同时存在的左心室流出道病变被证明是再手术和死亡的独立危险因素（P < 0.05）。
儿童 Shone 异常患者二尖瓣修复的长期功能结果令人满意。在随访过程中可能需要再次进行 MV 修复和/或置换。
