Delmo Walter Eva Maria, Hetzer Roland
Department of Cardiothoracic, Transplantation and Vascular Surgery, Medizinische Hochschule Hannover, Hannover, Germany.
Department of Cardiothoracic and Vascular Surgery, Cardio Centrum Berlin, Berlin, Germany.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2018 Mar;21:46-57. doi: 10.1053/j.pcsu.2017.11.008.
We report the techniques and long-term outcome of mitral valve (MV) repair to correct congenital mitral stenosis in children. Between 1986 and 2014, 137 children (mean age 4.1 ± 5.0, range 1 month-16.8 years) underwent repair of congenital mitral stenosis (CMS). In 48 patients, CMS is involved in Shone's anomaly. The typical congenital MS (type I) was seen in 56 patients. Hypoplastic MV (type II, n = 15) was associated with severe left ventricular outflow tract abnormalities and hypoplastic left ventricular cavity and muscle mass. Supravalvar ring (type III, n = 48) ranged from a thin membrane to a thick discrete fibrous ridge. Parachute MV (type IV, n = 10) have 2 leaflets and barely distinguishable commissures, but all chordae merged either into 1 major papillary muscle or asymmetric papillary muscles-1 dominant and the other minuscule. Hammock valve (type IV, n = 8) appeared dysplastic with shortened chordae directly inserted into the posterior left ventricular muscle mass. MV repair was performed using commissurotomy, chordal division, papillary muscle splitting and fenestration, and mitral ring resection, each applied according to the presenting morphology. During the 28-year follow-up period, 23 patients underwent repeat MV repair and 3 underwent MV replacement after failed attempts at repeat repair. At 1 and 15 years postoperatively, freedom from reoperation was 89.3 ± 5.1% and 52.8 ± 11.8%, and cumulative survival rates were 92.3 ± 4.3% and 70.3 ± 8.9, respectively. Mortality unrelated to repair accounted for 9 (20%) deaths. Long-term functional outcome of MV repair in children with CMS is satisfactory. Repeat repair or replacement may be deemed necessary during the course of follow-up.
我们报告了用于纠正儿童先天性二尖瓣狭窄的二尖瓣修复技术及长期疗效。1986年至2014年期间，137例儿童（平均年龄4.1±5.0岁，范围1个月至16.8岁）接受了先天性二尖瓣狭窄（CMS）修复术。48例患者的CMS合并了Shone综合征。56例患者可见典型的先天性二尖瓣狭窄（I型）。二尖瓣发育不全（II型，n = 15）与严重的左心室流出道异常、左心室腔及心肌质量发育不全有关。瓣上环（III型，n = 48）范围从薄隔膜到厚的离散纤维嵴。降落伞样二尖瓣（IV型，n = 10）有两个瓣叶且瓣叶交界几乎无法区分，但所有腱索均汇入1个主要乳头肌或不对称乳头肌——1个占主导，另1个极小。吊床样瓣膜（IV型，n = 8）表现为发育异常，腱索缩短并直接插入左心室后壁心肌。根据二尖瓣的形态，分别采用交界切开术、腱索切断术、乳头肌劈开及开窗术、二尖瓣环切除术进行二尖瓣修复。在28年的随访期内，23例患者接受了再次二尖瓣修复，3例在再次修复失败后接受了二尖瓣置换术。术后1年和15年，再次手术的自由度分别为89.3±5.1%和52.8±11.8%，累积生存率分别为92.3±4.3%和70.3±8.9%。与修复无关的死亡占9例（20%）。CMS患儿二尖瓣修复的长期功能疗效令人满意。随访过程中可能需要再次修复或置换。
