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目的:探讨室间隔心肌切除术对低龄儿童肥厚型梗阻性心肌病(HOCM)的临床疗效及早期随访结果.方法:回顾性分析截至2014-11我院9例行室间隔心肌切除术的低龄HOCM患儿的临床资料.患儿诊断时平均年龄为(1.9±2.5)岁 (1天~6.4岁),手术时平均年龄(5.1±2.1)岁(8个月~7岁),平均体重(19.3±9.8)kg(4.3 kg~38.2 kg).术前静息左心室流出道压差(79.4±40.0)mmHg(1 mmHg=0.133 kPa).二尖瓣收缩期前移9例,二尖瓣中、重度反流7例.患儿在低温全麻体外循环下经升主动脉根部切口行室间隔心肌切除术,术后接受随访,观察手术疗效.结果:所有患儿行室间隔心肌切除术,同期行二尖瓣成形1例,右室间隔心肌切除3例,房间隔缺损修补3例,主动脉瓣下隔膜切除1例.全组无围手术期死亡,手术效果良好.主动脉阻断时间平均(66.0±18.4)min(36~90 min),术后静息左心室流出道压差由(79.4±40.0)mmHg降至(24.4±17.3)mmHg(P<0.01).二尖瓣反流分级由(2.6 ±1.4)级降至(0.4 ±0.5)级(P<0.01).术后残余二尖瓣收缩期前移及左心室流出道梗阻(左心室流出道压差61 mmHg)1例,短暂性完全性房室传导阻滞1例.1例低体重婴儿(4.3 kg)术后接受了120.8 h的体外膜肺支持.所有患儿均顺利出院.通过门诊或电话随访(11.1±8.7)个月(3~31个月),所有患儿症状改善,纽约心脏协会心功能Ⅰ~Ⅱ级.随访静息左心室流出道压差为(24.7±20.7)mmHg,与术前相比差异有统计学意 义(P<0.01).2例患儿残余二尖瓣收缩期前移及左心室流出道梗阻(左心室流出道压差分别为49 mmHg和61 mmHg).1例患儿术后3个月置入永久性起搏器.结论:室间隔心肌切除术对于解除低龄儿童HOCM左心室流出道梗阻效果好,早期随访结果满意,但存在一定的复发风险.“,”Objective: To explore the efficacy and early follow-up result of ventricular septal myectomy (VSM) in young children with hypertrophic obstructive cardiomyopathy (HOCM). Methods: A total of 9 young children with HOCM received VSM in our hospital up to 2014-11 were retrospectively studied. The average age at diagnosis was (1.9 ± 2.5) years, at operation was (5.1 ± 2.1) years and the mean body weight at operation was (19.3 ± 9.8) kg. The pre-operative resting left ventricular outlfow tract gradient (LVOTG) was (79.4 ± 40.0) mmHg. All 9 patients had systolic anterior motion (SAM) of anterior mitral valve leaflet and 7 patients had moderate to severe mitral regurgitation (MR). VSM through the incision of ascending aortic root was performed in allpatients, and the early post-operative follow-up was conducted to observe the efficacy.Results: All patients received VSM, the concomitant procedures including mitral valve repair in 1 patient, rightventricular septal resection in 3 patients, atrial septal defect repairing in 3 patients and subaortic membrane resection in 1patient. There was no peri-operative death and the surgical effect was good. The average aortic cross-clamp time was (66.0± 18.4) minutes . The post-operative resting LVOTG from (79.4 ± 40.0) mmHg decreased to (24.4 ± 17.3) mmHg, P <0.01,the mean MR from (2.6 ± 1.4) degree decreased to (0.4 ± 0.5) degree, P <0.01. The residual SAM and LVOT obstructionwith LVOTG at 61 mmHg was found in 1 patient, transient complete AV block in 1 patient, and 1 low-weight infant at 4.3kg received extracorporeal membrane oxygenation (ECMO) support for 120.8 hours. All patients discharged smoothly andthey were followed-up by clinical or telephone visit for (11.1 ± 8.7) months. The symptoms were improved in all patientsand NYHA function reached at I-II classes, the average resting LVOTG was (24.7 ± 20.7) mmHg, compared with the preoperativecondition, P <0.01. There were 2 patients with residual SAM and LVOT obstruction with LVOTG at 49 mmHgand 61 mmHg respectively, 1 patient received permanent pacemaker implantation at 3 months after the operation.Conclusion: VSM has good effect for relieving LVOT obstruction in young children with HOCM, the early followupresult is satisfactory while it has certain risk of recurrence.