改良Morrow术治疗儿童肥厚型梗阻性心肌病远期疗效的单中心回顾性研究

Long-term effect of modified Morrow surgery on hypertrophic obstructive cardiomyopathy in children:A retrospective study in a single center

目的分析改良Morrow术(室间隔心肌切除术)治疗儿童肥厚型梗阻性心肌病(hypertrophic obstructive cardiomyopathy,HOCM)的远期疗效。方法回顾性分析广东省人民医院2010年1月—2022年8月进行改良Morrow术的HOCM患儿(年龄≤14岁)的临床资料,包括住院情况、围手术期以及远期十五导联心电图和超声心动图的变化。结果共纳入29例患者,其中男22例、女7例,年龄10.00(5.00,12.00)岁。纽约心脏协会(NYHA)心功能分级Ⅲ或Ⅳ级5例(17.2%)。所有患儿均进行室间隔心肌切除,单纯左心室间隔切除23例(79.3%),单纯右心室间隔切除4例(13.8%),双心室间隔切除2例(6.9%)。同期行二尖瓣成形9例(31.0%),二尖瓣置换1例(3.4%),肺动脉瓣成形1例(3.4%)。29例患儿均存活,术后心功能均明显改善。出院前心电图提示有1例出现Ⅰ度房室传导阻滞,2例出现完全性右束支传导阻滞,6例出现完全性左束支传导阻滞。出院前超声心动图与术前相比,左心室间隔心肌切除患儿左心房内径减小(P<0.001),左心室收缩末期内径增大(P=0.009),左心室流出道压力阶差降低(P<0.001),室间隔厚度减小(P<0.001)。术后二尖瓣前向运动均消失,二尖瓣反流束面积减小(P<0.001)。行右心室间隔心肌切除患儿右心室流出道流速及峰值压力阶差也有明显减小。29例患儿门诊或电话平均随访(69.03±10.60)个月,29例患儿均存活,患儿NYHA心功能分级均为Ⅰ级或Ⅱ级,心律较前无新发传导阻滞,超声心动图提示左心室流出道压力阶差仍保持较低水平(P<0.001),2例出现中度二尖瓣反流,1例行单纯右心室间隔心肌切除术后出现左心室流出道梗阻并二尖瓣中度反流。结论儿童HOCM容易累及右心室,甚至导致双心室梗阻,所以HOCM患儿术前症状更重,改良Morrow术可有效解除流出道梗阻,能明显改善HOCM患儿的心功能及症状,远期疗效也令人满意,但患儿术后左心室后壁仍肥厚,发生传导阻滞的风险也会增加。

Objective To analyze the long-term outcome of modified Morrow surgery(interventricular septal cardiomyectomy) in the treatment of hypertrophic obstructive cardiomyopathy(HOCM) in children.Methods The clinical data of the children with HOCM(aged≤14 years) who underwent modified Morrow surgery from January 2010 to August 2022 in Guangdong Provincial People's Hospital were retrospectively analyzed,including changes in hospitalization status,perioperative period,and long-term 15-lead electrocardiogram and echocardiography.Results A total of 29 patients were collected,including 22 males and 7 females,aged 10.00(5.00,12.00) years.Five(17.9%) patients had New York Heart Association(NYHA) heart function grade Ⅲ or Ⅳ.Ventricular septal cardiomyectomy was performed in all patients.All 29 patients survived and their cardiac function recovered after operation.Before discharge,right bundle branch block was observed in 2 patients and left bundle branch block in 6 patients.After surgery,in the left ventricular septal cardiomyectomy,the left atrial diameter decreased(P<0.001),left ventricular end-systolic diameter increased(P=0.009),the peak pressure gradient of left ventricular outflow tract decreased(P<0.001),and the thickness of ventricular septum decreased(P<0.001).The systolic anterior motion of mitral valve disappeared and mitral regurgitent jet area decreased(P<0.001).The flow velocity and peak pressure gradient of right ventricular outflow tract also decreased in the patients who underwent right ventricular septal cardiomyectomy.The average follow-up of the patients was69.03±10.60 months.All the patients survived with their NYHA cardiac function grading Ⅰ or Ⅱ.No new-onset arrythmia event was found.Echocardiography indicated that the peak pressure gradient of the left ventricular outflow tract remained low(P<0.001).Moderate mitral regurgitation occurred in 2 patients,and left ventricular outflow tract obstruction with moderate mitral regurgitation occurred in 1 patient after simple right ventricular septal cardiomyectomy.Conclusion Right ventricular or biventricular obstruction is frequent in the children with HOCM and they usually have more symptoms before surgery.Modified Morrow surgery can effectively relieve outflow tract obstruction and improve their cardiac function.The long-term outcome is satisfactory.However,the posterior wall of the left ventricle remains hypertrophic.Also,there is an increased risk of a conduction block.