Kotkar Kunal D, Said Sameh M, Dearani Joseph A, Schaff Hartzell V
Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
Ann Cardiothorac Surg. 2017 Jul;6(4):329-336. doi: 10.21037/acs.2017.07.03.
Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease characterized by left ventricular hypertrophy in the absence of other etiologies. Clinical presentation may vary from asymptomatic to sudden cardiac death. Medical treatment is the first-line therapy for symptomatic patients. Extended left ventricular septal myectomy is the procedure of choice if medical treatment is unsuccessful or intolerable.
More than 3,000 patients have had septal myectomy for HCM at the Mayo Clinic (MN, USA) from 1993 to 2016. Risk of hospital death after isolated septal myectomy for obstructive HCM is <1% and is similar to the risk of operation for elective mitral valve repair. Complications, such as complete heart block requiring permanent pacemaker, are uncommon (2%), although partial or complete left bundle branch block is a frequent finding on the postoperative ECG. Relief of left ventricular outflow tract (LVOT) obstruction with septal myectomy dramatically improves symptoms and exercise capacity in symptomatic patients with obstructive HCM. More than 90% of severely symptomatic patients have improvement by at least two functional classes, and reduction of outflow gradients by myectomy decreases or eliminates symptoms of dyspnea, angina and/or syncope. Basal obstruction with systolic anterior motion (SAM) is treated by transaortic myectomy. The transapical approach was applied in 115 patients with obstructive midventricular and apical variants of HCM between 1993 and 2012. All patients with midventricular obstruction had gradient relief and none developed an apical aneurysm or ventricular septal defect. Recurrent obstruction after satisfactory myectomy was rare.
Septal myectomy effectively and definitively relieves LVOT obstruction and cardiac symptoms in patients with obstructive HCM. In experienced centers, early mortality for isolated septal myectomy is less than 1%, and overall results are excellent and continue to improve in the current era.
肥厚型心肌病（HCM）是一种原发性心肌疾病，其特征为在无其他病因的情况下出现左心室肥厚。临床表现可从无症状到心源性猝死不等。药物治疗是有症状患者的一线治疗方法。如果药物治疗不成功或无法耐受，扩大的左心室间隔肌切除术是首选手术。
1993年至2016年期间，超过3000例患者在美国明尼苏达州梅奥诊所接受了针对HCM的间隔肌切除术。梗阻性HCM单纯间隔肌切除术后的院内死亡风险<1%，与择期二尖瓣修复手术的风险相似。并发症，如需要永久起搏器的完全性心脏传导阻滞并不常见（2%），尽管术后心电图上经常发现部分或完全性左束支传导阻滞。间隔肌切除术缓解左心室流出道（LVOT）梗阻可显著改善梗阻性HCM有症状患者的症状和运动能力。超过90%的重度有症状患者功能分级至少改善两级，肌切除术降低流出道梯度可减轻或消除呼吸困难、心绞痛和/或晕厥症状。伴有收缩期前向运动（SAM）的基底梗阻通过经主动脉肌切除术治疗。1993年至2012年期间，115例梗阻性心室中部和心尖部变异型HCM患者采用了经心尖入路。所有心室中部梗阻患者的梯度均得到缓解，且无一例发生心尖部动脉瘤或室间隔缺损。满意的肌切除术后复发梗阻很少见。
间隔肌切除术可有效且确切地缓解梗阻性HCM患者的LVOT梗阻和心脏症状。在经验丰富的中心，单纯间隔肌切除术的早期死亡率低于1%，总体效果良好，且在当今时代仍在不断改善。
