Theodoro D A, Danielson G K, Feldt R H, Anderson B J
Division of Thoracic and Cardiovascular Surgery, Mayo Clinic/Foundation, Rochester, Minn. 55905, USA.
J Thorac Cardiovasc Surg. 1996 Dec;112(6):1589-97; discussion 1597-9. doi: 10.1016/S0022-5223(96)70018-1.
Between April 1975 and May 1995, 25 pediatric patients on one hospital service underwent extended left ventricular septal myectomy because of hypertrophic obstructive cardiomyopathy. Ages ranged from 2 months to 20 years (mean, 11.2 years). Seventeen patients had moderate to severe mitral valve insufficiency. Medical therapy had failed in all patients and one patient had undergone dual-chamber pacemaker implantation without improvement. Left ventricular outflow tract gradients ranged from 50 to 154 mm Hg (mean, 99.9 +/- 25.2). Concomitant cardiac procedures included mitral valve repair (n = 2), automatic implantable cardioverter defibrillator implantation (n = 1), and closure of atrial septal defect (n = 1). Intraoperative premyectomy left ventricular outflow tract gradients ranged from 20 to 117 mm Hg (mean, 60.4 + 26.2) and postmyectomy gradients ranged from 0 to 20 mm Hg (mean, 6.6 +/- 5.9). Postmyectomy mitral insufficiency was reduced to a regurgitant fraction of 0% to 12%, and no patient required mitral valve replacement. One patient required a pacemaker because of complete heart block; on subsequent follow-up, normal sinus rhythm had returned. There was no early mortality and no instance of aortic or mitral valve injury or ventricular septal defect. Follow-up ranged from 10 months to 20 years (mean, 6.4 years). There were no late deaths. Left ventricular outflow tract gradients by echocardiography were a mean of 14.2 mm Hg with a median of 5.0 mm Hg. All patients had normal sinus rhythm. Reoperation because of recurrent left ventricular outflow tract obstruction was necessary in two patients at 3.2 years and 12.4 years after initial myectomy, respectively. All patients but one have New York Heart Association class I or II function. We conclude that extended septal myectomy is a safe and effective means of relieving cardiac symptoms and left ventricular outflow tract obstruction in pediatric patients with severe hypertrophic obstructive cardiomyopathy unresponsive to medical management, and late survivorship compares favorably with the natural history of the disease.
1975年4月至1995年5月期间，同一医院科室的25例儿科患者因肥厚性梗阻性心肌病接受了扩大的左心室间隔肌切除术。年龄范围从2个月至20岁（平均11.2岁）。17例患者有中度至重度二尖瓣关闭不全。所有患者药物治疗均失败，1例患者植入双腔起搏器后病情无改善。左心室流出道压力阶差范围为50至154 mmHg（平均99.9±25.2）。同期心脏手术包括二尖瓣修复（2例）、自动植入式心律转复除颤器植入（1例）和房间隔缺损封堵（1例）。术中肌切除术前左心室流出道压力阶差范围为20至117 mmHg（平均60.4 + 26.2），肌切除术后压力阶差范围为0至20 mmHg（平均6.6±5.9）。肌切除术后二尖瓣关闭不全减轻至反流分数为0%至12%，无患者需要二尖瓣置换。1例患者因完全性心脏传导阻滞需要起搏器；在随后的随访中，恢复了正常窦性心律。无早期死亡病例，无主动脉或二尖瓣损伤或室间隔缺损情况。随访时间为10个月至20年（平均6.4年）。无晚期死亡病例。超声心动图显示左心室流出道压力阶差平均为14.2 mmHg，中位数为5.0 mmHg。所有患者均为正常窦性心律。分别在初次肌切除术后3.2年和12.4年，2例患者因左心室流出道梗阻复发需要再次手术。除1例患者外，所有患者纽约心脏协会心功能分级为I级或II级。我们得出结论，扩大的间隔肌切除术是缓解药物治疗无效的重度肥厚性梗阻性心肌病儿科患者心脏症状和左心室流出道梗阻的一种安全有效的方法，晚期生存率与该疾病的自然病程相比具有优势。
