肥厚型梗阻性心肌病的手术治疗
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摘要
肥厚型梗阻性心肌病(HOCM)是一种显性遗传性疾病,肥厚型梗阻性心肌病是原发性心肌病中较常见的一种类型,是肥厚型心肌病的一种特殊类型。本病常有明显家族史(约30%),常为青年发生猝死的原因。在1952年Davie报道家9位兄弟姐妹,其中有5人得此病,5人中3人发生猝死。梗阻通常发生于主动脉瓣下或心室中部,梗阻症状可以隐匿、间歇或持续存在,一些病人合并二尖瓣前叶收缩期异常的前向运动即：SAM现象。其病变特征是血流动力学异常,主要表现为左心室容积缩小,造成左心室间隔与二尖瓣前叶之间的距离显著缩短,当心室收缩时二尖瓣前叶前向运动(SAM),从而加重左室流出道(LVOT)梗阻程度。肥厚型梗阻性心肌病临床表现主要为劳累性心悸、气短、心前区疼痛,甚至晕厥。晚期常伴有严重的进行性充血性心力衰竭,常由于严重流出道梗阻和并发心房纤维颤动引起。肥厚型梗阻性心肌病患者80%有劳累性呼吸困难,约2／3患者有非典型心绞痛,约1／3患者有先兆晕厥或晕厥,多在活动后发生。肥厚型梗阻性心肌病(HOCM)又称特发性肥厚型主动脉瓣下狭窄(IHSS),主要病理改变为室间隔及左室游离壁非对称性增厚,导致左室流出道排血受阻。肥厚型梗阻性心肌病的外科治疗已有近40年的历史,但最初的手术效果并不十分满意。近年来,随着其病理生理的进一步认识,外科矫正的观念和方法也随之发生了很大变化。
目的对有症状的肥厚型梗阻性心肌病的病人最有效的治疗方法仍存在争议。治疗方法包括药物治疗、双腔起搏治疗、经皮腔内室间隔心肌化学消融术、外科手术切除部分肥厚的心肌。各种治疗方法的长期效果不是很好界定。我们目的是探讨肥厚型梗阻性心肌病手术治疗的效果、手术方法及手术适应症。
方法回顾分析2000年10月至2009年12月手术治疗10例HOCM病人。其中9例部分肥厚室间隔切除或切开及1例部分肥厚室间隔切除同期行二尖瓣置换术。
结果10例手术均获得成功无1例死亡。术后左室流出道压差(LVOTPG)下降较术前10例均>60%.静息左室流出道压差(LVOTPG)由(68.40±16.12)mmHg下降为(16.65±12.75)mmHg (p<0.01),负荷左室流出道压差(LVOTPG)由(110.44±34.21)mmHg下降为(31.85±22.64)mmHg (p<0.01)。室间隔厚度由(23.44±2.51)mm下降为(11.35±1.73)mm(p<0.05)。左室流出道内径由(10.93±3.31)mm增加为(19.17±3.64)mm(p<0.05)。所有病人症状消失(晕厥、头晕、心绞痛、气喘),胸骨左缘杂音消失或减轻,震颤消失,心尖区杂音消失。心功能均恢复到Ⅰ-Ⅱ级。1例出现完全性房室传导阻滞,安装起搏器。超声心动图示室间隔变薄,二尖瓣反流明显减轻或消失,SAM现象消失,术后随访3个月至8年,无1例死亡,均能正常工作。
结论手术治疗通过切除部分肥厚室间隔能有效解除左室流出道梗阻,压差明显下降,可以使许多病人得到长期症状改善。目前认为室间隔部分肥厚心肌切除术是治疗症状性肥厚型梗阻性心肌病的第一选择的方法。
Hypertrophic obstructive cardiomyopathy (HOCM) is an autosomal dominant genetic disease. Hypertrophic obstructive cardiomyopathy is the more common type of primary Cardiomyopathy. Hypertrophic obstructive cardiomyopathy is a special type of hypertrophic cardiomyopathy.The disease often have significant family history (about 30%) which often causes sudden death for young people.Davies reported that five of nine brothers and sisters of one family had the disease in 1952,three of them arise sudden death.Obstruction usually occurs in the infer-aortic valve or the central of ventricle under, obstructive symptoms can be hidden, intermittent or persistent, the merger abnormal systolic anterior mitral valve prior to the movement that is:SAM phenomenon.The lesion is characterized by hemodynamic abnormalities, mainly for left ventricular volume reduced, resulting in left ventricular interval and the distance between the anterior mitral leaflet significantly shortened the contraction of careful room prior to the anterior mitral leaflet movement (SAM), thereby increasing the left ventricular outflow tract (LVOT) obstruction degree. The main clinical of Hypertrophic obstructive cardiomyopathy is manifestations of exertional palpitations, shortness of breath, chest pain, and even syncope. Frequently the patients suffer from the conduct of advanced congestive heart failure, often due to severe outflow tract obstruction and concurrent atrial fibrillation caused. The 80% of Hypertrophic obstructive cardiomyopathy patients have exertional dyspnea, about 2/3 of patients have atypical angina, about 1/3 of patients have threatened syncope or syncope, often after the event occurred. The time of operation treatment of Hypertrophic Obstructive Cardiomyopathy has had near 40 years,but the initial effect was not satisfied. In recent years, along with the physiopathology of Hypertrophic Obstructive Cardiomyopathy further understanding, The concepts and methods of surgical correction also will be changed a lot
Objective The most effective treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy is still disputed. Treatment options include medical therapy, pacemaker insertion, percutaneous transluminal septal myocardial ablation, mitral valve replacement, and surgical resection of obstructing muscle. To investigate the effective、means and indication of Operation Treatment of Hypertrophic Obstructive Cardiomyopathy.
Methods Ten patients with Hypertrophic Obstructive Cardiomyopathy were operated upon at the First Affiliated hospital of Zhengzhou University between Oct.2000 and Dec.2008.Myotomy and myectomy of interventricular septum by an aortic approach were performed ten patients and simultaneous mitral valve replacement through atrial incis in one of ten patients.
Results Ten cases were successful and no one died. Left ventricular outfiow tract pressure gradient(LVOTPG) was reduced over 60%after operating. The resting LVOTPG was reduced from (68.40±16.12) mmHg to (16.65±12.75)mmHg (p<0.01).The Provoked LVOTPG was reduced from(110.44±34.21) mmHg to (31.85±22.64)mmHg (p<0.01)。The thickness of heart ventricle septal decreased from (23.44±2.51)mm to (11.35±1.73)mm (p<0.05)。The inner diameter of LVOT was increased from (10.93±3.31)mm to (19.17±3.64) mm (p<0.05)。The clinical symptoms such as syncope, dizziness, angina, dyspnea and so on disappeared in all patients. The noise of the left border of breast bone disappeared or reduced,the chatter disappeared.The noise of apical region disappeared. The heart function of all patients recovered to theⅠ-Ⅱclass. One patient required permanent pacemaker because of the complete atrioventricular block. The thickness of heart ventricle septal thinningzed, the mitral regurgitation obviously decreased or disappeared and the phenomenon of SAM disappeared by ECHO.The time of follow-up visiting was from three months to eight years, no one died.All patients can normal work.
Conclusion Left ventricular outflow tract obstruction can be effectively relieved by Operation Treatment. The gradient pressure apparently came down.It can improve the symptom of many patiens with hypertrophic obstructive cardiomyopathy in long term. At present we think that surgical part resection of obstructing muscle is the first choice which treats the symptomatic patients with hypertrophic obstructive cardiomyopathy.
目的对有症状的肥厚型梗阻性心肌病的病人最有效的治疗方法仍存在争议。治疗方法包括药物治疗、双腔起搏治疗、经皮腔内室间隔心肌化学消融术、外科手术切除部分肥厚的心肌。各种治疗方法的长期效果不是很好界定。我们目的是探讨肥厚型梗阻性心肌病手术治疗的效果、手术方法及手术适应症。
方法回顾分析2000年10月至2009年12月手术治疗10例HOCM病人。其中9例部分肥厚室间隔切除或切开及1例部分肥厚室间隔切除同期行二尖瓣置换术。
结果10例手术均获得成功无1例死亡。术后左室流出道压差(LVOTPG)下降较术前10例均>60%.静息左室流出道压差(LVOTPG)由(68.40±16.12)mmHg下降为(16.65±12.75)mmHg (p<0.01),负荷左室流出道压差(LVOTPG)由(110.44±34.21)mmHg下降为(31.85±22.64)mmHg (p<0.01)。室间隔厚度由(23.44±2.51)mm下降为(11.35±1.73)mm(p<0.05)。左室流出道内径由(10.93±3.31)mm增加为(19.17±3.64)mm(p<0.05)。所有病人症状消失(晕厥、头晕、心绞痛、气喘),胸骨左缘杂音消失或减轻,震颤消失,心尖区杂音消失。心功能均恢复到Ⅰ-Ⅱ级。1例出现完全性房室传导阻滞,安装起搏器。超声心动图示室间隔变薄,二尖瓣反流明显减轻或消失,SAM现象消失,术后随访3个月至8年,无1例死亡,均能正常工作。
结论手术治疗通过切除部分肥厚室间隔能有效解除左室流出道梗阻,压差明显下降,可以使许多病人得到长期症状改善。目前认为室间隔部分肥厚心肌切除术是治疗症状性肥厚型梗阻性心肌病的第一选择的方法。
Hypertrophic obstructive cardiomyopathy (HOCM) is an autosomal dominant genetic disease. Hypertrophic obstructive cardiomyopathy is the more common type of primary Cardiomyopathy. Hypertrophic obstructive cardiomyopathy is a special type of hypertrophic cardiomyopathy.The disease often have significant family history (about 30%) which often causes sudden death for young people.Davies reported that five of nine brothers and sisters of one family had the disease in 1952,three of them arise sudden death.Obstruction usually occurs in the infer-aortic valve or the central of ventricle under, obstructive symptoms can be hidden, intermittent or persistent, the merger abnormal systolic anterior mitral valve prior to the movement that is:SAM phenomenon.The lesion is characterized by hemodynamic abnormalities, mainly for left ventricular volume reduced, resulting in left ventricular interval and the distance between the anterior mitral leaflet significantly shortened the contraction of careful room prior to the anterior mitral leaflet movement (SAM), thereby increasing the left ventricular outflow tract (LVOT) obstruction degree. The main clinical of Hypertrophic obstructive cardiomyopathy is manifestations of exertional palpitations, shortness of breath, chest pain, and even syncope. Frequently the patients suffer from the conduct of advanced congestive heart failure, often due to severe outflow tract obstruction and concurrent atrial fibrillation caused. The 80% of Hypertrophic obstructive cardiomyopathy patients have exertional dyspnea, about 2/3 of patients have atypical angina, about 1/3 of patients have threatened syncope or syncope, often after the event occurred. The time of operation treatment of Hypertrophic Obstructive Cardiomyopathy has had near 40 years,but the initial effect was not satisfied. In recent years, along with the physiopathology of Hypertrophic Obstructive Cardiomyopathy further understanding, The concepts and methods of surgical correction also will be changed a lot
Objective The most effective treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy is still disputed. Treatment options include medical therapy, pacemaker insertion, percutaneous transluminal septal myocardial ablation, mitral valve replacement, and surgical resection of obstructing muscle. To investigate the effective、means and indication of Operation Treatment of Hypertrophic Obstructive Cardiomyopathy.
Methods Ten patients with Hypertrophic Obstructive Cardiomyopathy were operated upon at the First Affiliated hospital of Zhengzhou University between Oct.2000 and Dec.2008.Myotomy and myectomy of interventricular septum by an aortic approach were performed ten patients and simultaneous mitral valve replacement through atrial incis in one of ten patients.
Results Ten cases were successful and no one died. Left ventricular outfiow tract pressure gradient(LVOTPG) was reduced over 60%after operating. The resting LVOTPG was reduced from (68.40±16.12) mmHg to (16.65±12.75)mmHg (p<0.01).The Provoked LVOTPG was reduced from(110.44±34.21) mmHg to (31.85±22.64)mmHg (p<0.01)。The thickness of heart ventricle septal decreased from (23.44±2.51)mm to (11.35±1.73)mm (p<0.05)。The inner diameter of LVOT was increased from (10.93±3.31)mm to (19.17±3.64) mm (p<0.05)。The clinical symptoms such as syncope, dizziness, angina, dyspnea and so on disappeared in all patients. The noise of the left border of breast bone disappeared or reduced,the chatter disappeared.The noise of apical region disappeared. The heart function of all patients recovered to theⅠ-Ⅱclass. One patient required permanent pacemaker because of the complete atrioventricular block. The thickness of heart ventricle septal thinningzed, the mitral regurgitation obviously decreased or disappeared and the phenomenon of SAM disappeared by ECHO.The time of follow-up visiting was from three months to eight years, no one died.All patients can normal work.
Conclusion Left ventricular outflow tract obstruction can be effectively relieved by Operation Treatment. The gradient pressure apparently came down.It can improve the symptom of many patiens with hypertrophic obstructive cardiomyopathy in long term. At present we think that surgical part resection of obstructing muscle is the first choice which treats the symptomatic patients with hypertrophic obstructive cardiomyopathy.
引文
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[1]Marian aj, Roberts R. The molecular genetic basis for hypertrophic cardiomyopathy[J]. J MolCell Cardiol,2002,33:655-670.
[2]徐敬,赵文增,孙宏涛等.主动脉瓣置换术后的SAM现象[J].中华心胸外科杂志,2001,6：335～336.
[3]Spirito P, Autore C. Management of hypertrophic cardiomyopath [J].BMJ,2006,332(7): 1251-1255.
[4]朱晓东,张宝仁.心脏外科学[M].北京：人民卫生出社,2007：1249-1256.
[5]刘玉清,杨涴宜,李坤成.心血管病影像诊断学[M].合肥：安徽科学技术出版社,2000,289-299.
[6]Borja J, Izquierdo J, Guindo J. Hypertrophic cardiomyopathy:combination ofβblockers and verapamilmay be risky [J].BMJ,2006,333 (7552):97-100.
[7]Maron MS, Gohman TE, Casey SA, et al. Significance and relationship between magnitude of left ventricular hypertrophy and congestive heart failure symptoms in hypertrophic cardiomyopathy [J].J Am Coll Cardiol,2002,39:173～177..
[8]Borja J, Izquierdo J, Guindo J. Hypertrophic cardiomyopathy:combination ofβblockers and verapamilmay be risky [J]. BMJ,2006,333 (7558):97-100.
[9]Maron BJ,McKennaWJ,Danielson GK, et al. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy [J]. Eur Heart J,2003,24 (21):1965～1991.
[10]Tseday E. Sirak, Mark V. Sherrid.Oral. Disopyramide for the Acute Treatment of Severe Outflow Obstruction in Hypertrophic Cardiomyopathy in the ICU Setting [J].Chest,2008, 133:1243-1246.
[11]中华医学会心血管病学分会,中国心肌病诊断与治疗建议工作组.心肌病诊断与治疗建议[J].中华心血管病杂志,2007,35(1)：5-16.
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[14]Enrique Galve, Antonia Sambola, German Saldana, et al. Late benefits of dual-chamber pacing in obstructive hypertrophic cardiomyopathy. A 10-year follow-up study [J]. Heart, 2010,96(3):352～356.
[15]Spirito P, Autore C. Management of hypertrophic cardiomyopathy [J]. BMJ,2006,332: 1251-1255.
[16]Eric Durand, Elie Mousseaux, Pierre Coste, et al. Non-surgical septal myocardial reduction by coil embolization for hypertrophic obstructive cardiomyopathy:early and 6 months follow-up[J]. Eur. Heart J.,2008; 29 (2):348～355.
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[18]Yusuke Hosokawa, Hitoshi Takano, Tadaaki Ohno. Impact of Percutaneous Transluminal Septal Myocardial Ablation on Refractory Paroxysmal Atrial Fibrillation in Patients With Hypertrophic Obstructive Cardiomyopathy[J]. Angiology,2008,59 (1):329～334.
[19]Mahboob Alam, Hisham Dokainish,Nasser M. Lakkis. Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs. myectomy:a meta-analysis[J]. Eur. Heart J, 2009,30 (3):1080-1087.
[20]Paul Sorajja, Uma Valeti, Rick A. Nishimura, et al. Outcome of Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy[J]. Circulation,2008,118 (7):131 ～ 139.
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[18]Yusuke Hosokawa, Hitoshi Takano, Tadaaki Ohno. Impact of Percutaneous Transluminal Septal Myocardial Ablation on Refractory Paroxysmal Atrial Fibrillation in Patients With Hypertrophic Obstructive Cardiomyopathy[J]. Angiology,2008,59 (1):329～334.
[19]Mahboob Alam, Hisham Dokainish,Nasser M. Lakkis. Hypertrophic obstructive cardiomyopathy-alcohol septal ablation vs. myectomy:a meta-analysis[J]. Eur. Heart J, 2009,30 (3):1080-1087.
[20]Paul Sorajja, Uma Valeti, Rick A. Nishimura, et al. Outcome of Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy[J]. Circulation,2008,118 (7):131 ～ 139.
[21]P Steendijk, E Meliga, M Valgimigli, Acute effects of alcohol septal ablation on systolic and diastolic left ventricular function in patients with hypertrophic obstructive cardiomyopathy[J]. Heart,2008,94 (10):1318～1322.
[22]W A Jaber, E H Yang, R A Nishimura. Immediate improvement in coronary flow reserve after alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy[J]. Heart, 2009,95 (4):564-569.
[23]Walter H. Merrill, Gottlieb C. Friesinger, Thomas P. Graham, et al. Long-Lasting Improvement After Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy[J]. Ann Thorac Surg,2000,69(6):1732-1735.
[24]Jeanne L. Theis, J. Martijn Bos, Jason D. Theis, et al. Expression Patterns of Cardiac Myofilament Proteins:Genomic and Protein Analysis of Surgical Myectomy Tissue From Patients With Obstructive Hypertrophic Cardiomyopathy[J]. Circ Heart Fail,2009,2(7): 325～333.
[25]顾恺时.顾恺时胸心外科手术学[M].上海：上海科学技术出版社,2003.1409-1418.
[26]Iacopo Olivotto, Steve R. Ommen, Martin S. Maron, et al. Surgical Myectomy Versus Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy:Will There Ever Be a Randomized Trial[J]? J. Am. Coll. Cardiol,2007,50(8):831～834.
[27]Tsutomu Matsushita, Tetsunori Kawase, Etsuko Tsuda, et al. Apicoaortic conduit for the dilated phase of hypertrophic obstructive cardiomyopathy as an alternative to heart transplantation[J]. Interact CardioVasc Thorac Surg,2009,8(1):232 ～234.
[28]Tsutomu Matsushita, Tetsunori Kawase, Etsuko Tsuda, et al. Apicoaortic conduit for the dilated phase of hypertrophic obstructive cardiomyopathy as an alternative to heart transplantation[J]. Interact CardioVasc Thorac Surg,2009,8(1):232～234.
[29]Calvin K.N. Wan, Joseph A. Dearani, Thoralf M. Sundt et al., What Is the Best Surgical Treatment for Obstructive Hypertrophic Cardiomyopathy and Degenerative Mitral Regurgitation [J]? Ann. Thorac. Surg.,2009,88(9):727～732.
[30]Sandhya K. Balaram, Mark V. Sherrid, Joseph J. Derose, etal. Beyond Extended Myectomy for Hypertrophic Cardiomyopathy:The Resection-Plication-Release (RPR) Repair[J]. Ann Thorac Surg,2005,80(23):217～223.
[31]Paolo Ferrazzi, Michele Triggiani, Attilio Iacovoni, et al. Debating About a Registry to Define the Best Invasive Treatment for Obstructive Hypertrophic Cardiomyopathy:Should It Also Include Obstructive Patients Medically Treated[J]? J. Am. Coll. Cardiol.2008,51(12): 1233-1234.
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[36]Kenji Minakata, Joseph A. Dearani, Rick A. Nishimura, et al. Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae [J]. J Thorac Cardiovasc Surg,2004,127:481～489.
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[38]Steve R. Ommen, Barry J. Maron, Iacopo Olivotto, et al. Long-Term Effects of Surgical Septal Myectomy on Survival in Patients With Obstructive Hypertrophic Cardiomyopathy [J]. J.Am. Coll. Cardiol,2005,46 (3):470-476.
[39]Qin JX, Shiota T, Lever HM, et al. Outcome of patients with hypertrophic obstructive cardiomyopathy after percutaneous transluminal septal myocardial ablation and septal myectomy surgery [J]. J Am Coll Cardiol,2001,38:1994～2000.
[40]Ommen SR, Park SH, Click RL, et al.Impact of intraoperative transesophageal echocardiography in the surgical management of hypertrophic cardiomyopathy [J]. J Am Cardiol2002;90:1022～1024.
[41]Nagueh SF, Ommen SR, Lakkis NM, et al. Comparison of ethanol septal reduction therapy with surgical myectomy for the treatment of hypertrophic obstructive cardiomyopathy [J]. J Am Coll Cardiol,2001,38:1701 ～ 1706.
[42]Woo A, Williams WG, Choi R, et al. Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy[J]. Circulation,2005,111:2033-2041.
[43]Deb SJ, Schaff HV, Dearani JA, Nishimura RA, et al.Septal myectomy results in regression of left ventricular hypertrophy in patients with hypertrophic obstructive cardiomyopathy[J].. Ann Thorac Surg.,2004,78:2118～2122.
[44]Kenji Minakata, Joseph A. Dearani, Hartzell V. Schaff, et al. Mechanisms for Recurrent Left Ventricular Outflow Tract Obstruction After Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy[J]. Ann Thorac Surg,2005,80:851-856.
[45]Earani JA, Danielson GK. Surgery for hypertrophic obstructive cardiomyopathy[J]. Oper Techn Thorac CardiovascSurg,2004,9:278～292.