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Contemporary Surgical Management of Hypertrophic Cardiomyopathy, the Need for More Myectomy Surgeons and Disease-Specific Centers, and the Tufts Initiative
Contemporary Surgical Management of Hypertrophic Cardiomyopathy, the Need for More Myectomy Surgeons and Disease-Specific Centers, and the Tufts Initiative Treatment strategies for symptomatic patients with obstructive hypertrophic cardiomyopathy (HC) represent an important component of the overall management armamentarium for this complex genetic disease, and have generated considerable debate in recent years.1e14 Surgical septal myectomy has been the time-honored treatment for patients with severely symptomatic obstructive HC. Myectomy has had a favorable experience extending over almost 50 years,1e4,10,13e23 first with the classic Morrow approach15 and more recently with the modified extended myectomy operation.10,24,25 It is generally acknowledged that septal myectomy has substantially benefited patients with HC over many decades by reliably relieving left ventricular (LV) outflow tract obstruction and normalizing LV pressures (with no or trivial residual gradients), thereby reducing or obliterating heart failure-related symptoms.2,3,10 During the last 15 years, a competing percutaneous interventional technique (alcohol septal ablation) has been performed with considerable frequency, which by design produces a transmural septal infarct to reduce outflow tract gradient and symptoms.8,9,11,12,25,26 In fact, the number of alcohol ablations performed each year worldwide now easily exceeds the number of surgical myectomies. Notably, this exuberance for alcohol ablation paradoxically has increased the number of myectomy operations at referral centers, presumably by enhancing recognition of the clinical significance of outflow obstruction in HC in the practicing community,22,23 but also by demonstrating that there is an adequate volume of potential candidates for myectomy to support more expert surgeons. We do not wish to review in detail here the vast and rapidly accumulating published works comparing myectomy and ablation, now assembled over 15 years. Furthermore, it is highly unlikely that an adequately powered randomized trial could be designed and carried out in obstructive HC to definitively resolve this issue, because of a myriad of virtually insurmountable practical problems, including the low event rate characteristic of this disease, and the necessity to screen as many as >30,000 candidates to enroll >500 suitable study patients in each arm.27 Nevertheless, it should be underscored that the process of comparing myectomy and ablation has in fact been vetted extensively in an unbiased fashion over the last decade— that is, first by the American College of Cardiology and European Society of Cardiology in 2003 as consensus panel recommendations2 and more recently in 2012 by the
Manuscript received June 11, 2013; accepted June 18, 2013. Am J Cardiol 2013;112:1512e1515 0002-9149/13/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved.
American College of Cardiology and American Heart Association as formal clinical practice guidelines.3 Both expert panels reached identical positions regarding the respective indications for myectomy versus alcohol ablation. Surgery is the preferred option for most severely symptomatic drug-refractory patients with obstructive HC, whereas ablation is a selective alternative for older patients or those who are not optimal operative candidates because of associated co-morbidities, profound aversion to surgery, or particularly marked septal hypertrophy.3 The expert panels recognized that although each of these treatment strategies can reduce LV outflow gradient and thereby relieve symptoms, myectomy is most consistent in achieving an optimal hemodynamic and symptomatic result, and with procedural or postprocedural risks similar to (or lower than) that achieved percutaneously with alcohol ablation. With the advantage of a longer follow-up period to study the consequences of HC surgery, it is now recognized that myectomy not only leads to sustained and substantially improved quality of life but also importantly to enhanced survival equivalent to that expected in the general population.10 The Problem: Factors Impeding Availability of Myectomy These observations suggest that greater availability of myectomy would generally be advantageous to the HC patient population. However, a major factor obstructing expansion of the myectomy operation is the relative paucity of surgeons currently available with a sufficient reservoir of experience in performing this particular operation. Indeed, it is largely agreed that experience with myectomy in a sufficient number of patients is essential for the most favorable procedural outcome and that this operation is difficult to perform reliably at the highest level of expertise when undertaken only occasionally.2,3 Certainly, in this regard, the heterogenous LV outflow tract anatomy characteristic of HC requires operator experience28e30 but also creates a distinct advantage for the myectomy surgeon having direct visualization of the complex morphology, compared with interventional alcohol ablation for which the course of the first septal perforator (and therefore, the location of the septal infarct) is fixed. Another historic obstacle to increasing the footprint of surgery in HC is the challenge of “demystifying” myectomy and conveying the “art” and technical acumen necessary to perform the operation expertly. In this regard, surgeonto-surgeon mentorship (as described below for Mayo Clinic and Tufts) is a highly beneficial and desirable strategy. www.ajconline.org
Editorial/Myectomy for HC
These issues are not dissimilar to the dilemma that can arise in selecting surgeons for mitral valve repair.31 Also, we should underscore the often-ignored issue in this myectomy versus alcohol ablation debate, that is, the importance of operator experience in performing alcohol ablation reliably with optimal hemodynamic results, and the likelihood that not all interventional laboratories are equally facile with this technique.3 For many years, surgical myectomy has been confined to a few select centers in North America, for example, Mayo Clinic, Cleveland Clinic, Toronto General, Stanford, Roosevelt-St. Lukes (New York City), most recently the Tufts Medical Center (as described below), and previously the National Heart and Lung Institute (closed by the United States government [Dr. Claude Lenfant] in 1990). However, many surgical candidates cannot (or will not) choose to travel to select centers for myectomy. Therefore, although myectomy is considered the gold standard treatment for most severely symptomatic patients with HC and outflow obstruction by expert consensus panels,2,3 a referral vacuum has nevertheless been created by the relative paucity of experienced surgeons prepared to perform this operation. Consequently, in this regard, 2 separate HC populations have evolved. Some patients with the motivation, resources, and means to travel can be referred to select centers for surgery.32 Many other potential operative candidates, outside of the dedicated HC centers,32 will by default elect the catheterization laboratory and undergo alcohol septal ablation based on local referral patterns, including some patients who may be under the misconception that septal myectomy is no longer an available treatment option.2e4 Although alcohol ablation may be the only septal reduction procedure available at a particular institution, it may not necessarily be the preferred treatment option for each individual patient with HC. Indeed, without new surgical myectomy programs, it is inevitable that many patients eligible for the myectomy operation (the preferred treatment option) as a matter of convenience willdisproportionately become candidates for the alternative therapy (alcohol septal ablation). This unique HC referral scenario is even more pronounced in Europe where septal myectomy has been virtually abandoned for about 20 years, although with some recent evidence of rejuvenation.7,18 Notably, the views expressed here concerning the contemporary management of HC are consistent with the positions of all major cardiovascular societies (American College of Cardiology/American Heart Association/ European Society of Cardiology)—that is, each individual patient with HC should have access to (and the opportunity for) all appropriate treatments.2,3 When patients are presented with only 1 treatment option (e.g., alcohol ablation), they do not, in fact, have an informed choice, and the most effective way for patients to have that choice is through a program focused on HC and segregated from the general cardiology population.
1513
HC Patients and Family Members HC Regional Referral Centers (Clinical Cardiology Director) Comprehensive and Multidisciplinary Disease Management
Family Screening
Genetic Testing/ Interpretation/Genetic Counseling
Invasive Therapies
Advanced Imaging
Contrast-enhanced CMR
Surgical Myectomy
CTA
Alcohol Septal Ablation
Figure 1. Diagnostic and therapeutic strategies available in the dedicated HC Center, which in particular includes both expert surgical myectomy and the option of alcohol ablation. CMR ¼ cardiovascular magnetic resonance; CTA ¼ computed tomography angiography.
A Solution: The Tufts Initiative How can this perceived disparity in management options for patients with HC be rectified? For the septal myectomy operation to continue to flourish, it must have wider accessibility, rather than confinement to only a very few (<5) select centers and surgeons. However, truly widespread availability of myectomy is an unrealistic expectation, given that a sufficient case volume (and operating surgeons) that would be necessary to sustain multiple programs in every possible jurisdiction and venue in the Unites States is lacking. Rather, expanded access to surgery can only be achieved within cardiology divisions with formalized HC programs32 offering specialized disease management and all therapeutic options. Available to patients in the setting of a formalized multidiscipline HC program are: contemporary diagnosis with advanced imaging, genetic testing, risk stratification for sudden death, the option for implantable defibrillator therapy, and importantly the possibility of expert surgical myectomy and alcohol ablation (Figure 1). Furthermore, only in such referral environments can operative candidates be sufficiently clustered to accumulate the critical threshold patient volume to enable the myectomy surgeon to acquire and sustain a necessary level of expertise.3 Therefore, future advancement of the myectomy operation depends on (and is intertwined with) promotion and growth of HC centers. The challenges in formulating such subspecialty programs that afford specialized disease management strategies within cardiology departments include: pre-existing barriers and entrenched referral patterns within the local practice environment, and reticence to refer patients out of the institution for surgery. Also, crucial to this concept of an HC center is an established clinical director knowledgeable in the disease, through which all patients are directed for evaluation to assure that major diagnostic and management decisions are uniform.
1514
The American Journal of Cardiology (www.ajconline.org)
A prime example of a disease-specific HC program incorporating surgical myectomy32 was created de novo within the cardiovascular division of the Tufts Medical Center (Boston) in 2004. During 10 years, the Tufts program has enrolled and evaluated 1,300 patients with HC. During that time, Dr. Hassan Rastegar, initially mentored in the myectomy operation by Dr. Joseph Dearani (Mayo Clinic), has operated on 230 patients with obstructive HC successfully to relieve LV outflow obstruction and disabling heart failure symptoms. Operative mortality is 0.4%, consistent with the current low postoperative death rates at the largest surgical centers (Mayo Clinic, Cleveland Clinic, and Toronto General4), and largely without the morbidity associated with iatrogenic ventricular septal defect, or necessity for pacemaker implant. In addition, 95% of these patients have achieved clinical improvement postoperatively by 1 New York Heart Association functional class, and with 83% returned to an asymptomatic lifestyle, consistent with previous reports from older HC surgical programs.4 Therefore, the experiment at Tufts serves as a model for what can be accomplished in bringing the myectomy option to patients with HC, many of whom would otherwise be excluded from that opportunity. Recently, a few other similar surgical initiatives have proved successful in Europe (e.g., Bergamo, Florence, and Milan in Italy),7,18,21 in addition to the long-standing program at the Thoraxcenter in Rotterdam.26 Furthermore, the formalized multidiscipline and specialized management approach to HC, with patients segregated within selected and experienced centers (with myectomy surgeons) as described here, is perhaps not unlike that currently being advised and promoted for transcatheter aortic valve replacement.33 Based on expert consensus recommendations and extensive clinical experience, surgical septal myectomy is the preferred treatment option for most severely symptomatic patients with obstructive HC. Consequently, greater availability of the myectomy operation performed by surgeons producing optimal hemodynamic and clinical results is a key element in the evolving contemporary management of HC to serve the best interests of the patient population. Novel initiatives such as the medical and surgical program at the Tufts Medical Center reported here demonstrate the potential for fulfilling the aspirations of patients with HC, and their cardiovascular specialists, to create an optimal therapeutic environment. Barry J. Maron, MD Hypertrophic Cardiomyopathy Center Minneapolis Heart Institute Foundation Minneapolis, Minnesota Hassan Rastegar, MD James E. Udelson, MD Hypertrophic Cardiomyopathy Center Tufts Medical Center Boston, Massachusetts
Joseph A. Dearani, MD Mayo Clinic, Rochester, Minnesota Martin S. Maron, MD Hypertrophic Cardiomyopathy Center Tufts Medical Center Boston, Massachusetts 1. Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet 2013;381:242e255. 2. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, ten Cate FJ, Wigle ED. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines committee to Develop an expert consensus Document on hypertrophic cardiomyopathy. J Am Coll Cardiol 2003;42:1687e1713; Eur Heart J 2003;24:1965-1991. 3. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rawkowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 2011;124: e783ee831; J Am Coll Cardiol 2011;58:e212ee260. 4. Maron BJ. Controversies in cardiovascular medicine. Surgical myectomy remains the primary treatment option for severely symptomatic patients with obstructive hypertrophic cardiomyopathy. Circulation 2007;116:196e206. 5. Nishimura RA, Ommen SR. Septal reduction therapy for obstructive hypertrophic cardiomyopathy and sudden death: what statistics cannot tell you. Circ Cardiovasc Interv 2010;3:91e93. 6. Maron BJ. Commentary and re-appraisal: surgical septal myectomy vs. alcohol ablation: after a decade of controversy and mismatch between clinical practice and guidelines. Prog Cardiovasc Dis 2012;54:523e528. 7. Yacoub MH. Surgical versus alcohol septal ablation for hypertrophic obstructive cardiomyopathy: the pendulum swings. Circulation 2005;112:450e452. 8. Leonardi RA, Kransdorf EP, Simel DL, Wang A. Meta-analyses of septal reduction therapies for obstructive hypertrophic cardiomyopathy: comparative rates of overall mortality and sudden cardiac death after treatment. Circ Cardiovasc Interv 2010;3:97e104. 9. Sorajja P, Ommen SR, Holmes DR Jr, Dearani JA, Rihal CS, Gersh BJ, Lennon RJ, Nishimura RA. Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation 2012;126: 2374e2380. 10. Ommen SR, Maron BJ, Olivotto I, Maron MS, Cecchi F, Betocchi S, Gersh BJ, Ackerman MJ, McCully RB, Dearani JA, Schaff HV, Danielson GK, Tajik AJ, Nishimura RA. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol 2005;46:470e476. 11. Argawal S, Tuzcu EM, Desai MY, Smedira N, Lever HM, Lytle BW, Kapadia SR. Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy. J Am Coll Cardiol 2010;55:823e834. 12. Sorajja P, Valeti U, Nishimura RA, Ommen SR, Rihal CS, Gersh BJ, Hodge DO, Schafff HV, Holmes DR. Outcome of alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation 2008;118:131e139. 13. Schulte HD, Bircks WH, Loesse B, Godehardt EA, Schwartzkopff B. Prognosis of patients with hypertrophic obstructive cardiomyopathy after transaortic myectomy. Late results up to twenty-five years. J Thorac Cardiovasc Surg 1993;106:709e717. 14. Maron BJ, Dearani JA, Ommen SR, Maron MS, Schaff HV, Gersh BJ, Nishimura RA. The case for surgery in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol 2004;44:2044e2053. 15. Morrow AG, Reitz BA, Epstein SE, Henry WL, Conkle DM, Itscoitz SB, Redwood DR. Operative treatment in hypertrophic subaortic stenosis. Techniques, and the results of pre and postoperative assessments in 83 patients. Circulation 1975;52:88e102. 16. Woo A, Williams WG, Choi R, Wigle ED, Rozenblyum E, Fedwick K, Siu S, Ralph-Edwards A, Rakowski H. Clinical and echocardiographic
Editorial/Myectomy for HC
17.
18.
19.
20.
21.
22. 23. 24.
25.
26.
determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy. Circulation 2005;111:2033e2041. Ball W, Ivanov J, Rakowski H, Wigle ED, Linghorne M, Ralph-Edwards A, Williams WG, Schwartz L, Guttman A, Woo A. Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment. J Am Coll Cardiol 2011;58:2313e2321. Iacovoni A, Spirito P, Simon C, Iascone M, Di Dedda G, De Filippo P, Pentiricci S, Boni L, Senni M, Gavazzi A, Ferrazzi P. A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy. Eur Heart J 2012;33: 2080e2087. Smedira NG, Lytle BW, Lever HM, Rajeswaran J, Krishnaswamy G, Kaple RK, Dolney DO, Blackstone EH. Current effectiveness and risks of isolated septal myectomy for hypertrophic obstructive cardiomyopathy. Ann Thorac Surg 2008;85:127e133. Altarabsheh SE, Dearani JA, Burkhart HM, Schaff HV, Deo SV, Eidem BW, Ommen SR, Li Z, Ackerman MJ. Outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in children and young adults. Ann Thorac Surg 2013;95:663e669. Maron BJ, Yacoub M, Dearani JA. Controversies in cardiovascular medicine. Benefits of surgery for obstructive hypertrophic cardiomyopathy: bring septal myectomy back for European patients. Eur Heart J 2011;32:1055e1058. Maron BJ. Surgery for hypertrophic obstructive cardiomyopathy: alive and quite well. Circulation 2005;111:2016e2018. Maron BJ, Ommen SR, Nishimura RA, Dearani JA. Myths about surgical myectomy: rumors of its death have been greatly exaggerated. Am J Cardiol 2008;101:887e889. Minakata K, Dearani JA, Nishimura RA, Maron BJ, Danielson GK. Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae. J Thorac Cardiovasc Surg 2004;127:481e489. Maron BJ, Maron MS, Wigle ED, Braunwald E. The 50-year history, controversy, and clinical implications of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy. J Am Coll Cardiol 2009;54:191e200. ten Cate FJ, Soliman OI, Michels M, Theuns DA, de Jong PL, Geleijnse ML, Serruys PW. Long-term outcome of alcohol septal
27.
28.
29.
30.
31.
32. 33.
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ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution. Circ Heart Fail 2010;3:362e369. Olivotto I, Ommen SR, Maron MS, Cecchi F, Maron BJ. Surgical myectomy versus alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Will there ever be a randomized trial? J Am Coll Cardiol 2007;50:831e834. Maron MS, Maron BJ, Harrigan C, Buros J, Gibson CM, Olivotto I, Biller L, Lesser JR, Udelson JE, Manning WJ, Appelbaum E. Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance. J Am Coll Cardiol 2009;54: 220e228. Klues HG, Roberts WC, Maron BJ. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction. Circulation 1991;84:1188e1197. Maron BJ, Nishimura RA, Danielson GK. Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction due to anomalous papillary muscle. Circulation 1998;98:2505e2508. Bonow RO, Carabello BA, Kanu C, de Leon AC Jr, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O’Gara PT, O’Rourke RA, Otto CM, Shah PM, Shanewise JS, Smith SC Jr, Jacobs AK, Adams CD, Anderson JL, Antman EM, Faxon DP, Fuster V, Halperin JL, Hiratzka LF, Hunt SA, Lytle BW, Nishimura R, Page RL, Riegel B. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients with Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation 2006;114:e84ee231. Maron BJ. Hypertrophic cardiomyopathy centers. Am J Cardiol 2009;104:1158e1159. Bakaeen FG, Kar B, Chu D, Cornwell LD, Blaustein A, Levine GN, Paniagua D, Jneid H, Jensen C, Atluri PV, Bechara CF, Kougias P, Pawlak C, Bozkurt B, Burdon TA, Carabello BA. Establishment of a transcatheter aortic valve program and heart valve team at a Veterans Affairs facility. Am J Surg 2012;204:643e648.
Manuscript received June 11, 2013; accepted June 18, 2013. Am J Cardiol 2013;112:1512e1515 0002-9149/13/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved.
American College of Cardiology and American Heart Association as formal clinical practice guidelines.3 Both expert panels reached identical positions regarding the respective indications for myectomy versus alcohol ablation. Surgery is the preferred option for most severely symptomatic drug-refractory patients with obstructive HC, whereas ablation is a selective alternative for older patients or those who are not optimal operative candidates because of associated co-morbidities, profound aversion to surgery, or particularly marked septal hypertrophy.3 The expert panels recognized that although each of these treatment strategies can reduce LV outflow gradient and thereby relieve symptoms, myectomy is most consistent in achieving an optimal hemodynamic and symptomatic result, and with procedural or postprocedural risks similar to (or lower than) that achieved percutaneously with alcohol ablation. With the advantage of a longer follow-up period to study the consequences of HC surgery, it is now recognized that myectomy not only leads to sustained and substantially improved quality of life but also importantly to enhanced survival equivalent to that expected in the general population.10 The Problem: Factors Impeding Availability of Myectomy These observations suggest that greater availability of myectomy would generally be advantageous to the HC patient population. However, a major factor obstructing expansion of the myectomy operation is the relative paucity of surgeons currently available with a sufficient reservoir of experience in performing this particular operation. Indeed, it is largely agreed that experience with myectomy in a sufficient number of patients is essential for the most favorable procedural outcome and that this operation is difficult to perform reliably at the highest level of expertise when undertaken only occasionally.2,3 Certainly, in this regard, the heterogenous LV outflow tract anatomy characteristic of HC requires operator experience28e30 but also creates a distinct advantage for the myectomy surgeon having direct visualization of the complex morphology, compared with interventional alcohol ablation for which the course of the first septal perforator (and therefore, the location of the septal infarct) is fixed. Another historic obstacle to increasing the footprint of surgery in HC is the challenge of “demystifying” myectomy and conveying the “art” and technical acumen necessary to perform the operation expertly. In this regard, surgeonto-surgeon mentorship (as described below for Mayo Clinic and Tufts) is a highly beneficial and desirable strategy. www.ajconline.org
Editorial/Myectomy for HC
These issues are not dissimilar to the dilemma that can arise in selecting surgeons for mitral valve repair.31 Also, we should underscore the often-ignored issue in this myectomy versus alcohol ablation debate, that is, the importance of operator experience in performing alcohol ablation reliably with optimal hemodynamic results, and the likelihood that not all interventional laboratories are equally facile with this technique.3 For many years, surgical myectomy has been confined to a few select centers in North America, for example, Mayo Clinic, Cleveland Clinic, Toronto General, Stanford, Roosevelt-St. Lukes (New York City), most recently the Tufts Medical Center (as described below), and previously the National Heart and Lung Institute (closed by the United States government [Dr. Claude Lenfant] in 1990). However, many surgical candidates cannot (or will not) choose to travel to select centers for myectomy. Therefore, although myectomy is considered the gold standard treatment for most severely symptomatic patients with HC and outflow obstruction by expert consensus panels,2,3 a referral vacuum has nevertheless been created by the relative paucity of experienced surgeons prepared to perform this operation. Consequently, in this regard, 2 separate HC populations have evolved. Some patients with the motivation, resources, and means to travel can be referred to select centers for surgery.32 Many other potential operative candidates, outside of the dedicated HC centers,32 will by default elect the catheterization laboratory and undergo alcohol septal ablation based on local referral patterns, including some patients who may be under the misconception that septal myectomy is no longer an available treatment option.2e4 Although alcohol ablation may be the only septal reduction procedure available at a particular institution, it may not necessarily be the preferred treatment option for each individual patient with HC. Indeed, without new surgical myectomy programs, it is inevitable that many patients eligible for the myectomy operation (the preferred treatment option) as a matter of convenience willdisproportionately become candidates for the alternative therapy (alcohol septal ablation). This unique HC referral scenario is even more pronounced in Europe where septal myectomy has been virtually abandoned for about 20 years, although with some recent evidence of rejuvenation.7,18 Notably, the views expressed here concerning the contemporary management of HC are consistent with the positions of all major cardiovascular societies (American College of Cardiology/American Heart Association/ European Society of Cardiology)—that is, each individual patient with HC should have access to (and the opportunity for) all appropriate treatments.2,3 When patients are presented with only 1 treatment option (e.g., alcohol ablation), they do not, in fact, have an informed choice, and the most effective way for patients to have that choice is through a program focused on HC and segregated from the general cardiology population.
1513
HC Patients and Family Members HC Regional Referral Centers (Clinical Cardiology Director) Comprehensive and Multidisciplinary Disease Management
Family Screening
Genetic Testing/ Interpretation/Genetic Counseling
Invasive Therapies
Advanced Imaging
Contrast-enhanced CMR
Surgical Myectomy
CTA
Alcohol Septal Ablation
Figure 1. Diagnostic and therapeutic strategies available in the dedicated HC Center, which in particular includes both expert surgical myectomy and the option of alcohol ablation. CMR ¼ cardiovascular magnetic resonance; CTA ¼ computed tomography angiography.
A Solution: The Tufts Initiative How can this perceived disparity in management options for patients with HC be rectified? For the septal myectomy operation to continue to flourish, it must have wider accessibility, rather than confinement to only a very few (<5) select centers and surgeons. However, truly widespread availability of myectomy is an unrealistic expectation, given that a sufficient case volume (and operating surgeons) that would be necessary to sustain multiple programs in every possible jurisdiction and venue in the Unites States is lacking. Rather, expanded access to surgery can only be achieved within cardiology divisions with formalized HC programs32 offering specialized disease management and all therapeutic options. Available to patients in the setting of a formalized multidiscipline HC program are: contemporary diagnosis with advanced imaging, genetic testing, risk stratification for sudden death, the option for implantable defibrillator therapy, and importantly the possibility of expert surgical myectomy and alcohol ablation (Figure 1). Furthermore, only in such referral environments can operative candidates be sufficiently clustered to accumulate the critical threshold patient volume to enable the myectomy surgeon to acquire and sustain a necessary level of expertise.3 Therefore, future advancement of the myectomy operation depends on (and is intertwined with) promotion and growth of HC centers. The challenges in formulating such subspecialty programs that afford specialized disease management strategies within cardiology departments include: pre-existing barriers and entrenched referral patterns within the local practice environment, and reticence to refer patients out of the institution for surgery. Also, crucial to this concept of an HC center is an established clinical director knowledgeable in the disease, through which all patients are directed for evaluation to assure that major diagnostic and management decisions are uniform.
1514
The American Journal of Cardiology (www.ajconline.org)
A prime example of a disease-specific HC program incorporating surgical myectomy32 was created de novo within the cardiovascular division of the Tufts Medical Center (Boston) in 2004. During 10 years, the Tufts program has enrolled and evaluated 1,300 patients with HC. During that time, Dr. Hassan Rastegar, initially mentored in the myectomy operation by Dr. Joseph Dearani (Mayo Clinic), has operated on 230 patients with obstructive HC successfully to relieve LV outflow obstruction and disabling heart failure symptoms. Operative mortality is 0.4%, consistent with the current low postoperative death rates at the largest surgical centers (Mayo Clinic, Cleveland Clinic, and Toronto General4), and largely without the morbidity associated with iatrogenic ventricular septal defect, or necessity for pacemaker implant. In addition, 95% of these patients have achieved clinical improvement postoperatively by 1 New York Heart Association functional class, and with 83% returned to an asymptomatic lifestyle, consistent with previous reports from older HC surgical programs.4 Therefore, the experiment at Tufts serves as a model for what can be accomplished in bringing the myectomy option to patients with HC, many of whom would otherwise be excluded from that opportunity. Recently, a few other similar surgical initiatives have proved successful in Europe (e.g., Bergamo, Florence, and Milan in Italy),7,18,21 in addition to the long-standing program at the Thoraxcenter in Rotterdam.26 Furthermore, the formalized multidiscipline and specialized management approach to HC, with patients segregated within selected and experienced centers (with myectomy surgeons) as described here, is perhaps not unlike that currently being advised and promoted for transcatheter aortic valve replacement.33 Based on expert consensus recommendations and extensive clinical experience, surgical septal myectomy is the preferred treatment option for most severely symptomatic patients with obstructive HC. Consequently, greater availability of the myectomy operation performed by surgeons producing optimal hemodynamic and clinical results is a key element in the evolving contemporary management of HC to serve the best interests of the patient population. Novel initiatives such as the medical and surgical program at the Tufts Medical Center reported here demonstrate the potential for fulfilling the aspirations of patients with HC, and their cardiovascular specialists, to create an optimal therapeutic environment. Barry J. Maron, MD Hypertrophic Cardiomyopathy Center Minneapolis Heart Institute Foundation Minneapolis, Minnesota Hassan Rastegar, MD James E. Udelson, MD Hypertrophic Cardiomyopathy Center Tufts Medical Center Boston, Massachusetts
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