Hypertrophic cardiomyopathy invasive therapy guidelines
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D. [2]; Caitlin J. Harrigan [3]; Martin S. Maron, M.D.; Barry J. Maron, M.D.; Lakshmi Gopalakrishnan, M.B.B.S. [4]
Overview
Alcohol septal ablation
Alcohol septal ablation, introduced by Ulrich Sigwart in 1994, is a percutaneous technique that involves injection of alcohol into the first septal perferator of the left anterior descending artery. This is a technique with results similar to the surgical septal myectomy procedure but is less invasive, since it does not involve general anaesthesia and opening of the chest wall and pericardium (which are done in a septal myomectomy). In a select population with symptoms secondary to a high outflow tract gradient, alcohol septal ablation can reduce the symptoms of HCM.[1][2][3] When performed properly, an alcohol septal ablation induces a controlled heart attack, in which the portion of the interventricular septum that involves the left ventricular outflow tract is infarcted and will contract into a scar.
Efficacy and Procedural Success
Relief of obstruction is noted immediately in the majority of appropriately selected patients. Clinical success is defined as a 50% or more reduction in peak gradient across the outflow tract, predicting continued improvement in gradient and cardiac remodeling over the ensuing 1 to 2 years. Over 90% of patients experience a successful procedure, with improvement in outflow tract gradient and mitral regurgitation. Patients typically report progressive reduction in symptoms, including improved shortness of breath, lightheadedness and chest pain.
Follow-Up
Serial echocardiograms are routinely obtained to follow the cardiac remodeling over time, and document reduction in outflow tract gradient.
Comparison to Myectomy
Non-randomized data from the Netherlands suggests caution in the utilization of alcohol septal ablation, which may be inferior to surgical myectomy [4]. The outcomes (the risk of cardiac death and aborted sudden cardiac death including appropriate cardioverter-defibrillator discharges for fast ventricular tachycardia/ventricular fibrillation) in 91 consecutive alcohol septal ablation patients were compared with 40 patients who underwent septal myectomy. The 1-, 5-, and 8-year event free survival was 96%, 86%, and 67%, respectively in the alcohol septal ablation patients which was poorer than the 100%, 96%, and 96%, event free rates in the myectomy patients over 6.6±2.7 years (P=0.01). Stated differently, the alcohol septal ablation patients faced a 5-fold increase in the risk of the primary endpoint on an annual basis (4.4% versus 0.9%) even when adjustments were made in a propensity adjusted multivariable model (p=0.02). Based upon non-randomized data, myectomy may be prefferable to alcohol septal ablation.
It is important to note that patients who fail to respond to alcohol septal ablation may still be candidates for surgical myectomy. Likewise, patients who fail surgical myectomy may still respond to alcohol septal ablation.
2011 ACCF/AHA Guideline Recommendations: Alcohol Septal Ablation [5][6]
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Class IIa1. Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: C) 2. When surgery is contraindicated or the risk is considered unacceptable because of serious comorbidities or advanced age, alcohol septal ablation, when performed in experienced centers, can be beneficial in eligible adult patients with HCM with LVOT obstruction and severe drug-refractory symptoms (usually NYHA functional classes III or IV).(62,153,277–281) (Level of Evidence: B) Class IIb1. Alcohol septal ablation, when performed in experienced centers, may be considered as an alternative to surgical myectomy for eligible adult patients with HCM with severe drug-refractory symptoms and LVOT obstruction when, after a balanced and thorough discussion, the patient expresses a preference for septal ablation.(153,273,278,280,281) (Level of Evidence: B) 2. The effectiveness of alcohol septal ablation is uncertain in patients with HCM with marked (i.e., >30 mm) septal hypertrophy, and therefore the procedure is generally discouraged in such patients. (Level of Evidence: C) Class III (Harm)1. Alcohol septal ablation should not be done in patients with HCM with concomitant disease that independently warrants surgical correction (e.g., coronary artery bypass grafting for CAD, mitral valve repair for ruptured chordae) in whom surgical myectomy can be performed as part of the operation. (Level of Evidence: C) 2. Alcohol septal ablation should not be done in patients with HCM who are less than 21 years of age and is discouraged in adults less than 40 years of age if myectomy is a viable option. (Level of Evidence: C) |
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Septal Myectomy
Septal myectomy is a surgical treatment for hypertrophic cardiomyopathy (HCM). Septal myectomies have been successfully performed for more than 25 years.
Outcomes
Septal myectomy is associated with a low perioperative mortality and a high late survival rate. A study at the Mayo Clinic found surgical myectomy performed to relieve outflow obstruction and severe symptoms in HCM was associated with long-term survival equivalent to that of the general population, and superior to obstructive HCM without operation. The results are shown below:[7]
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* Includes 0.8% operative mortality.
Comparison with alcohol ablation
Either alcohol septal ablation or myectomy offers substantial clinical improvement for patients with hypertrophic obstructive cardiomyopathy.
Hemodynamic resolution of the obstruction and its sequelae is more complete with myectomy.[8]
2011 ACCF/AHA Guideline Recommendations: Septal Myectomy [5][6]
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Class IIa1. Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. (Level of Evidence: C) 2. Surgical septal myectomy, when performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction.(61,62,155,273–275) (Level of Evidence: B) 3. Surgical septal myectomy, when performed at experienced centers, can be beneficial in symptomatic children with HCM and severe resting obstruction (>50 mm Hg) for whom standard medical therapy has failed.(276) (Level of Evidence: C) |
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Ventricular pacing
The use of a pacemaker has been advocated in a subset of individuals, in order to cause asynchronous contraction of the left ventricle. Since the pacemaker activates the interventricular septum before the left ventricular free wall, the gradient across the left ventricular outflow tract may decrease. The AV interval must be shortened to do this, but not at the expense of diastolic filling. This form of treatment has been shown to provide less relief of symptoms and less of a reduction in the left ventricular outflow tract gradient when compared to surgical myectomy [9]. Dual chamber pacing does not decrease the risk of sudden cardiac death in these patients.
2011 ACCF/AHA Guideline Recommendations: Pacing [5][6]
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Class IIa1. In patients with HCM who have had a dual-chamber device implanted for non-HCM indications, it is reasonable to consider a trial of dual-chamber atrial-ventricular pacing (from the right ventricular apex) for the relief of symptoms attributable to LVOT obstruction.(292,294,295,366) (Level of Evidence: B) Class IIb1. Permanent pacing may be considered in medically refractory symptomatic patients with obstructive HCM who are suboptimal candidates for septal reduction therapy.(283,292,294,295,366) (Level of Evidence: B) Class III (No Benefit)1. Permanent pacemaker implantation for the purpose of reducing gradient should not be performed in patients with HCM who are asymptomatic or whose symptoms are medically controlled.(283,284,367) (Level of Evidence: C) 2. Permanent pacemaker implantation should not be performed as a first-line therapy to relieve symptoms in medically refractory symptomatic patients with HCM and LVOT obstruction who are candidates for septal reduction.(283,284,367) (Level of Evidence: B) |
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2007 ESC Guidelines- Cardiac Pacing in HCM [10]
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Class IIa1. Symptomatic bradycardia due to beta-blockade when alternative therapies are unacceptable. (Level of Evidence: C) Class IIb1. Patients with drug refractory hypertrophic cardiomyopathy with significant resting or provoked LVOT gradient [11][12][13] and contraindications for septal ablation or myectomy. (Level of Evidence: A) Class III1. Asymptomatic patients. (Level of Evidence: C) 2. Symptomatic patients who do not have LVOT obstruction. (Level of Evidence: C) |
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Automatic Implantable Cardiac Defibrillator (AICD) placement
The role of AICD (automatic implantable cardiac defibrillator) placement in HCM is controversial. It offers the best potential benefit for survival and should probably be implanted in survivors of SCD and those deemed at high risk by clinical parameters. Nonetheless, the impact on prognosis is unclear because tachyarrhythmias may not always be the mechanism for syncope and sudden death. In addition, older patients may be self-selected “survivors” that stand to gain less from ICD placement. One recent retrospective study showed that at an average follow-up of 128 patients at 3.1 years, 23 percent had shocks for VT (ventricular tachycardia) and 25% had inappropriate shocks. Of those receiving the ICD prophylactically, 5% were shocked per year. This study did not evaluate the role of clinical predictors, evaluate total mortality and was a non-randomized retrospective design that does not establish the need for ICD placement in all patients with HCM or superiority to amiodarone therapy.
2011 ACCF/AHA Guideline Recommendations: Invasive Therapies
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Class I1. Septal reduction therapy should be performed only by experienced operators in the context of a comprehensive HCM clinical program and only for the treatment of eligible patients with severe drug-refractory symptoms and LVOT obstruction.† (272) (Level of Evidence: C) Class III (Harm)1. Septal reduction therapy should not be done for adult patients with HCM who are asymptomatic with normal exercise tolerance or whose symptoms are controlled or minimized on optimal medical therapy. (Level of Evidence: C) 2. Septal reduction therapy should not be done unless performed as part of a program dedicated to the longitudinal and multidisciplinary care of patients with HCM. (Level of Evidence: C) 3. Mitral valve replacement for relief of LVOT obstruction should not be performed in patients with HCM in whom septal reduction therapy is an option. (Level of Evidence: C) |
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Guideline Resources
- The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in Collaboration with the European Heart Rhythm Association [10]
References
- ↑ Maron BJ (2002). "Hypertrophic cardiomyopathy: a systematic review". JAMA. 287 (10): 1308–20. PMID 11886323.
- ↑ Wigle ED, Rakowski H, Kimball BP, Williams WG (1995). "Hypertrophic cardiomyopathy. Clinical spectrum and treatment". Circulation. 92 (7): 1680–92. PMID 7671349.
- ↑ Brilakis ES, Nishimura RA. Severe pulmonary hypertension in a patient with hypertrophic cardiomyopathy: response to alcohol septal ablation. Heart. 2003 Jul; 89(7):790. (Medline abstract)
- ↑ Ten Cate FJ, Soliman OI, Michels M, Theuns DA, de Jong PL, Geleijnse ML, Serruys PW (2010). "Long-Term Outcome of Alcohol Septal Ablation in Patients with Obstructive Hypertrophic Cardiomyopathy: A Word of Caution". Circulation. Heart Failure. doi:10.1161/CIRCHEARTFAILURE.109.862359. PMID 20332420. Unknown parameter
|month=ignored (help) - ↑ 5.0 5.1 5.2 5.3 5.4 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 58 (25): 2703–38. doi:10.1016/j.jacc.2011.10.825. PMID 22075468. Retrieved 2011-12-19. Unknown parameter
|month=ignored (help) - ↑ 6.0 6.1 6.2 6.3 6.4 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011). "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 Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology. 58 (25): e212–60. doi:10.1016/j.jacc.2011.06.011. PMID 22075469. Retrieved 2011-12-19. Unknown parameter
|month=ignored (help) - ↑ Ommen S, Maron B, Olivotto I, Maron M, Cecchi F, Betocchi S, Gersh B, Ackerman M, McCully R, Dearani J, Schaff H, Danielson G, Tajik A, Nishimura R (2005). "Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy". J Am Coll Cardiol. 46 (3): 470–6. PMID 16053960.
- ↑ Ralph-Edwards A, Woo A, McCrindle B, Shapero J, Schwartz L, Rakowski H, Wigle E, Williams W (2005). "Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by propensity score". J Thorac Cardiovasc Surg. 129 (2): 351–8. PMID 15678046.
- ↑ Ommen SR, Nishimura RA, Squires RW, Schaff HV, Danielson GK, Tajik AJ. Comparison of dual-chamber pacing versus septal myectomy for the treatment of patients with hypertropic obstructive cardiomyopathy: a comparison of objective hemodynamic and exercise end points. J Am Coll Cardiol. 1999 Jul; 34(1):191–6. (Medline abstract)
- ↑ 10.0 10.1 Vardas PE, Auricchio A, Blanc JJ, Daubert JC, Drexler H, Ector H; et al. (2007). "Guidelines for cardiac pacing and cardiac resynchronization therapy. The Task Force for Cardiac Pacing and Cardiac Resynchronization Therapy of the European Society of Cardiology. Developed in collaboration with the European Heart Rhythm Association". Europace. 9 (10): 959–98. doi:10.1093/europace/eum189. PMID 17726043.
- ↑ Fananapazir L, Cannon RO, Tripodi D, Panza JA (1992). "Impact of dual-chamber permanent pacing in patients with obstructive hypertrophic cardiomyopathy with symptoms refractory to verapamil and beta-adrenergic blocker therapy". Circulation. 85 (6): 2149–61. PMID 1350522.
- ↑ Fananapazir L, Epstein ND, Curiel RV, Panza JA, Tripodi D, McAreavey D (1994). "Long-term results of dual-chamber (DDD) pacing in obstructive hypertrophic cardiomyopathy. Evidence for progressive symptomatic and hemodynamic improvement and reduction of left ventricular hypertrophy". Circulation. 90 (6): 2731–42. PMID 7994815.
- ↑ Kappenberger L, Linde C, Daubert C, McKenna W, Meisel E, Sadoul N; et al. (1997). "Pacing in hypertrophic obstructive cardiomyopathy. A randomized crossover study. PIC Study Group". Eur Heart J. 18 (8): 1249–56. PMID 9458416.
- ↑ Epstein AE, DiMarco JP, Ellenbogen KA, Estes NAM III, Freedman RA, Gettes LS, Gillinov AM, Gregoratos G, Hammill SC, Hayes DL, Hlatky MA, Newby LK, Page RL, Schoenfeld MH, Silka MJ, Stevenson LW, Sweeney MO. ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices). Circulation. 2008; 117: 2820–2840. PMID 18483207