Hipertrofik Kardiyomiyopatide Girişimsel Tedavi
Özet
Hipertrofik kardiyomiyopati (HKM), kalp kasının anormal derecede kalınlaşması ile karakterize edilen genetik bir hastalıktır. Bu kalınlaşma, kalbin kan pompalama yeteneğini etkileyebilir ve çeşitli semptomlara yol açabilir. Girişimsel tedavi yöntemleri, bu semptomların hafifletilmesi ve hastaların yaşam kalitesinin artırılması amacıyla uygulanır.
Girişimsel tedavi yöntemleri arasında septal alkol ablasyonu ve cerrahi miyektomi en yaygın olanlardır. Septal alkol ablasyonu, septumun kalınlaşmış kısmına alkol enjekte edilerek bu bölgenin küçültülmesini sağlar. Bu yöntem, invaziv olmaması nedeniyle tercih edilir, ancak bazı riskler ve komplikasyonlar taşır. Cerrahi miyektomi ise, kalınlaşmış septumun cerrahi olarak çıkarılmasıdır ve genellikle daha ciddi vakalarda uygulanır. Bu yöntem, semptomların belirgin şekilde azalmasını sağlar ve uzun vadeli sonuçları genellikle olumludur.
Bu tedavi yöntemlerinin seçimi, hastanın semptomları, anatomik özellikleri ve genel sağlık durumu göz önünde bulundurularak yapılır. Her iki yöntemin de kendine özgü avantajları ve dezavantajları bulunmaktadır ve tedavi planı bireyselleştirilmiş yaklaşımla belirlenir. Girişimsel tedavi, ilaç tedavisi ile kombine edildiğinde, hastaların semptomlarının yönetiminde ve yaşam kalitesinin artırılmasında etkili olabilir.
Hypertrophic cardiomyopathy (HCM) is a genetic disorder characterized by the abnormal thickening of the heart muscle. This thickening can impair the heart's ability to pump blood and lead to various symptoms. Interventional treatment methods are employed to alleviate these symptoms and improve patients' quality of life.
Among the interventional treatment options, septal alcohol ablation and surgical myectomy are the most common. Septal alcohol ablation involves injecting alcohol into the thickened part of the septum to reduce its size. This method is preferred for its minimally invasive nature, but it carries certain risks and potential complications. Surgical myectomy, on the other hand, involves the surgical removal of the thickened septum and is typically used in more severe cases. This approach significantly reduces symptoms and generally yields favorable long-term outcomes.
The choice of treatment method depends on the patient's symptoms, anatomical characteristics, and overall health status. Each method has its unique advantages and disadvantages, and treatment plans are tailored to the individual. When combined with pharmacological therapy, interventional treatment can effectively manage symptoms and enhance the quality of life for patients with HKM.
Referanslar
Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and manage¬ment of hypertrophic cardiomyopathy: The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733.
Coppini R, Ho CY, Ashley E, et al. Clinical phenotype and outcome of hypertrophic cardio¬myopathy associated with thin-filament gene mutations. J Am Coll Cardiol. 2014; 64: 2589.
Maron BJ, Nishimura RA, Cooper LT Jr, et al. Eligibility and disqualification recommenda¬tions for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis. J Am Coll Cardiol 66:2362, Circulation. 2015; 132:e273.
Olivotto I, Cecchi F, Casey SA, Dolara A, Traverse JH, Maron BJ. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 2001; 104: 2517–24. doi: 10.1161/hc4601.097997.
Maron BJ, Rowin EJ, Casey SA, et al. Hypertrophic cardiomyopathy in children, adolescents and young adults associated with low cardiovascular mortality with contemporary management strategies. Circulation. 2016; 133: 62.
Semsarian C, Ingles J, Maron MS, et al. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015; 65: 1249.
Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet. 2013; 381: 242.
Maron MS, Rowin EJ, Olivotto I, et al. Contemporary natural history and management of nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2016; 67: 1399.
Sherrid MV, Balaram S, Kim B, et al. The mitral valve in obstructive hypertrophic cardiomy¬opathy: A text in context. J Am Coll Cardiol. 2016; 67: 1846.
Olivotto I, Girolami F, Sciagra R, et al. Microvascular function is selectively impaired in patients with hypertrophic cardiomyopathy and sarcomere myofilament gene mutations. J Am Coll Cardiol. 2011; 58: 839.
Kampmann C, Wiethoff CM, Wenzel A, et al. Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe. Heart 2000; 83: 667–72. doi. org/10.1136/heart.83.6.667
Dimitrow PP, Bober M, Michalowska J, Sorysz D. Left ventricular outflow tract gradient provoked by upright position or exercise in treated patients with hypertrophic cardiomyopathy without obstruction at rest. Echocardiography 2009; 26: 513–520. doi: 10.1111/j.1540-8175.2008.00851.x.
Rudolph A, Abdel-Aty H, Bohl S, et al. Noninvasive detection of fibrosis applying contrast-enhanced cardiac magnetic resonance in different forms of left ventricular hypertrophy relation to remodeling. J Am Coll Cardiol 2009; 53: 284–291. doi: 10.1016/j.jacc.2008.08.064.
Richard P, Charron P, Carrier L, et al. Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Circulation, 2003; 107: 2227–32. doi: 10.1161/01.CIR.0000066323.15244.54.
Gruner C, Ivanov J, Care M, et al. Toronto hypertrophic cardiomyopathy genotype score for prediction of a positive genotype in hypertrophic cardiomyopathy. Circ Cardiovasc Genet 2013; 6: 19–26. doi: 10.1161/CIRCGENETICS.112.963363.
Kubo T, Gimeno JR, Bahl A, et al. Prevalence, clinical significance, and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype. J Am Coll Cardiol 2007; 49: 2419–26. doi:10.1016/j.jacc.2007. 02.061.
Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography. Eur J Echocardiogr 2009; 10: 165–193. doi: 10.1093/ejechocard/ jep007.
Wigle ED, Henderson M, Rakowski H, Wilansky S. Muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy): the evidence for true obstruction to left ventricular outflow. Postgrad Med J 1986; 62: 531–536. doi: 10. 1136/pgmj.62.728.531.
Stauffer JC, Ruiz V, Morard JD. Subaortic obstruction after sildenafil in a patient with hypertrophic cardiomyopathy. N Engl J Med 1999; 341: 700–701. doi:10. 1056/NEJM199908263410916.
Sherrid MV, Shetty A, Winson G, et al. Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with beta-blockade or verapamil. Circ Heart Fail 2013; 6: 694–702. doi: 10.1161/CIRCHEARTFAILURE.112.000122
Olivotto I, Oreziak A, Barriales-Villa R, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HKM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet 2020; 396: 759–769. doi: 10.1016/S0140-6736(20)31792-X.
Sorajja P, Nishimura RA, Gersh BJ, et al. Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy: a long-term follow-up study. J Am Coll Cardiol 2009; 54: 234–241. doi:10. 1016/j.jacc.2009.01.079
Cavigli L, Fumagalli C, Maurizi N, et al. Timing of invasive septal reduction therapies and outcome of patients with obstructive hypertrophic cardiomyopathy. Int J Cardiol 2018; 273: 155–161. doi:10.1016/j.ijcard.2018.09.004.
Menon SC, Ackerman MJ, Ommen SR, et al. Impact of septal myectomy on left atrial volume and left ventricular diastolic filling patterns: an echocardiographic study of young patients with obstructive hypertrophic cardiomyopathy. J Am Soc Echocardiogr 2008; 21: 684–688. doi: 10.1016/j.echo. 2007.11.006.
ten Cate FJ, Soliman OI, Michels M, et al. Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution. Circ Heart Fail. 2010; 3: 362.
Veselka J, Liebregts M, Cooper R, et al. Outcomes of patients with hypertrophic obstructive cardiomyopathy and pacemaker implanted after alcohol septal ablation. JACC Cardiovasc Interv 2022; 15: 1910–1917. doi: 10.1016/j.jcin.2022.06.034.
Batzner A, Pfeiffer B, Neugebauer A, Aicha D, Blank C, Seggewiss H. Survival after alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 2018; 72: 3087– 94. doi: 10.1016/j.jacc.2018.09.064.
Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guidelines 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(24): e783-e831.
Ferrazzi P, Spirito P, Iacovoni A, et al. Transaortic chordal cutting: mitral valve repair for obstructive hypertrophic cardiomyopathy and mild septal hypertrophy. J Am Coll Cardiol. 2015; 66: 1687.
Iacovoni A, Spirito P, Simon C, et al. A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy. Eur Heart J 2012; 33: 2080–2087. doi: 10.1093/eurheartj/ehs064.
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: 481–489. doi: 10.1016/j.jtcvs.2003.09.040.
Boll G, Rowin EJ, Maron BJ, Wang W, Rastegar H, Maron MS. Efficacy of combined Cox-Maze IV and ventricular septal myectomy for treatment of atrial fibrillation in patients with obstructive hypertrophic cardiomyopathy. Am J Cardiol 2020; 125: 120–126. doi: 10.1016/j.amjcard.2019.09.029.
Noseworthy PA, Rosenberg MA, Fifer MA, et al. Ventricular arrhythmia following alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Am J Cardiol. 2009; 104: 128.
Ommen SR, Maron BJ, Olivotto I, et al. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005; 46: 470.
Menon SC, Ackerman MJ, Ommen SR, et al. Impact of septal myectomy on left atrial volume and left ventricular diastolic filling patterns: an echocardiographic study of young patients with obstructive hypertrophic cardiomyopathy. J Am Soc Echocardiogr 2008; 21: 684–688. doi: 10.1016/j.echo. 2007.11.006.
Maron BJ, Rowin EJ, Casey SA, et al. Risk stratification and outcome of patients with hypertrophic cardiomyopathy over 60 years of age. Circulation. 2013; 127: 585.
O’Mahony C, Lambiase PD, Quarta G, et al. The long-term survival and the risks and benefits of implantable cardioverter defibrillators in patients with hypertrophic cardiomyopathy. Heart. 2012; 98: 116.
Weinstock J, Bader YH, Maron MS, et al. Subcutaneous implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy: an initial experience. J Am Heart Assoc. 2016; 5.
Maron BJ, Maron MS. Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy. Heart Rhythm. 2016; 1: 1683.
Rowin E, Maron BJ, Haas TS, et al. Hypertrophic cardiomyopathy with left ventricular apical aneurysm expands risk stratification and management. J Am Coll Cardiol. 2017; 69: 761–73.
Glikson M, Nielsen JC, Kronborg MB, et al. 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J 2021; 42: 3427–3520. doi: 10.1093/eurheartj/ehab364.
Qintar M, Morad A, Alhawasli H, et al. Pacing for drug-refractory or drug-intolerant hypertrophic cardiomyopathy. CochraneDatabase Syst Rev 2012; CD008523. doi: 10.1002/14651858.CD008523.pub2.
Maron MS, Kalsmith BM, Udelson JE, et al. Survival after cardiac transplantation in patients with hypertrophic cardiomyopathy. Circ Heart Fail. 2010; 3: 574.
Referanslar
Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and manage¬ment of hypertrophic cardiomyopathy: The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733.
Coppini R, Ho CY, Ashley E, et al. Clinical phenotype and outcome of hypertrophic cardio¬myopathy associated with thin-filament gene mutations. J Am Coll Cardiol. 2014; 64: 2589.
Maron BJ, Nishimura RA, Cooper LT Jr, et al. Eligibility and disqualification recommenda¬tions for competitive athletes with cardiovascular abnormalities: Task Force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis. J Am Coll Cardiol 66:2362, Circulation. 2015; 132:e273.
Olivotto I, Cecchi F, Casey SA, Dolara A, Traverse JH, Maron BJ. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 2001; 104: 2517–24. doi: 10.1161/hc4601.097997.
Maron BJ, Rowin EJ, Casey SA, et al. Hypertrophic cardiomyopathy in children, adolescents and young adults associated with low cardiovascular mortality with contemporary management strategies. Circulation. 2016; 133: 62.
Semsarian C, Ingles J, Maron MS, et al. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015; 65: 1249.
Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet. 2013; 381: 242.
Maron MS, Rowin EJ, Olivotto I, et al. Contemporary natural history and management of nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2016; 67: 1399.
Sherrid MV, Balaram S, Kim B, et al. The mitral valve in obstructive hypertrophic cardiomy¬opathy: A text in context. J Am Coll Cardiol. 2016; 67: 1846.
Olivotto I, Girolami F, Sciagra R, et al. Microvascular function is selectively impaired in patients with hypertrophic cardiomyopathy and sarcomere myofilament gene mutations. J Am Coll Cardiol. 2011; 58: 839.
Kampmann C, Wiethoff CM, Wenzel A, et al. Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe. Heart 2000; 83: 667–72. doi. org/10.1136/heart.83.6.667
Dimitrow PP, Bober M, Michalowska J, Sorysz D. Left ventricular outflow tract gradient provoked by upright position or exercise in treated patients with hypertrophic cardiomyopathy without obstruction at rest. Echocardiography 2009; 26: 513–520. doi: 10.1111/j.1540-8175.2008.00851.x.
Rudolph A, Abdel-Aty H, Bohl S, et al. Noninvasive detection of fibrosis applying contrast-enhanced cardiac magnetic resonance in different forms of left ventricular hypertrophy relation to remodeling. J Am Coll Cardiol 2009; 53: 284–291. doi: 10.1016/j.jacc.2008.08.064.
Richard P, Charron P, Carrier L, et al. Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Circulation, 2003; 107: 2227–32. doi: 10.1161/01.CIR.0000066323.15244.54.
Gruner C, Ivanov J, Care M, et al. Toronto hypertrophic cardiomyopathy genotype score for prediction of a positive genotype in hypertrophic cardiomyopathy. Circ Cardiovasc Genet 2013; 6: 19–26. doi: 10.1161/CIRCGENETICS.112.963363.
Kubo T, Gimeno JR, Bahl A, et al. Prevalence, clinical significance, and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype. J Am Coll Cardiol 2007; 49: 2419–26. doi:10.1016/j.jacc.2007. 02.061.
Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography. Eur J Echocardiogr 2009; 10: 165–193. doi: 10.1093/ejechocard/ jep007.
Wigle ED, Henderson M, Rakowski H, Wilansky S. Muscular (hypertrophic) subaortic stenosis (hypertrophic obstructive cardiomyopathy): the evidence for true obstruction to left ventricular outflow. Postgrad Med J 1986; 62: 531–536. doi: 10. 1136/pgmj.62.728.531.
Stauffer JC, Ruiz V, Morard JD. Subaortic obstruction after sildenafil in a patient with hypertrophic cardiomyopathy. N Engl J Med 1999; 341: 700–701. doi:10. 1056/NEJM199908263410916.
Sherrid MV, Shetty A, Winson G, et al. Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with beta-blockade or verapamil. Circ Heart Fail 2013; 6: 694–702. doi: 10.1161/CIRCHEARTFAILURE.112.000122
Olivotto I, Oreziak A, Barriales-Villa R, et al. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HKM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet 2020; 396: 759–769. doi: 10.1016/S0140-6736(20)31792-X.
Sorajja P, Nishimura RA, Gersh BJ, et al. Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy: a long-term follow-up study. J Am Coll Cardiol 2009; 54: 234–241. doi:10. 1016/j.jacc.2009.01.079
Cavigli L, Fumagalli C, Maurizi N, et al. Timing of invasive septal reduction therapies and outcome of patients with obstructive hypertrophic cardiomyopathy. Int J Cardiol 2018; 273: 155–161. doi:10.1016/j.ijcard.2018.09.004.
Menon SC, Ackerman MJ, Ommen SR, et al. Impact of septal myectomy on left atrial volume and left ventricular diastolic filling patterns: an echocardiographic study of young patients with obstructive hypertrophic cardiomyopathy. J Am Soc Echocardiogr 2008; 21: 684–688. doi: 10.1016/j.echo. 2007.11.006.
ten Cate FJ, Soliman OI, Michels M, et al. Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution. Circ Heart Fail. 2010; 3: 362.
Veselka J, Liebregts M, Cooper R, et al. Outcomes of patients with hypertrophic obstructive cardiomyopathy and pacemaker implanted after alcohol septal ablation. JACC Cardiovasc Interv 2022; 15: 1910–1917. doi: 10.1016/j.jcin.2022.06.034.
Batzner A, Pfeiffer B, Neugebauer A, Aicha D, Blank C, Seggewiss H. Survival after alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol 2018; 72: 3087– 94. doi: 10.1016/j.jacc.2018.09.064.
Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guidelines 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(24): e783-e831.
Ferrazzi P, Spirito P, Iacovoni A, et al. Transaortic chordal cutting: mitral valve repair for obstructive hypertrophic cardiomyopathy and mild septal hypertrophy. J Am Coll Cardiol. 2015; 66: 1687.
Iacovoni A, Spirito P, Simon C, et al. A contemporary European experience with surgical septal myectomy in hypertrophic cardiomyopathy. Eur Heart J 2012; 33: 2080–2087. doi: 10.1093/eurheartj/ehs064.
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: 481–489. doi: 10.1016/j.jtcvs.2003.09.040.
Boll G, Rowin EJ, Maron BJ, Wang W, Rastegar H, Maron MS. Efficacy of combined Cox-Maze IV and ventricular septal myectomy for treatment of atrial fibrillation in patients with obstructive hypertrophic cardiomyopathy. Am J Cardiol 2020; 125: 120–126. doi: 10.1016/j.amjcard.2019.09.029.
Noseworthy PA, Rosenberg MA, Fifer MA, et al. Ventricular arrhythmia following alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Am J Cardiol. 2009; 104: 128.
Ommen SR, Maron BJ, Olivotto I, et al. Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005; 46: 470.
Menon SC, Ackerman MJ, Ommen SR, et al. Impact of septal myectomy on left atrial volume and left ventricular diastolic filling patterns: an echocardiographic study of young patients with obstructive hypertrophic cardiomyopathy. J Am Soc Echocardiogr 2008; 21: 684–688. doi: 10.1016/j.echo. 2007.11.006.
Maron BJ, Rowin EJ, Casey SA, et al. Risk stratification and outcome of patients with hypertrophic cardiomyopathy over 60 years of age. Circulation. 2013; 127: 585.
O’Mahony C, Lambiase PD, Quarta G, et al. The long-term survival and the risks and benefits of implantable cardioverter defibrillators in patients with hypertrophic cardiomyopathy. Heart. 2012; 98: 116.
Weinstock J, Bader YH, Maron MS, et al. Subcutaneous implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy: an initial experience. J Am Heart Assoc. 2016; 5.
Maron BJ, Maron MS. Contemporary strategies for risk stratification and prevention of sudden death with the implantable defibrillator in hypertrophic cardiomyopathy. Heart Rhythm. 2016; 1: 1683.
Rowin E, Maron BJ, Haas TS, et al. Hypertrophic cardiomyopathy with left ventricular apical aneurysm expands risk stratification and management. J Am Coll Cardiol. 2017; 69: 761–73.
Glikson M, Nielsen JC, Kronborg MB, et al. 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J 2021; 42: 3427–3520. doi: 10.1093/eurheartj/ehab364.
Qintar M, Morad A, Alhawasli H, et al. Pacing for drug-refractory or drug-intolerant hypertrophic cardiomyopathy. CochraneDatabase Syst Rev 2012; CD008523. doi: 10.1002/14651858.CD008523.pub2.
Maron MS, Kalsmith BM, Udelson JE, et al. Survival after cardiac transplantation in patients with hypertrophic cardiomyopathy. Circ Heart Fail. 2010; 3: 574.
