Historical and modern aspects of diagnostics and treatment of hypertrophic cardiomyopathy: literature review
Published 2016-03-25
Keywords
- hypertrophic cardiomyopathy,
- surgical myectomy,
- alcohol septal ablation
How to Cite
Copyright (c) 2016 Kashtanov M. G., Idov E. M.

This work is licensed under a Creative Commons Attribution 4.0 International License.
Abstract
Hypertrophic cardiomyopathy is a relatively rare and challenging pathology. The last 70 years have seen a great progress in understanding the diagnostics and treatment of this disorder. The review summarizes various data from the original sources that help see the current state of this challenge. The paper also describes some aspects of surgical and interventional treatment of patients with obstructive hypertrophic cardiomyopathy. Special emphasis is placed on such widespread treatment modality as alcohol septal ablation.
References
- Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE. Prevalence of Hypertrophic Cardiomyopathy in a General Population of Young Adults: Echocardiographic Analysis of 4111 Subjects in the CARDIA Study. Circulation. 1995;92(4):785-789, doi:10.1161/01.CIR.92.4.785/
- Hada Y, Sakamoto T, Amano K, Yamaguchi T, Takenaka K, Takahashi H, Takikawa R, Hasegawa I, Takahashi T, Suzuki J. et al. Prevalence of hypertrophic cardiomyopathy in a population of adult Japanese workers as detected by echocardiographic screening. Am J Cardiol. 1987;59(1):183-4.
- Codd MB, Sugrue DD, Gersh BJ, Melton LJ3rd. Epidemiology of idiopathic dilated and hypertrophic cardiomyopathy. A population-based study in Olmsted County, Minnesota, 1975-1984. Circulation. 1989;80(3):564-72.
- Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes. N Engl J Med. 1998;339(6):364-9. doi: 10.1056/NEJM199808063390602.
- Maron BJ, Mathenge R, Casey SA, Poliac LC, Longe TF. Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J Am Coll Cardiol. 1999;33(6):1590-5.
- Nistri S, Thiene G, Basso C, Corrado D, Vitolo A, Maron BJ. Screening for hypertrophic cardiomyopathy in a young male military population. Am J Cardiol. 2003;91(8):1021-3, A8.
- Zou Y, Song L, Wang Z., Ma A, Liu T, Gu H, Lu S, Wu P, Zhang Y, Shen L., Cai Y, Zhen Y, Liu Y, Hui R. Prevalence of idiopathic hypertrophic cardiomyopathy in China: a population-based echocardiographic analysis of 8080 adults. Am J Med. 2004;116(1):14-8.
- Maron BJ, Spirito P, Roman MJ, Paranicas M, Okin PM, Best LG, Lee ET, Devereux RB. Prevalence of hypertrophic cardiomyopathy in a population-based sample of American Indians aged 51 to 77 years (the Strong Heart Study). Am J Cardiol. 2004;93(12):1510-4.
- Maro EE, Janabi M, Kaushik R. Clinical and echocardiographic study of hypertrophic cardiomyopathy in Tanzania. Trop Doct. 2006;36(4):225-7.
- Ng CT, Chee TS, Ling LF, Lee YP, Ching CK, Chua TS, Cheok C, Ong HY. Prevalence of hypertrophic cardiomyopathy on an electrocardiogram-based pre-participation screening programme in a young male South-East Asian population: results from the Singapore Armed Forces Electrocardiogram and Echocardiogram screening protocol. Europace. 2011;13(6):883-8. doi: 10.1093/europace/eur051.
- Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, Cecchi F, Maron BJ. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med. 2003;348(4):295-303. doi: 10.1056/NEJMoa021332.
- Liouville H. Rétrécissement cardiaque sous aortique. Gazette Med Paris. 1869;24:161-3.
- Vulpian A. Contribution à l’étude des rétrécissements de l’orifice ventriculo-aortique. Arch Pathol. 1868;3:456-7.
- Hallopeau M. Rétrécissement ventriculo-aortique. Gazette Med Paris. 1869;24:683.
- Schmincke A. Ueber linkseitige muskulose conustenosen. Dtsch Med Wochenschr. 1907;33:2082-5.
- Levy RL, von Glahn WC. Cardiac hypertrophy of unknown cause. A study of the clinical and pathologic features in ten adults. Am Heart J. 1944;28(6):714-41.
- Evans W. Familial cardiomegaly. Br Heart J. 1949;11(1):68-82.
- Brock RC. Functional obstruction of the left ventricle (acquired aortic subvalvar stenosis). Guys Hosp Rep. 1957;106(4):221-238.
- Teare D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J. 1958;20:1-8.
- Morrow AG, Braunwald E. Functional aortic stenosis. A malformation characterized by resistance to left ventricular outflow without anatomic obstruction. 1959;20(2):181-189.
- Morrow AG, Brockenbrough EC. Surgical treatment of idiopathic hypertrophic subaortic stenosis. Ann Surg. 1961;154:181-189.
- Wigle ED, Auger P, Marquis Y. Muscular subaortic stenosis - the initial left ventricular inflow tract pressure as evidence of outflow tract obstruction. Can Med Assoc J. 1966;95(16):793-7.
- Braunwald E, Ebert PA. Hemodynamic alterations in idiopathic hypertrophic subaortic stenosis induced by sympathomimetic drugs. Am J Cardiol. 1962;10:489-95.
- Brockenbrough EC, Braunwald E, Morrow AG. A hemodynamic technique for the detection of hypertrophic subaortic stenosis. Circulation. 1961;23:189-94. doi: 10.1161/01.CIR.23.2.189.
- Harrison DC, Braunwald E, Glick G, Mason DT, Chidsey CA, Ross J Jr. Effects of beta-adrenergic blockade on the circulation with particular reference to observations in patients with hypertrophic subaortic stenosis. Circulation. 1964;29:84-98.
- Braunwald U, Brockenbrough EC, Morrow AG. Editorial. Hypertropic Subaortic Stenosis - A Broadened Concept. Circulation. 1962;26(2):161-6.
- Criley JM, Lewis KB, White RI, Ross RS. Pressure gradients without obstruction: a new concept of “hypertrophic subaortic stenosis”. 1965;32(6):881-7.
- Criley JM, Siegel RJ. Has “obstruction” hindered our understanding of hypertrophic cardiomyopathy? Circulation. 1985;72(6):1148-54.
- Shah PM, Gramiak R, Kramer DH. Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy. Circulation. 1969;40(1):3-11.
- Shah PM, Gramiak R, Adelman AG, Wigle ED. Role of echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis. Circulation. 1971;44(5):891-898.
- Abbasi AS, MacAlpin RN, Eber LM, Pearce ML. Echocardiographic diagnosis of idiopathic hypertrophic cardiomyopathy without outflow obstruction. Circulation. 1972;46(5):897-904.
- Abbasi AS, MacAlpin RN, Eber LM, Pearce ML. Left ventricular hypertrophy diagnosed by echocardiography. N Engl J Med. 1973;289(3):118-21.
- Maron BJ, Clark CE, Henry WL, Fukuda T, Edwards JE, Mathews EC Jr, Redwood DR, Epstein SE. Prevalence and characteristics of disproportionate ventricular septal thickening in patients with acquired or congenital heart disease: echocardiographic and morphologic findings. 1977;55(3):489-96.
- Maron BJ, Savage DD, Clark CE, Henry WL, Vlodaver Z, Edwards JE, Epstein SE. Prevalence and characteristics of disproportionate ventricular septal thickening in patients with coronary artery disease. Circulation. 1978;57(2):250-5.
- Maron BJ, Edwards JE, Epstein SE. Disproportionate ventricular thickening in patients with systemic hypertension. 1978;73(4):466-70.
- Henry WL, Clark CE, Glancy DL, Epstein SE. Echocardiographic measurement of the left ventricular outflow gradient in idiopathic hypertrophic subaortic stenosis. N Engl J Med. 1973;288(19):989-93.
- Maron BJ, Epstein SE. Hypertrophic cardiomyopathy: a discussion of nomenclature. Am J Cardiol. 1979;43(6):1242-1244.
- Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of the distribution of left ventricular hypertrophy in a large population of patients with hypertrophic cardiomyopathy: A wide-angle two-dimensional echocardiographic study. Am J Cardiol. 1981;48(3):418-28.
- Yock PG, Hatle L, Popp RL. Patterns and timing of Doppler-detected intracavitary and aortic flow in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1986;8(5):1047-58.
- Sherrid MV, Chu CK, Delia E, Mograder A, Dwyer EM Jr. An echocardiography study of the fluid mechanics of obstruction in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1993;22(3):816-25.
- Sasson Z, Henderson M, Wilansky S, Rakowski H, Wigle ED. Causal relation between the pressure gradient and left ventricular ejection time in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1989;13(6):1275-9.
- Pollick C, Rakowski H, Wigle ED. Muscular subaortic stenosis: the quantitative relationship between systolic anterior motion and the pressure gradient. Circulation. 1984;69(1):43-9.
- Panza JA, Petrone RK, Fananapazir L, Maron BJ. Utility of continuous wave Doppler echocardiography in the noninvasive assessment of left ventricular outflow tract pressure gradient in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1992;19(1):91-9.
- Spirito P, Maron BJ, Rosing DR. Morphologic determinants of hemodynamic state following ventricular septal myotomy-myectomy in patients with hypertrophic cardiomyopathy: M-mode and two- dimensional echocardiographic assessment. Circulation. 1984;70(6):984-95.
- Maron BJ, Gottdiener JS, Arce J, Rosing DR, Wesley YE, Epstein SE. Dynamic subaortic obstruction in hypertrophic cardiomyopathy: analysis by pulsed Doppler echocardiography. J Am Coll Cardiol. 1985;6(1):1-18.
- Murgo JP, Alter BR, Dorethy JF, Altobelli SA, McGranahan GM Jr. Dynamics of left ventricular ejection in obstructive and nonobstructive hypertrophic cardiomyopathy. J Clin Invest. 1980;66(6):1369-82.
- Murgo JP. Does outflow obstruction exist in hypertrophic cardiomyopathy? N Engl J Med. 1982;307(16):1008-9.
- Maron BJ, Maron MS. The 20 advances that have defined contemporary hypertrophic cardiomyopathy. Trends in cardiovascular medicine. 2015;25(1):54-64. doi: 10.1016/j.tcm.2014.09.004.
- Coats CJ, Hollman A. Hypertrophic cardiomyopathy: lessons from history. Heart. 2008;94(10):1258-63. doi:10.1136/hrt.2008.153452.
- Morrow AG, Brockenbrough EC. Surgical treatment of idiopathic hypertrophic subaortic stenosis. Ann Surg. 1961;154:181-9.
- Kirklin JW, Ellis FH. Surgical relief of diffuse subvalvular aortic stenosis. Circulation. 1961;24:739-42.
- Falicov RE, Resnekov L, Bharati S, Lev M. Mid-ventricular obstruction: a variant of obstructive cardiomyopathy. Am J Cardiol. 1976;37(3):432-7.
- Cooley DA, Leachman RD, Wukasch DC. Mitral valve replacement for idiopathic hypertrophic cardiomyopathy. J Cardiovasc Surg. 1976;17(5):380-7.
- McIntosh CL, Greenberg GJ, Maron BJ, Leon MB, Cannon RO 3rd, Clark RE. Clinical and hemodynamic results after mitral valve replacement in patients with obstructive hypertrophic cardiomyopathy. Ann Thorac Surg. 1989;47(2):236-46.
- McIntosh CL, Maron BJ, Cannon RO 3rd, Klues HG. Initial results of combined anterior mitral leaflet plication and ventricular septal myotomy-myectomy for relief of left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy. Circulation. 1992;86(5 Suppl):II60-7.
- Messmer BJ. Extended myectomy for hypertrophic obstructive cardiomyopathy. Ann Thorac Surg. 1994;58(2):575-7.
- Swistel DG, DeRose JJ Jr, Sherrid MV. Management of patients with complex hypertrophic cardiomyopathy: resection/plication/ release. Oper Tech Thorac Cardiovasc Surg. 2004;9(4):261-267. doi: http://dx.doi.org/10.1053/j.optechstcvs.2004.10.002.
- Bokeria LA. Hypertrophic obstructive cardiomyopathy. Annaly Khirurgii [Annals of Surgery]. 2013;5:5-14. (In Russ).
- Malekov DA, Sanakoev MK, Nehaj JA, Tetvadze IV, Berseneva MI, Bokeria LA. Our experience of two-ventricle approach for surgical treatment of hypertrophic cardiomyopahy. Byulleten’ Nauchnogo Tsentra Serdechno-Sosudistoy Khirurgii imeni A.N. Bakuleva Rossiyskoy Academii Meditsinskikh Nauk Serdechno-sosudistye zabolevaniya [The Bulletin of A.N. Bakoulev Scientific Center for Cardiovascular Surgery of Russian Academy of Medical Sciences CARDIOVASCULAR ASCULAR DISEASES]. 2015;16(S3):95. (In Russ).
- Bokeria LA, Borisov KV, Sinev AF. Original approach for surgical correction of hypertrophic cardiomyopathy. Grudnaya i Serdechno-Sosudistaya Khirurgiya [Thoracic and Cardiovascular Surgery]. 1997;S2:80. (In Russ).
- Bogachev-Prokophiev A, Zheleznev S, Fomenko M, Pivkin A, Afanasiev A, Sharifulin R, Karaskov A. Concomitant ablation for atrial fibrillation during septal myectomy in patients with hypertrophic obstructive cardiomyopathy. Journal of Cardiothoracic Surgery. 2015;10(Suppl 1):A139. doi:10.1186/1749-8090-10-S1-A139.
- Bogachev-Prokophiev A, Zheleznev S, Fomenko M, Pivkin A, Afanasiev A, Sharifulin R, Karaskov A. Mitral Subvalvular Apparatus Intervention In Patients With Obstructive Hypertrophic Cardiomyopathy: does it need? Journal of Cardiothoracic Surgery. 2015;10(Suppl 1):A138.
- Ommen SR,Maron BJ,Olivotto I,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(3):470-476.
- Jeanrenaud X, Goy JJ, Kappenberger L. Effects of dual-chamber pacing in hypertrophic obstructive cardiomyopathy. Lancet. 1992;339(8805):1318-23.
- Slade AKB, Sadoul N, Shapiro L, Chojnowska L, Simon JP, Saumarez RC, Dodinot B, Camm AJ, McKenna WJ, Aliot E. DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience. Heart. 1996;75(1):44-9.
- Fananapazir L, Epstein ND, Curiel RV, Panza JA, Tripodi D, McAreavey D. 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. 1994;90(6):2731-42.
- Nishimura RA, Trusty JM, Hayes DL, Ilstrup DM, Larson DR, Hayes SN, Allison TG, Tajik AJ. Dual-chamber pacing for hypertrophic cardiomyopathy: a randomized, double-blind cross- over study. J Am Coll Cardiol. 1997;29(2):435-41.
- Maron BJ, Nishimura RA, McKenna WJ, Rakowski H, Josephson ME, Kieval RS. Assessment of permanent dual-chamber pacing as a treatment for drug-refractory symptomatic patients with obstructive hypertrophic cardiomyopathy. A randomized, double-blind, crossover study (M-PATHY). 1999;99(22):2927-33.
- Linde C, Gadler F, Kappenberger L, Rydén L. Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy. Am J Cardiol. 1999;83(6):903-7.
- Sigwart U. Non-surgical myocardial reduction of hypertrophic obstructive cardiomyopathy. Lancet. 1995;346(8969):211-4.
- Kuhn HJ. The history of alcohol septal ablation. Cardiovascular Revascularization Medicine. 2010;11(4):260-261.
- Goodwin JF, Oakley CM. Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy. Lancet. 1995;346(8990):1624.
- Sigwart U. Author's reply. Lancet. 1995.346(1995):1624.
- Maron BJ, Shen WK, Link MS, Epstein AE, Almquist AK, Daubert JP, Bardy GH, Favale S, Rea RF, Boriani G, Estes NA 3rd, Spirito P. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N Engl J Med. 2000;342(6):365-73.
- Maron BJ, Spirito P, Shen WK, Haas TS, Formisano F, Link MS, Epstein AE, Almquist AK, Daubert JP, Lawrenz T, Boriani G, Estes NA 3rd, Favale S, Piccininno M, Winters SL, Santini M, Betocchi S, Arribas F, Sherrid MV, Buja G, Semsarian C, Bruzzi P. Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy. J Am Med Assoc. 2007;298(4):405-12.
- Maron BJ, Spirito P, Ackerman MJ, Casey SA, Semsarian C, Estes NA 3rd, Shannon KM, Ashley EA, Day SM, Pacileo G, Formisano F, Devoto E, Anastasakis A, Bos JM, Woo A, Autore C, Pass RH, Boriani G, Garberich RF, Almquist AK, Russell MW, Boni L, Berger S, Maron MS, Link MS. Prevention of sudden cardiac death with the implantable cardioverter- defibrillator in children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2013;61(14):1527-35.
- Bogachev-Prokofiev AV, Zheleznev SI, Fomenko MS, Afanasyev AV, Sharifullin RM, Nazarov VM, Malakhova OYu, Karaskov AM. Mitral valve replacement or repair: choosing optimal surgical treatment of hypertrophic obstructive cardiomyopathy. Patologiya krovoobrashcheniya i kardiokhirurgiya [Circulation Pathology and Cardiac Surgery]. 2015;19(4):26-33. (In Russ.).
- Bogachev-Prokofiev AV, Zheleznev SI, Fomenko MS, Afanasyev AV, Sharifullin RM, Pivkin AN, Demidov DP, Karaskov AM. Аpplication extanded myoectomy in surgical treatment obstructive hypertrophic cardiomyopathy: immediate results. Patologiya krovoobrashcheniya i kardiokhirurgiya [Circulation Pathology and Cardiac Surgery]. 2015;19(2):20-25. (In Russ.).