Progress in Cardiovascular Diseases
Volume 49, Issue 2 , Pages 123-151 , September 2006

Pathophysiology and Treatment of Hypertrophic Cardiomyopathy

  • Mark V. Sherrid

      Affiliations

    • Corresponding Author InformationAddress reprint requests to Mark V. Sherrid, MD, 1000 10th Avenue 3B-30, New York City, NY 10019.

References 

  1. Teare D. Asymmetrical hypertrophy of the heart in young adults. Br. Heart J. 1958;20:1–18
  2. Seidman JG, Seidman C. The genetic basis for cardiomyopathy: from mutation identification to mechanistic paradigms. Cell. 2001;104:557–567
  3. Mogensen J, Bahl A, McKenna WJ. Hypertrophic cardiomyopathy—the clinical challenge of managing a hereditary heart condition. Eur. Heart J. 2003;24:496–498
  4. Watkins H, Rosenzweig A, Hwang DS, et al. Characteristics and prognostic implications of myosin missense mutations in familial hypertrophic cardiomyopathy. N. Engl. J. Med. 1992;326:1108–1114
  5. Van Driest SL, Ommen SR, Tajik AJ, et al. Yield of genetic testing in hypertrophic cardiomyopathy. Mayo Clin. Proc. 2005;80:739–744
  6. Binder J, Ommen SR, Gersh BJ, et al. Echocardiography-guided genetic testing in hypertrophic cardiomyopathy: septal morphological features predict the presence of myofilament mutations. Mayo Clin. Proc. 2006;4:459–467
  7. Lever HM, Karam RF, Currie PJ, et al. Hypertrophic cardiomyopathy in the elderly. Distinctions from the young based on cardiac shape. Circulation. 1989;79:580–589
  8. Marian AJ. Pathogenesis of diverse clinical and pathological phenotypes in hypertrophic cardiomyopathy. Lancet. 2000;355:58–60
  9. Maron BJ, McKenna WJ, Danielson GK, et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J. Am. Coll. Cardiol. 2003;42:1687–1713
  10. Maron BJ, Epstein SE. Hypertrophic cardiomyopathy: a discussion of nomenclature. Am. J. Cardiol. 1979;43:1242–1244
  11. Wigle ED, Sasson Z, Henderson MA, et al. Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. Prog. Cardiovasc. Dis. 1985;28:1–83
  12. Wigle ED. Hypertrophic cardiomyopathy: a 1987 viewpoint. Circulation. 1987;75:311–322
  13. Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA. 2002;287:1308–1320
  14. Ommen SR, Nishimura RA. Hypertrophic cardiomyopathy. Curr. Probl. Cardiol. 2004;29:239–291
  15. Eriksson MJ, Sonnenberg B, Woo A, et al. Long-term outcome in patients with apical hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2002;39:638–645
  16. Rickers C, Wilke NM, Jerosch-Herold M, et al. Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation. 2005;112:855–861
  17. Maron BJ, Wolfson JK, Roberts WC. Relation between extent of cardiac muscle cell disorganization and left ventricular wall thickness in hypertrophic cardiomyopathy. Am. J. Cardiol. 1992;70:785–790
  18. Maron BJ, Anan TJ, Roberts WC. Quantitative analysis of the distribution of cardiac muscle cell disorganization in the left ventricular wall of patients with hypertrophic cardiomyopathy. Circulation. 1981;63:882–894
  19. Varnava AM, Elliott PM, Baboonian C, et al. Hypertrophic cardiomyopathy: histopathological features of sudden death in cardiac troponin T disease. Circulation. 2001;104:1380–1384
  20. Shirani J, Pick R, Roberts WC, et al. Morphology and significance of the left ventricular collagen network in young patients with hypertrophic cardiomyopathy and sudden cardiac death. J. Am. Coll. Cardiol. 2000;35:36–44
  21. St John Sutton MG, Lie JT, Anderson KR, et al. Histopathological specificity of hypertrophic obstructive cardiomyopathy. Myocardial fibre disarray and myocardial fibrosis. Br. Heart J. 1980;44:433–443
  22. Tanaka M, Fujiwara H, Onodera T, et al. Quantitative analysis of myocardial fibrosis in normals, hypertensive hearts, and hypertrophic cardiomyopathy. Br. Heart J. 1986;55:575–581
  23. Factor SM, Butany J, Sole MJ, et al. Pathologic fibrosis and matrix connective tissue in the subaortic myocardium of patients with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1991;17:1343–1351
  24. Nihoyannopoulos P, Karatasakis G, Frenneaux M, et al. Diastolic function in hypertrophic cardiomyopathy: relation to exercise capacity. J. Am. Coll. Cardiol. 1992;19:536–540
  25. Nagueh SF, Lakkis NM, Middleton KJ, et al. Changes in left ventricular diastolic function 6 months after nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy. Circulation. 1999;99:344–347
  26. Varnava AM, Elliott PM, Mahon N, et al. Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy. Am. J. Cardiol. 2001;88:275–279
  27. Maron BJ, Wolfson JK, Epstein SE, et al. Intramural (“small vessel”) coronary artery disease in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1986;8:545–557
  28. Sherrid MV, Mahenthiran J, Casteneda V, et al. Comparison of diastolic septal perforator flow velocities in hypertrophic cardiomyopathy versus hypertensive left ventricular hypertrophy. Am. J. Cardiol. 2006;97:106–112
  29. Maron BJ, Epstein SE, Roberts WC. Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. Am. J. Cardiol. 1979;43:1086–1102
  30. Maron MS, Olivotto I, Betocchi S, et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N. Engl. J. Med. 2003;348:295–303
  31. Sherrid MV, Chu CK, Delia E, et al. An echocardiographic study of the fluid mechanics of obstruction in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1993;22:816–825
  32. Sherrid MV, Gunsburg DZ, Moldenhauer S, et al. Systolic anterior motion begins at low left ventricular outflow tract velocity in obstructive hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2000;36:1344–1354
  33. Shah PM, Gramiak R, Kramer DH. Ultrasound localization of left ventricular outflow obstruction in hypertrophic obstructive cardiomyopathy. Circulation. 1969;40:3–11
  34. Henry WL, Clark CE, Griffith JM, et al. Mechanism of left ventricular outflow obstruction in patients with obstructive asymmetric septal hypertrophy (idiopathic hypertrophic subaortic stenosis). Am. J. Cardiol. 1975;35:337–345
  35. Schwammenthal E, Levine RA. Dynamic subaortic obstruction: a disease of the mitral valve suitable for surgical repair?. J. Am. Coll. Cardiol. 1996;28:203–206
  36. Murgo JP. Does outflow obstruction exist in hypertrophic cardiomyopathy?. N. Engl. J. Med. 1982;307:1008–1009
  37. Criley JM, Siegel RJ. Has ‘obstruction’ hindered our understanding of hypertrophic cardiomyopathy?. Circulation. 1985;72:1148–1154
  38. Sherrid MV, Gunsburg DZ, Pearle G. Mid-systolic drop in left ventricular ejection velocity in obstructive hypertrophic cardiomyopathy—the lobster claw abnormality. J. Am. Soc. Echocardiogr. 1997;10:707–712
  39. Conklin HM, Huang X, Davies CH, et al. Biphasic left ventricular outflow and its mechanism in hypertrophic obstructive cardiomyopathy. J. Am. Soc. Echocardiogr. 2004;17:375–383
  40. Barac I, Pilchik R, Shteerman E, et al. Midsystolic drop in left ventricular ejection velocity in hypertrophic cardiomyopathy is caused by premature termination and dyssynchrony of longitudinal contraction. Doppler flow and tissue velocity study of the “lobster claw abnormality” (abstract). Circulation. 2004;110(Suppl III):746
  41. 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;84:1188–1197
  42. Falicov RE, Resnekov L, Bharati S, et al. Mid-ventricular obstruction: a variant of obstructive cardiomyopathy. Am. J. Cardiol. 1976;37:432–437
  43. Nakamura T, Matsubara K, Furukawa K, et al. Diastolic paradoxic jet flow in patients with hypertrophic cardiomyopathy: evidence of concealed apical asynergy with cavity obliteration. J. Am. Coll. Cardiol. 1992;19:516–524
  44. Sasson Z, Yock PG, Hatle LK, et al. Doppler echocardiographic determination of the pressure gradient in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1988;11:752–756
  45. Marwick TH, Nakatani S, Haluska B, et al. Provocation of latent left ventricular outflow tract gradients with amyl nitrite and exercise in hypertrophic cardiomyopathy. Am. J. Cardiol. 1995;75:805–809
  46. Klues HG, Maron BJ, Dollar AL, et al. Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy. Circulation. 1992;85:1651–1660
  47. Cape EG, Simons D, Jimoh A, et al. Chordal geometry determines the shape and extent of systolic anterior mitral motion: in vitro studies. J. Am. Coll. Cardiol. 1989;13:1438–1448
  48. Jiang L, Levine RA, King ME, et al. An integrated mechanism for systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy based on echocardiographic observations. Am. Heart J. 1987;113:633–644
  49. Shah PM, Taylor RD, Wong M. Abnormal mitral valve coaptation in hypertrophic obstructive cardiomyopathy: proposed role in systolic anterior motion of mitral valve. Am. J. Cardiol. 1981;48:258–262
  50. Levine RA, Vlahakes GJ, Lefebvre X, et al. Papillary muscle displacement causes systolic anterior motion of the mitral valve. Experimental validation and insights into the mechanism of subaortic obstruction. Circulation. 1995;91:1189–1195
  51. Lefebvre XP, Yoganathan AP, Levine RA. Insights from in-vitro flow visualization into the mechanism of systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy under steady flow conditions. J. Biomech. Eng. 1992;114:406–413
  52. Vogel S. Life in moving fluids. In: The physical biology of flow. Princeton, NJ: Princeton University Press; 1994;p. 81–155
  53. Spirito P, Maron BJ. Patterns of systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy: assessment by two-dimensional echocardiography. Am. J. Cardiol. 1984;54:1039–1046
  54. Maron BJ, Harding AM, Spirito P, et al. Systolic anterior motion of the posterior mitral leaflet: a previously unrecognized cause of dynamic subaortic obstruction in patients with hypertrophic cardiomyopathy. Circulation. 1983;68:282–293
  55. Sherrid MV, Chaudhry FA, Swistel DG. Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and the continuing evolution of surgery for obstruction. Ann. Thorac. Surg. 2003;75:620–632
  56. Roberts CS, McIntosh CL, Brown PS, et al. Reoperation for persistent outflow obstruction in hypertrophic cardiomyopathy. Ann. Thorac. Surg. 1991;51:455–460
  57. Messmer BJ. Extended myectomy for hypertrophic obstructive cardiomyopathy. Ann. Thorac. Surg. 1994;58:575–577
  58. Schoendube FA, Klues HG, Reith S, et al. Long-term clinical and echocardiographic follow-up after surgical correction of hypertrophic obstructive cardiomyopathy with extended myectomy and reconstruction of the subvalvular mitral apparatus. Circulation. 1995;92:II122–II127
  59. Nakatani S, Schwammenthal E, Lever HM, et al. New insights into the reduction of mitral valve systolic anterior motion after ventricular septal myectomy in hypertrophic obstructive cardiomyopathy. Am. Heart J. 1996;131:294–300
  60. Grigg LE, Wigle ED, Williams WG, et al. Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy: clarification of pathophysiology and importance in intraoperative decision making. J. Am. Coll. Cardiol. 1992;20:42–52
  61. Balaram SK, Sherrid MV, Derose JJ. Beyond extended myectomy for hypertrophic cardiomyopathy: the resection-plication-release (RPR) repair. Ann. Thorac. Surg. 2005;80:217–223
  62. McIntosh CL, Maron BJ, Cannon RO. 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:II60–II67
  63. Spirito P, Rapezzi C, Bellone P, et al. Infective endocarditis in hypertrophic cardiomyopathy: prevalence, incidence, and indications for antibiotic prophylaxis. Circulation. 1999;99:2132–2137
  64. Sorajja P, Ommen SR, Nishimura RA, et al. Adverse prognosis of patients with hypertrophic cardiomyopathy who have epicardial coronary artery disease. Circulation. 2003;108:2342–2348
  65. Spirito P, Bellone P, Harris KM, et al. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N. Engl. J. Med. 2000;342:1778–1785
  66. Elliott PM, Gimeno J, Blanes R, et al. Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy. Lancet. 2001;357:420–424
  67. Maron BJ, Shen WK, Link MS, et al. Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. N. Engl. J. Med. 2000;342:365–373
  68. Watkins H, McKenna WJ, Thierfelder L, et al. Mutations in the genes for cardiac troponin T and alpha-tropomyosin in hypertrophic cardiomyopathy. N. Engl. J. Med. 1995;332:1058–1064
  69. Maron BJ, Olivotto I, Spirito P, et al. Epidemiology of hypertrophic cardiomyopathy-related death: revisited in a large non–referral-based patient population. Circulation. 2000;102:858–864
  70. Maron BJ, Roberts WC, Edwards JE, et al. Sudden death in patients with hypertrophic cardiomyopathy: characterization of 26 patients with functional limitation. Am. J. Cardiol. 1978;41:803–810
  71. Elliott PM, Poloniecki J, Dickie S, et al. Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J. Am. Coll. Cardiol. 2000;36:2212–2218
  72. Maron BJ, Roberts WC, Epstein SE. Sudden death in hypertrophic cardiomyopathy: a profile of 78 patients. Circulation. 1982;65:1388–1394
  73. Maron BJ, Casey SA, Poliac LC, et al. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA. 1999;281:650–655
  74. McKenna WJ, Franklin RC, Nihoyannopoulos P, et al. Arrhythmia and prognosis in infants, children and adolescents with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1988;11:147–153
  75. McKenna W, Deanfield J, Faruqui A, et al. Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features. Am. J. Cardiol. 1981;47:532–538
  76. Maron BJ, Mathenge R, Casey SA, et al. Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J. Am. Coll. Cardiol. 1999;33:1590–1595
  77. Cecchi F, Olivotto I, Montereggi A, et al. Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J. Am. Coll. Cardiol. 1995;26:1529–1536
  78. Maron BJ, Olivotto I, Spirito P, et al. Epidemiology of hypertrophic cardiomyopathy–related death: revised in a large non–referral-based patient population. Circulation. 2000;102:858–864
  79. Maron BJ, Spirito P. Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history. Am. J. Cardiol. 1993;72:970–972
  80. Fay WP, Taliercio CP, Ilstrup DM, et al. Natural history of hypertrophic cardiomyopathy in the elderly. J. Am. Coll. Cardiol. 1990;16:821–826
  81. Elliott PM, Sharma S, Varnava A, et al. Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1999;33:1596–1601
  82. Maron BJ, Lipson LC, Roberts WC, et al. “Malignant” hypertrophic cardiomyopathy: identification of a subgroup of families with unusually frequent premature death. Am. J. Cardiol. 1978;41:1133–1140
  83. Monserrat L, Elliott PM, Gimeno JR, et al. Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J. Am. Coll. Cardiol. 2003;42:873–879
  84. Sadoul N, Prasad K, Elliott PM, et al. Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy. Circulation. 1997;96:2987–2991
  85. Olivotto I, Maron BJ, Montereggi A, et al. Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1999;33:2044–2051
  86. Ciampi Q, Betocchi S, Lombardi R, et al. Hemodynamic determinants of exercise-induced abnormal blood pressure response in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2002;40:278–284
  87. Cecchi F, Olivotto I, Montereggi A, et al. Prognostic value of non-sustained ventricular tachycardia and the potential role of amiodarone treatment in hypertrophic cardiomyopathy: assessment in an unselected non–referral based patient population. Heart. 1998;79:331–336
  88. Van Driest SL, Ellsworth EG, Ommen SR, et al. Prevalence and spectrum of thin filament mutations in an outpatient referral population with hypertrophic cardiomyopathy. Circulation. 2003;108:445–451
  89. Mogensen J, Murphy RT, Kubo T, et al. Frequency and clinical expression of cardiac troponin I mutations in 748 consecutive families with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2004;44:2315–2325
  90. Ackerman MJ, VanDriest SL, Ommen SR, et al. Prevalence and age-dependence of malignant mutations in the beta-myosin heavy chain and troponin T genes in hypertrophic cardiomyopathy: a comprehensive outpatient perspective. J. Am. Coll. Cardiol. 2002;39:2042–2048
  91. Van Driest SL, Ackerman MJ, Ommen SR, et al. Prevalence and severity of “benign” mutations in the beta-myosin heavy chain, cardiac troponin T, and alpha-tropomyosin genes in hypertrophic cardiomyopathy. Circulation. 2002;106:3085–3090
  92. Marian AJ, Yu QT, Workman R, et al. Angiotensin-converting enzyme polymorphism in hypertrophic cardiomyopathy and sudden cardiac death. Lancet. 1993;342:1085–1086
  93. Lechin M, Quinones MA, Omran A, et al. Angiotensin-I converting enzyme genotypes and left ventricular hypertrophy in patients with hypertrophic cardiomyopathy. Circulation. 1995;92:1808–1812
  94. Saad EB, Saliba WI, Schweikert RA, et al. Nonthoracotomy implantable defibrillator lead extraction: results and comparison with extraction of pacemaker leads. Pacing Clin. Electrophysiol. 2003;26:1944–1950
  95. Maron BJ, Shirani J, Poliac LC, et al. Sudden death in young competitive athletes. Clinical, demographic, and pathological profiles. JAMA. 1996;276:199–204
  96. Maron BJ, Isner JM, McKenna WJ. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 3: hypertrophic cardiomyopathy, myocarditis and other myopericardial diseases and mitral valve prolapse. J. Am. Coll. Cardiol. 1994;24:880–885
  97. Maron BJ, Mitten MJ, Quandt EF, et al. Competitive athletes with cardiovascular disease—the case of Nicholas Knapp. N. Engl. J. Med. 1998;339:1632–1635
  98. Lele SS, Thomson HL, Seo H, et al. Exercise capacity in hypertrophic cardiomyopathy. Role of stroke volume limitation, heart rate, and diastolic filling characteristics. Circulation. 1995;92:2886–2894
  99. Wigle ED, Rakowski H, Kimball BP, et al. Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation. 1995;92:1680–1692
  100. Schwammenthal E, Nakatani S, He S, et al. Mechanism of mitral regurgitation in hypertrophic cardiomyopathy: mismatch of posterior to anterior leaflet length and mobility. Circulation. 1998;98:856–865
  101. Cecchi F, Olivotto I, Gistri R, et al. Coronary microvascular dysfunction and prognosis in hypertrophic cardiomyopathy. N. Engl. J. Med. 2003;349:1027–1035
  102. Cannon RO, Rosing DR, Maron BJ, et al. Myocardial ischemia in patients with hypertrophic cardiomyopathy: contribution of inadequate vasodilator reserve and elevated left ventricular filling pressures. Circulation. 1985;71:234–243
  103. Krams R, Kofflard MJ, Duncker DJ, et al. Decreased coronary flow reserve in hypertrophic cardiomyopathy is related to remodeling of the coronary microcirculation. Circulation. 1998;97:230–233
  104. Cannon RO, Schenke WH, Maron BJ, et al. Differences in coronary flow and myocardial metabolism at rest and during pacing between patients with obstructive and patients with nonobstructive hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1987;10:53–62
  105. Nienaber CA, Hiller S, Spielmann RP, et al. Syncope in hypertrophic cardiomyopathy: multivariate analysis of prognostic determinants. J. Am. Coll. Cardiol. 1990;15:948–955
  106. McKenna W, Harris L, Deanfield J. Syncope in hypertrophic cardiomyopathy. Br. Heart J. 1982;47:177–179
  107. Frenneaux MP, Counihan PJ, Caforio AL, et al. Abnormal blood pressure response during exercise in hypertrophic cardiomyopathy. Circulation. 1990;82:1995–2002
  108. Maron BJ, Casey SA, Hauser RG, et al. Clinical course of hypertrophic cardiomyopathy with survival to advanced age. J. Am. Coll. Cardiol. 2003;42:882–888
  109. Kass DA, Wolff MR, Ting CT, et al. Diastolic compliance of hypertrophied ventricle is not acutely altered by pharmacologic agents influencing active processes. Ann. Intern. Med. 1993;119:466–473
  110. TenCate FJ, Serruys PW, Mey S, et al. Effects of short-term administration of verapamil on left ventricular relaxation and filling dynamics measured by a combined hemodynamic-ultrasonic technique in patients with hypertrophic cardiomyopathy. Circulation. 1983;68:1274–1279
  111. Sherrid M, Barac I. Pharmacologic treatment of symptomatic hypertrophic cardiomyopathy. In:  Maron B editors. Diagnosis and management of hypertrophic cardiomyopathy. London: Blackwell-Futura; 2004;p. 200–219
  112. Bonow RO, Ostrow HG, Rosing DR, et al. Effects of verapamil on left ventricular systolic and diastolic function in patients with hypertrophic cardiomyopathy: pressure-volume analysis with a nonimaging scintillation probe. Circulation. 1983;68:1062–1073
  113. Bonow RO. Effects of calcium-channel blocking agents on left ventricular diastolic function in hypertrophic cardiomyopathy and in coronary artery disease. Am. J. Cardiol. 1985;55:172B–178B
  114. Bonow RO, Rosing DR, Bacharach SL, et al. Effects of verapamil on left ventricular systolic function and diastolic filling in patients with hypertrophic cardiomyopathy. Circulation. 1981;64:787–796
  115. Nishimura RA, Holmes DR, Tajik AJ. Failure of calcium channel blockers to improve ventricular relaxation in humans. J. Am. Coll. Cardiol. 1993;21:182–188
  116. Udelson JE, Bonow RO, O'Gara PT, et al. Verapamil prevents silent myocardial perfusion abnormalities during exercise in asymptomatic patients with hypertrophic cardiomyopathy. Circulation. 1989;79:1052–1060
  117. Gilligan DM, Chan WL, Joshi J, et al. A double-blind, placebo-controlled crossover trial of nadolol and verapamil in mild and moderately symptomatic hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1993;21:1672–1679
  118. Matsubara H, Nakatani S, Nagata S, et al. Salutary effect of disopyramide on left ventricular diastolic function in hypertrophic obstructive cardiomyopathy. J. Am. Coll. Cardiol. 1995;26:768–775
  119. Pollick C, Kimball B, Henderson M, et al. Disopyramide in hypertrophic cardiomyopathy: I. Hemodynamic assessment after intravenous administration. Am. J. Cardiol. 1988;62:1248–1251
  120. Tsybouleva N, Zhang L, Chen S, et al. Aldosterone, through novel signaling proteins, is a fundamental molecular bridge between the genetic defect and the cardiac phenotype of hypertrophic cardiomyopathy. Circulation. 2004;109:1284–1291
  121. Senthil V, Chen SN, Tsybouleva N, et al. Prevention of cardiac hypertrophy by atorvastatin in a transgenic rabbit model of human hypertrophic cardiomyopathy. Circ. Res. 2005;97:285–292
  122. Sherrid MV, Pearle G, Gunsburg DZ. Mechanism of benefit of negative inotropes in obstructive hypertrophic cardiomyopathy. Circulation. 1998;97:41–47
  123. Flamm MD, Harrison DC, Hancock EW. Muscular subaortic stenosis. Prevention of outflow obstruction with propranolol. Circulation. 1968;38:846–858
  124. Stenson RE, Flamm MD, Harrison DC, et al. Hypertrophic subaortic stenosis. Clinical and hemodynamic effects of long-term propranolol therapy. Am. J. Cardiol. 1973;31:763–773
  125. Rosing DR, Condit JR, Maron BJ, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: III. Effects of long-term administration. Am. J. Cardiol. 1981;48:545–553
  126. Rosing DR, Kent KM, Maron BJ, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: II. Effects on exercise capacity and symptomatic status. Circulation. 1979;60:1208–1213
  127. Sherrid MV, Barac I, McKenna WJ, et al. Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2005;45:1251–1258
  128. Pollick C. Muscular subaortic stenosis: hemodynamic and clinical improvement after disopyramide. N. Engl. J. Med. 1982;307:997–999
  129. Sherrid M, Delia E, Dwyer E. Oral disopyramide therapy for obstructive hypertrophic cardiomyopathy. Am. J. Cardiol. 1988;62:1085–1088
  130. Pollick C. Disopyramide in hypertrophic cardiomyopathy: II. Noninvasive assessment after oral administration. Am. J. Cardiol. 1988;62:1252–1255
  131. Rosing DR, Idanpaan-Heikkila U, Maron BJ, et al. Use of calcium-channel blocking drugs in hypertrophic cardiomyopathy. Am. J. Cardiol. 1985;55:185B–195B
  132. Rosing DR, Kent KM, Borer JS, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: I. Hemodynamic effects. Circulation. 1979;60:1201–1207
  133. Epstein SE, Rosing DR. Verapamil: its potential for causing serious complications in patients with hypertrophic cardiomyopathy. Circulation. 1981;64:437–441
  134. Pollick CGK, Blaschke TF, Nelson WL, et al. The cardiac effects of d- and l-disopyramide in normal subjects: a noninvasive study. Circulation. 1982;66:447–453
  135. Olivotto I, Maron BJ, Cecchi F. Clinical significance of atrial fibrillation in hypertrophic cardiomyopathy. Curr. Cardiol. Rep. 2001;3:141–146
  136. Olivotto I, Cecchi F, Casey SA, et al. Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation. 2001;104:2517–2524
  137. Maron BJ, Olivotto I, Bellone P, et al. Clinical profile of stroke in 900 patients with hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2002;39:301–307
  138. Robinson K, Frenneaux MP, Stockins B, et al. Atrial fibrillation in hypertrophic cardiomyopathy: a longitudinal study. J. Am. Coll. Cardiol. 1990;15:1279–1285
  139. Herweg B, Polosajian L, Vloka M, et al. Anatomic substrate, procedural results and clinical outcome of ultrasound-guided left atrial–pulmonary vein disconnection for treatment of atrial fibrillation. Am. J. Cardiol. 2005;95:871–875
  140. Williams WG, Wigle ED, Rakowski H, et al. Results of surgery for hypertrophic obstructive cardiomyopathy. Circulation. 1987;76:V104–V108
  141. Maron BJ, Dearani JA, Ommen SR, et al. The case for surgery in obstructive hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 2004;44:2044–2053
  142. 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;46:470–476
  143. McCully RB, Nishimura RA, Bailey KR, et al. Hypertrophic obstructive cardiomyopathy: preoperative echocardiographic predictors of outcome after septal myectomy. J. Am. Coll. Cardiol. 1996;27:1491–1496
  144. McCully RB, Nishimura RA, Tajik AJ, et al. Extent of clinical improvement after surgical treatment of hypertrophic obstructive cardiomyopathy. Circulation. 1996;94:467–471
  145. Schulte HD, Borisov K, Gams E, et al. Management of symptomatic hypertrophic obstructive cardiomyopathy—long-term results after surgical therapy. Thorac. Cardiovasc. Surg. 1999;47:213–218
  146. Schulte HD, Bircks WH, Loesse B, et al. Prognosis of patients with hypertrophic obstructive cardiomyopathy after transaortic myectomy. Late results up to twenty-five years. J. Thorac. Cardiovasc. Surg. 1993;106:709–717
  147. Schoendube FA, Klues HG, Reith S, et al. Surgical correction of hypertrophic obstructive cardiomyopathy with combined myectomy, mobilisation and partial excision of the papillary muscles. Eur. J. Cardiothorac. Surg. 1994;8:603–608
  148. Heric B, Lytle BW, Miller DP, et al. Surgical management of hypertrophic obstructive cardiomyopathy. Early and late results. J. Thorac. Cardiovasc. Surg. 1995;110:195–206[discussion 206-8]
  149. 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. Am. Coll. Cardiol. 2001;38:1994–2000
  150. Marwick TH, Stewart WJ, Lever HM, et al. Benefits of intraoperative echocardiography in the surgical management of hypertrophic cardiomyopathy. J. Am. Coll. Cardiol. 1992;20:1066–1072
  151. Ommen SR, Park SH, Click RL, et al. Impact of intraoperative transesophageal echocardiography in the surgical management of hypertrophic cardiomyopathy. Am. J. Cardiol. 2002;90:1022–1024
  152. Krajcer Z, Leachman RD, Cooley DA, et al. Mitral valve replacement and septal myomectomy in hypertrophic cardiomyopathy. Ten-year follow-up in 80 patients. Circulation. 1988;78:I35–I43
  153. Roberts WC. Operative treatment of hypertrophic obstructive cardiomyopathy. The case against mitral valve replacement. Am. J. Cardiol. 1973;32:377–381
  154. Seggewiss H, Gleichmann U, Faber L, et al. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: acute results and 3-month follow-up in 25 patients. J. Am. Coll. Cardiol. 1998;31:252–258
  155. Lakkis NM, Nagueh SF, Kleiman NS, et al. Echocardiography-guided ethanol septal reduction for hypertrophic obstructive cardiomyopathy. Circulation. 1998;98:1750–1755
  156. Nagueh SF, Lakkis NM, He ZX, et al. Role of myocardial contrast echocardiography during nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy. J. Am. Coll. Cardiol. 1998;32:225–229
  157. Faber L, Seggewiss H, Fassbender D, et al. Percutaneous transluminal septal myocardial ablation in hypertrophic obstructive cardiomyopathy: acute results in 66 patients with reference to myocardial contrast echocardiography. Z. Kardiol. 1998;87:191–201
  158. Faber L, Ziemssen P, Seggewiss H. Targeting percutaneous transluminal septal ablation for hypertrophic obstructive cardiomyopathy by intraprocedural echocardiographic monitoring. J. Am. Soc. Echocardiogr. 2000;13:1074–1079
  159. Gietzen FH, Leuner CJ, Obergassel L, et al. Role of transcoronary ablation of septal hypertrophy in patients with hypertrophic cardiomyopathy, New York Heart Association functional class III or IV, and outflow obstruction only under provocable conditions. Circulation. 2002;106:454–459
  160. Lakkis N, Plana JC, Nagueh S, et al. Efficacy of nonsurgical septal reduction therapy in symptomatic patients with obstructive hypertrophic cardiomyopathy and provocable gradients. Am. J. Cardiol. 2001;88:583–586
  161. Faber L, Seggewiss H, Welge D, et al. Echo-guided percutaneous septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: 7 years of experience. Eur. J. Echocardiogr. 2004;5:347–355
  162. Flores-Ramirez R, Lakkis NM, Middleton KJ, et al. Echocardiographic insights into the mechanisms of relief of left ventricular outflow tract obstruction after nonsurgical septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy. J. Am. Coll. Cardiol. 2001;37:208–214
  163. Fernandes VL, Nagueh SF, Wang W, et al. A prospective follow-up of alcohol septal ablation for symptomatic hypertrophic obstructive cardiomyopathy—the Baylor experience (1996-2002). Clin. Cardiol. 2005;28:124–130
  164. 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. Am. Coll. Cardiol. 2001;38:1701–1706
  165. Fananapazir L, Epstein ND, Curiel RV, et al. 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;90:2731–2742
  166. Maron BJ, Nishimura RA, McKenna WJ, et al. 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). Circulation. 1999;99:2927–2933
  167. Kappenberger L, Linde C, Daubert C, et al. Pacing in hypertrophic obstructive cardiomyopathy. A randomized crossover study. PIC Study Group. Eur. Heart J. 1997;18:1249–1256
  168. Jeanrenaud X, Kappenberger L. Regional wall motion during pacing for hypertrophic obstructive cardiomyopathy. Pacing Clin. Electrophysiol. 1997;20:1673–1681
  169. Nishimura RA, Hayes DL, Ilstrup DM, et al. Effect of dual-chamber pacing on systolic and diastolic function in patients with hypertrophic cardiomyopathy. Acute Doppler echocardiographic and catheterization hemodynamic study. J. Am. Coll. Cardiol. 1996;27:421–430
  170. Ommen SR, Nishimura RA, Squires RW, et al. 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;34:191–196
  171. Gadler F, Linde C, Daubert C, et al. Significant improvement of quality of life following atrioventricular synchronous pacing in patients with hypertrophic obstructive cardiomyopathy. Data from 1 year of follow-up. PIC study group. Pacing in cardiomyopathy. Eur. Heart J. 1999;20:1044–1050
  172. Linde C, Gadler F, Kappenberger L, et al. Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy. PIC Study Group. Pacing in cardiomyopathy. Am. J. Cardiol. 1999;83:903–907
  173. Nishimura RA, Trusty JM, Hayes DL, et al. Dual-chamber pacing for hypertrophic cardiomyopathy: a randomized, double-blind, crossover trial. J. Am. Coll. Cardiol. 1997;29:435–441
  174. Gadler F, Linde C, Ryden L. Rapid return of left ventricular outflow tract obstruction and symptoms following cessation of long-term atrioventricular synchronous pacing for obstructive hypertrophic cardiomyopathy. Am. J. Cardiol. 1999;83:553–557
  175. Topilski ISJ, Keren G, Copperman I. Long-term effects of dual-chamber pacing with periodic echocardiographic evaluation of optimal atrioventricular delay in patients with hypertrophic cardiomyopathy >50 years of age. Am. J. Cardiol. 2006;97:1769–1775
  176. Topol EJ, Traill TA, Fortuin NJ. Hypertensive hypertrophic cardiomyopathy of the elderly. N. Engl. J. Med. 1985;312:277–283
  177. Spirito P, Maron BJ, Bonow RO, et al. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am. J. Cardiol. 1987;60:123–129

PII: S0033-0620(06)00085-5

doi: 10.1016/j.pcad.2006.08.001

Progress in Cardiovascular Diseases
Volume 49, Issue 2 , Pages 123-151 , September 2006

Article Tools

* Image Usage & Resolution