0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Clinical Cardiology |

Hypertrophic Cardiomyopathy:  A Systematic Review FREE

Barry J. Maron, MD
[+] Author Affiliations

Author Affiliation: Minneapolis Heart Institute Foundation, Minneapolis, Minn.


JAMA. 2002;287(10):1308-1320. doi:10.1001/jama.287.10.1308.
Text Size: A A A
Published online

Context Throughout the past 40 years, a vast and sometimes contradictory literature has accumulated regarding hypertrophic cardiomyopathy (HCM), a genetic cardiac disease caused by a variety of mutations in genes encoding sarcomeric proteins and characterized by a broad and expanding clinical spectrum.

Objectives To clarify and summarize the relevant clinical issues and to profile rapidly evolving concepts regarding HCM.

Data Sources Systematic analysis of the relevant HCM literature, accessed through MEDLINE (1966-2000), bibliographies, and interactions with investigators.

Study Selection and Data Extraction Diverse information was assimilated into a rigorous and objective contemporary description of HCM, affording greatest weight to prospective, controlled, and evidence-based studies.

Data Synthesis Hypertrophic cardiomyopathy is a relatively common genetic cardiac disease (1:500 in the general population) that is heterogeneous with respect to disease-causing mutations, presentation, prognosis, and treatment strategies. Visibility attached to HCM relates largely to its recognition as the most common cause of sudden death in the young (including competitive athletes). Clinical diagnosis is by 2-dimensional echocardiographic identification of otherwise unexplained left ventricular wall thickening in the presence of a nondilated cavity. Overall, HCM confers an annual mortality rate of about 1% and in most patients is compatible with little or no disability and normal life expectancy. Subsets with higher mortality or morbidity are linked to the complications of sudden death, progressive heart failure, and atrial fibrillation with embolic stroke. Treatment strategies depend on appropriate patient selection, including drug treatment for exertional dyspnea (β-blockers, verapamil, disopyramide) and the septal myotomy-myectomy operation, which is the standard of care for severe refractory symptoms associated with marked outflow obstruction; alcohol septal ablation and pacing are alternatives to surgery for selected patients. High-risk patients may be treated effectively for sudden death prevention with the implantable cardioverter-defibrillator.

Conclusions Substantial understanding has evolved regarding the epidemiology and clinical course of HCM, as well as novel treatment strategies that may alter its natural history. An appreciation that HCM, although an important cause of death and disability at all ages, does not invariably convey ominous prognosis and is compatible with normal longevity should dictate a large measure of reassurance for many patients.

Hypertrophic cardiomyopathy (HCM) is a complex and relatively common genetic cardiac disease that has been the subject of intense scrutiny and investigation for more than 40 years.110 Hypertrophic cardiomyopathy is an important cause of disability and death in patients of all ages, although sudden and unexpected death in young people is perhaps the most devastating component of its natural history. Because of marked heterogeneity in clinical expression, natural history, and prognosis,1120 HCM often represents a dilemma to primary care clinicians and cardiovascular specialists, even to those for whom this disease is a focus of their investigative careers. Controversy abounds with regard to diagnostic criteria, clinical course, and management for which difficult questions often arise, particularly among practitioners infrequently engaged in the evaluation of HCM patients. Consequently, it is timely to place in perspective and clarify many of these relevant clinical issues and profile the rapidly evolving concepts regarding HCM.

Clinical Cardiology Section Editor: Michael S. Lauer, MD, Contributing Editor.
Teare D. Asymmetrical hypertrophy of the heart in young adults.  Br Heart J.1958;20:1-18.
Braunwald E, Lambrew CT, Rockoff D.  et al.  Idiopathic hypertrophic subaortic stenosis, I: a description of the disease based upon an analysis of 64 patients.  Circulation.1964;30(suppl IV):3-217.
Maron BJ. Hypertrophic cardiomyopathy.  Lancet.1997;350:127-133.
Maron BJ, Bonow RO, Cannon III RO, Leon MB, Epstein SE. Hypertrophic cardiomyopathy: interrelation of clinical manifestations, pathophysiology, and therapy.  N Engl J Med.1987;316:780-789, 844-852.
Frank S, Braunwald E. Idiopathic hypertrophic subaortic stenosis: clinical analysis of 126 patients with emphasis on the natural history.  Circulation.1968;37:759-788.
Wigle ED, Sasson Z, Henderson MA.  et al.  Hypertrophic cardiomyopathy: the importance of the site and extent of hypertrophy.  Prog Cardiovasc Dis.1985;28:1-83.
Spirito P, Seidman CE, McKenna WJ, Maron BJ. Management of hypertrophic cardiomyopathy.  N Engl J Med.1997;336:775-785.
Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy: clinical spectrum and treatment.  Circulation.1995;92:1680-1692.
Maron BJ, Moller JH, Seidman CE.  et al.  Impact of laboratory molecular diagnosis on contemporary diagnostic criteria for genetically transmitted cardiovascular diseases: hypertrophic cardiomyopathy, long-QT syndrme, and Marfan syndrome. A statement for healthcare professions from the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association.  Circulation.1998;98:1460-1471.
Louie EK, Edwards LC. Hypertrophic cardiomyopathy.  Prog Cardiovasc Dis.1994;36:275-308.
Marian AJ, Roberts R. Recent advances in the molecular genetics of hypertrophic cardiomyopathy.  Circulation.1995;92:1336-1347.
Schwartz K, Carrier L, Guicheney P, Komajda M. Molecular basis of familial cardiomyopathies.  Circulation.1995;91:532-540.
Niimura H, Bachinski LL, Sangwatanaroj S.  et al.  Mutations in the gene for human cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy.  N Engl J Med.1998;338:1248-1257.
Ciro E, Nichols III PF, Maron BJ. Heterogeneous morphologic expression of genetically transmitted hypertrophic cardiomyopathy.  Circulation.1983;67:1227-1233.
Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy.  Am J Cardiol.1981;48:418-428.
Shapiro LM, McKenna WJ. Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1983;2:437-444.
Klues HG, Roberts WC, Maron BJ. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in hypertrophic cardiomyopathy.  Circulation.1991;84:1188-1197.
Klues HG, Maron BJ, Dollar AL.  et al.  Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy.  Circulation.1992;85:1651-1660.
Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1995;26:1699-1708.
Maron BJ, Gardin JM, Flack JM.  et al.  Prevalence of hypertrophic cardiomyopathy in a general population of young adults.  Circulation.1995;92:785-789.
Maron BJ, Peterson EE, Maron MS, Peterson JE. Prevalence of hypertrophic cardiomyopathy in an outpatient population referred for echocardiographic study.  Am J Cardiol.1994;73:577-580.
Maron BJ, Epstein SE. Hypertrophic cardiomyopathy: a discussion of nomenclature.  Am J Cardiol.1979;43:1242-1244.
Watkins H, McKenna WJ, Thierfelder L.  et al.  The role of cardiac troponin T and α-tropomyosin mutations in hypertrophic cardiomyopathy.  N Engl J Med.1995;332:1058-1064.
Moolman JC, Corfield VA, Posen B.  et al.  Sudden death due to troponin T mutations.  J Am Coll Cardiol.1997;29:549-555.
Maron BJ, Niimura H, Casey SA.  et al.  Development of left ventricular hypertrophy in adults with hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C mutations.  J Am Coll Cardiol.2001;38:315-321.
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.
Anan R, Greve G, Thierfelder L.  et al.  Prognostic implications of novel β-cardiac myosin heavy chain gene mutations that cause familial hypertrophic cardiomyopathy.  J Clin Invest.1994;93:280-285.
Marian AJ. Pathogenesis of diverse clinical and pathological phenotypes in hypertrophic cardiomyopathy.  Lancet.2000;355:58-60.
Rosenzweig A, Watkins H, Hwang DS.  et al.  Preclinical diagnosis of familial hypertrophic cardiomyopathy by genetic analysis of blood lymphocytes.  N Engl J Med.1991;325:1753-1760.
Gollob MH, Green MS, Tang AS.  et al.  Identification of a gene responsible for familial Wolff-Parkinson-White syndrome.  N Engl J Med.2001;344:1823-1831.
Arad M, Benson DW, Perez-Atayde Not Available.  et al.  Constitutively active AMP kinase mutations cause glycogen storage disease mimicking hypertrophic cardiomyopathy.  J Clin Invest.2002;109:357-362.
Geisterfer-Lowrance AA, Christe M, Conner DA.  et al.  A mouse model of familial hypertrophic cardiomyopathy.  Science.1996;272:731-734.
Marian AJ, Wu Y, Lim DS.  et al.  A transgenic rabbit model for human hypertrophic cardiomyopathy.  J Clin Invest.1999;104:1683-1692.
Yang Q, Sanbe A, Osinska H, Hewett TE, Klevitsky R, Robbins J. A mouse model of myosin binding protein C human familial hypertrophic cardiomyopathy.  J Clin Invest.1998;102:1292-1300.
Lim DS, Lutucuta S, Bachireddy P.  et al.  Angiotensin II blockade reverses myocardial fibrosis in a transgenic mouse model of human hypertrophic cardiomyopathy.  Circulation.2001;103:789-791.
Fox PR, Liu S-K, Maron BJ. Echocardiographic assessment of spontaneously occurring feline hypertrophic cardiomyopathy.  Circulation.1995;92:2645-2651.
Webb JG, Sasson Z, Rakowski H.  et al.  Apical hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:83-90.
Louie EK, Maron BJ. Apical hypertrophic cardiomyopathy: clinical and two-dimensional echocardiographic assessment.  Ann Intern Med.1987;106:663-670.
Louie EK, Maron BJ. Hypertrophic cardiomyopathy with extreme increase in left ventricular wall thickness.  J Am Coll Cardiol.1986;8:57-65.
Maron BJ, Gross BW, Stark SI. Images in cardiovascular medicine: extreme left ventricular hypertrophy.  Circulation.1995;92:2748.
Spirito P, Bellone P, Harris KM.  et al.  Magnitude of left ventricular hypertrophy predicts the risk of sudden death in hypertrophic cardiomyopathy.  N Engl J Med.2000;342:1778-1785.
Elliott PM, Gimeno Blanes JR.  et al.  Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy.  Lancet.2001;357:420-424.
Maron BJ, Shirani J, Poliac LC.  et al.  Sudden death in young competitive athletes: clinical, demographic and pathological profiles.  JAMA.1996;276:199-204.
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.
Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes.  N Engl J Med.1998;339:364-369.
Spirito P, Maron BJ, Bonow RO.  et al.  Severe functional limitation in patients with hypertrophic cardiomyopathy and only mild localized left ventricular hypertrophy.  J Am Coll Cardiol.1986;8:537-544.
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.
Pelliccia A, Maron BJ, Spataro A.  et al.  The upper limit of physiologic cardiac hypertrophy in highly trained elite athletes.  N Engl J Med.1991;324:295-301.
Maron BJ, Pelliccia A, Spirito P. Cardiac disease in young trained athletes: insights into methods for distinguishing athlete's heart from structural heart disease with particular emphasis on hypertrophic cardiomyopathy.  Circulation.1995;91:1596-1601.
Maron BJ. The electrocardiogram as a diagnostic tool for hypertrophic cardiomyopathy: revisited.  Ann Noninvasive Electrocardiol.2001;6:277-279.
Charron P, Dubourg O, Desnos M.  et al.  Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in a genotyped adult population.  Circulation.1997;96:214-219.
Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy.  N Engl J Med.1986;315:610-614.
Charron P, Dubourg O, Desnos M.  et al.  Clinical features and prognostic implications of familial hypertrophic cardiomyopathy related to the cardiac myosin-binding protein C gene.  Circulation.1998;97:2230-2236.
Hagege AA, Dubourg O, Desnos M.  et al.  Familial hypertrophic cardiomyopathy.  Eur Heart J.1998;19:490-499.
Maron BJ, Nichols III PF, Pickle LW.  et al.  Patterns of inheritance in hypertrophic cardiomyopathy.  Am J Cardiol.1984;53:1087-1094.
Panza JA, Maron BJ. Relation of electrocardiographic abnormalities to evolving left ventricular hypertrophy in hypertrophic cardiomyopathy.  Am J Cardiol.1989;63:1258-1265.
Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;9:1013-1017.
Maron BJ, Spirito P. Implications of left ventricular remodeling in hypertrophic cardiomyopathy.  Am J Cardiol.1998;81:1339-1344.
Nagueh SF, Bachinski LL, Meyer D.  et al.  Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy.  Circulation.2001;104:128-130.
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.
Obeid AI, Maron BJ. Apical hypertrophic cardiomyopathy developing at a relatively advanced age.  Circulation.2001;103:1605.
Shirani J, Maron BJ, Cannon III RO.  et al.  Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation.  Am J Cardiol.1993;72:434-440.
Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and age in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:820-823.
Yamaguchi H, Ishimura T, Nishiyama S.  et al.  Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy).  Am J Cardiol.1979;44:401-412.
Klues HG, Roberts WC, Maron BJ. Morphologic determinants of echocardiographic patterns of mitral valve systolic anterior motion in obstructive hypertrophic cardiomyopathy.  Circulation.1993;87:1570-1579.
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 hypertrophic cardiomyopathy.  Circulation.1983;68:282-293.
Schwammenthal E, Block M, Schwartzkopff B.  et al.  Prediction of the site and severity of obstruction in hypertrophic cardiomyopathy by color flow mapping and continuous wave Doppler echocardiography.  J Am Coll Cardiol.1992;20:964-972.
Shah PM, Taylor RD, Wong M. Abnormal mitral valve coaptation in hypertrophic obstructive cardiomyopathy.  Am J Cardiol.1981;48:258-262.
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.
Pollick C, Rakowski H, Wigle ED. Muscular subaortic stenosis: the quantitative relationship between systolic anterior motion and pressure gradient.  Circulation.1984;69:43-49.
Ferrans VJ, Morrow AG, Roberts WC. Myocardial ultrastructure in idiopathic hypertrophic subaortic stenosis.  Circulation.1972;45:769-792.
Maron BJ, Roberts WC. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy.  Circulation.1979;59:689-706.
St. John Sutton MG, Lie JT, Anderson KR.  et al.  Histopathological specificity of hypertrophic obstructive cardiomyopathy.  Br Heart J.1980;44:433-443.
Varnava AM, Elliott PM, Mahon N.  et al.  Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy.  Am J Cardiol.2001;88:275-279.
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.
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.
Tanaka M, Fujiwara H, Onodera T.  et al.  Quantitative analysis of narrowings of intramyocardial small arteries in normal hearts, hypertensive hearts, and hearts with hypertrophic cardiomyopathy.  Circulation.1987;75:1130-1139.
Cannon III RO, Rosing DR, Maron BJ.  et al.  Myocardial ischemia in patients with hypertrophic cardiomyopathy.  Circulation.1985;71:234-243.
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.
O'Gara PT, Bonow RO, Maron BJ.  et al.  Myocardial perfusion abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1987;76:1214-1223.
Cannon III 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 non-obstructive hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;10:53-62.
Nienaber CA, Gambhir SS, Mody FV.  et al.  Regional myocardial blood flow and glucose utilization in symptomatic patients with hypertrophic cardiomyopathy.  Circulation.1993;87:1580-1590.
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.
Tanaka M, Fujiwara H, Onodera T.  et al.  Quantitative analysis of myocardial fibrosis in normal, hypertensive hearts, and hypertrophic cardiomyopathy.  Br Heart J.1986;55:575-581.
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.
Camici P, Chiriatti G, Lorenzoni R.  et al.  Coronary vasodilation is impaired in both hypertrophied and nonhypertrophied myocardium of patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1991;17:879-886.
Hanrath P, Mathey D, Montz R.  et al.  Myocardial thallium-201 imaging in hypertrophic obstructive cardiomyopathy.  Eur Heart J.1981;2:177-185.
Suzuki Y, Kadota K, Nohara R.  et al.  Recognition of regional hypertrophy in hypertrophic cardiomyopathy using thallium-201 emission computed tomography.  Am J Cardiol.1984;53:1095-1102.
Grover-McKay M, Schwaiger M, Krivokapich J.  et al.  Regional myocardial blood flow and metabolism at rest in mildly symptomatic patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:317-324.
Basso C, Thiene G, Corrado D.  et al.  Hypertrophic cardiomyopathy and sudden death in the young: pathologic evidence of myocardial ischemia.  Hum Pathol.2000;31:988-998.
Takata J, Counihan PJ, Gane JN.  et al.  Regional thallium-201 washout and myocardial hypertrophy in hypertrophic cardiomyopathy and its relation to exertional chest pain.  Am J Cardiol.1993;72:211-217.
Pasternac A, Noble J, Streulens Y.  et al.  Pathophysiology of chest pain in patients with cardiomyopathies and normal coronary arteries.  Circulation.1982;65:778-789.
Elliott PM, Kaski JC, Prasad K.  et al.  Chest pain during daily life in patients with hypertrophic cardiomyopathy.  Eur Heart J.1996;17:1056-1064.
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.
Spirito P, Maron BJ, Chiarella F.  et al.  Diastolic abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1985;72:310-316.
Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and diastolic filling abnormalities in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:808-813.
Shirani J, Pick R, Roberts WC, Maron BJ. 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.
Maron BJ, Shen W-K, 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.
Silka MJ, Kron J, Dunnigan A, Dick II M. Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients.  Circulation.1993;87:800-807.
Elliott PM, Sharma S, Varnava A.  et al.  Survival after cardiac arrest in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1999;33:1596-1601.
Nicod P, Polikar R, Peterson KL. Hypertrophic cardiomyopathy and sudden death.  N Engl J Med.1988;318:1255-1257.
Marian AJ, Yu QT, Workman R.  et al.  Angiotensin-converting enzyme polymorphism in hypertrophic cardiomyopathy and sudden cardiac death.  Lancet.1993;342:1085-1086.
Lechin M, Quiñones MA, Omran A.  et al.  Angiotensin-I converting enzyme genotypes and left ventricular hypertrophy in patients with hypertrophic cardiomyopathy.  Circulation.1995;92:1808-1812.
Grigg LE, Wigle ED, Williams WG.  et al.  Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;20:42-52.
Lewis JF, Maron BJ. Elderly patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:36-45.
Skinner JR, Manzoor A, Hayes AM.  et al.  A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants.  Heart.1997;77:229-233.
Maron BJ, Tajik AJ, Ruttenberg HD.  et al.  Hypertrophic cardiomyopathy in infants.  Circulation.1982;65:7-17.
McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death.  Arch Dis Child.1984;59:971-975.
Fiddler GI, Tajik AJ, Weidman WH.  et al.  Idiopathic hypertrophic subaortic stenosis in the young.  Am J Cardiol.1978;42:793-799.
Panza JA, Maris TJ, Maron BJ. Development and determinants of dynamic obstruction to left ventricular outflow in young patients with hypertrophic cardiomyopathy.  Circulation.1992;85:1398-1405.
Maron BJ, Casey SA, Poliac LC.  et al.  Clinical course of hypertrophic cardiomyopathy in a regional United States cohort.  JAMA.1999;281:650-655.
Lever HM, Karam RF, Currie PJ, Healy BP. Hypertrophic cardiomyopathy in the elderly.  Circulation.1989;79:580-589.
Fay WP, Taliercio CP, Ilstrup DM.  et al.  Natural history of hypertrophic cardiomyopathy in the elderly.  J Am Coll Cardiol.1990;16:821-826.
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.
Lewis JF, Maron BJ. Clinical and morphologic expression of hypertrophic cardiomyopathy in patients ≥ 65 years of age.  Am J Cardiol.1994;73:1105-1111.
McKenna WJ, England D, Doi YL.  et al.  Arrhythmia in hypertrophic cardiomyopathy.  Br Heart J.1981;46:168-172.
Maron BJ, Savage DD, Wolfson JK, Epstein SE. The prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy.  Am J Cardiol.1981;48:252-257.
Maron BJ, Roberts WC, Epstein SE. Sudden death in hypertrophic cardiomyopathy.  Circulation.1982;65:1388-1394.
Shah PM, Adelman AG, Wigle ED.  et al.  The natural (and unnatural) history of hypertrophic obstructive cardiomyopathy.  Circ Res.1974;35:suppl II:179-195.
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.
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.
Spirito P, Chiarella F, Carratino L.  et al.  Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatient population.  N Engl J Med.1989;320:749-755.
Shapiro LM, Zezulka A. Hypertrophic cardiomyopathy: a common disease with a good prognosis: five year experience of a district general hospital.  Br Heart J.1983;50:530-533.
Cannan CR, Reeder GS, Bailey KR, Melton III LJ, Gersh BJ. Natural history of hypertrophic cardiomyopathy.  Circulation.1995;92:2488-2495.
Spirito P, Rapezzi C, Autore C.  et al.  Prognosis in asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia.  Circulation.1994;90:2743-2747.
Kofflard MJ, Waldstein DJ, Vos J, ten Cate FJ. Prognosis in hypertrophic cardiomyopathy.  Am J Cardiol.1993;72:939-943.
Maki S, Ikeda H, Muro A.  et al.  Predictors of sudden cardiac death in hypertrophic cardiomyopathy.  Am J Cardiol.1998;82:774-778.
Niimura H, Patton KK, Maron BJ.  et al.  Sarcomere protein gene mutations in hypertrophic cardiomyopathy of the elderly.  Circulation.2002;105:446-451.
Robinson KC, Frenneaux MP, Stockins B.  et al.  Atrial fibrillation in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:1279-1285.
Olivotto I, Cecchi F, Casey SA.  et al.  Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy.  Circulation.2001;104:2517-2524.
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.
Krikler DM, Davies MJ, Rowland E.  et al.  Sudden death in hypertrophic cardiomyopathy: associated accessory atrioventricular pathways.  Br Heart J.1980;43:245-251.
Stafford WJ, Trohman RG, Bilsker M.  et al.  Cardiac arrest in an adolescent with atrial fibrillation and hypertrophic cardiomyopathy.  J Am Coll Cardiol.1986;7:701-704.
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.
Sadoul N, Prasad K, Elliott PM.  et al.  Prospective diagnostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy.  Circulation.1997;96:2987-2991.
Cecchi F, Maron BJ, Epstein SE. Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest.  J Am Coll Cardiol.1989;13:1283-1288.
Romeo F, Pelliccia F, Cristofani R.  et al.  Hypertrophic cardiomyopathy: is a left ventricular outflow tract gradient a major prognostic determinant?  Eur Heart J.1990;11:233-240.
Yetman AT, McCrindle BW, MacDonald LC.  et al.  Myocardial bridging in children with hypertrophic cardiomyopathy.  N Engl J Med.1998;339:1201-1209.
Anan R, Shono H, Kisanuki A.  et al.  Patients with familial hypertrophic cardiomyopathy caused by a Phe110Ile missense mutation in the cardiac troponin T gene have variable cardiac morphologies and a favorable prognosis.  Circulation.1998;98:391-397.
Watkins H. Sudden death in hypertrophic cardiomyopathy.  N Engl J Med.2000;342:422-424.
Saumarez RC, Slade AK, Grace AA.  et al.  The significance of paced electrocardiogram fractionation in hypertrophic cardiomyopathy.  Circulation.1995;91:2762-2768.
Fananapazir L, Tracy CM, Leon MB.  et al.  Electrophysiologic abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1989;80:1259-1268.
McKenna WJ, Oakley CM, Krikler DM.  et al.  Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia.  Br Heart J.1985;53:412-416.
Ostman-Smith I, Wettrell G, Riesenfeld T. A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose β-adrenoceptor antagonist treatment.  J Am Coll Cardiol.1999;34:1813-1822.
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.  Heart.1998;79:331-336.
Camm AJ, Nisam S. The utilization of the implantable defibrillator.  Eur Heart J.2000;21:1998-2004.
Maron BJ, Isner JM, McKenna WJ. Hypertrophic cardiomyopathy, myocarditis and other myopericardial disease, and mitral valve prolapse. In: 26th Bethesda Conference. Recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities.  J Am Coll Cardiol.1994;24:880-885.
Maron BJ, Spirito P, Green KJ.  et al.  Noninvasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;10:733-742.
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.
Briguori C, Betocchi S, Romano M.  et al.  Exercise capacity in hypertrophic cardiomyopathy depends on left ventricular diastolic function.  Am J Cardiol.1999;84:309-315.
Lele SS, Thomson HL, Seo H.  et al.  Exercise capacity in hypertrophic cardiomyopathy.  Circulation.1995;92:2886-2894.
Frenneaux MP, Porter A, Caforio ALP.  et al.  Determinants of exercise capacity in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:1521-1526.
Chikamori T, Counihan PJ, Doi YL.  et al.  Mechanisms of exercise limitations in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;19:507-512.
Nihoyannopoulos P, Karatasakis G, Frenneaux M.  et al.  Diastolic function in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;19:536-540.
Sharma S, Elliott P, Whyte G.  et al.  Utility of cardiopulmonary exercise in the assessment of clinical determinants of functional capacity in hypertrophic cardiomyopathy.  Am J Cardiol.2000;86:162-168.
Lazzeroni E, Picano E, Morozzi L.  et al.  Dipyridamole-induced ischemia as a prognostic marker of future adverse cardiac events in adult patients with hypertrophic cardiomyopathy.  Circulation.1997;96:4268-4272.
Dilsizian V, Bonow RO, Epstein SE.  et al.  Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1993;22:796-804.
Spicer RL, Rocchini AP, Crowley DC, Rosenthal A. Chronic verapamil therapy in pediatric and young adult patients with hypertrophic cardiomyopathy.  Am J Cardiol.1984;53:1614-1619.
Gistri R, Cecchi F, Choudhury L.  et al.  Effect of verapamil on absolute myocardial blood flow in hypertrophic cardiomyopathy.  Am J Cardiol.1994;74:363-368.
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.
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.
Rosing DR, Kent KM, Maron BJ, Epstein SE. 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.
Epstein SE, Rosing DR. Verapamil: its potential for causing serious complications in patients with hypertrophic cardiomyopathy.  Circulation.1981;64:437-441.
Sherrid M, Delia E, Dwyer E. Oral disopyramide therapy for obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1988;62:1085-1088.
Pollick C. Muscular subaortic stenosis: hemodynamic and clinical improvement after disopyramide.  N Engl J Med.1982;307:997-999.
Sherrid MV, Pearle G, Gunsburg DZ. Mechanism of benefit of negative inotropes in obstructive hypertrophic cardiomyopathy.  Circulation.1998;97:41-47.
Hecht GM, Klues HG, Roberts WC, Maron BJ. Coexistence of sudden cardiac death and end-stage heart failure in familial hypertrophic cardiomyopathy.  J Am Coll Cardiol.1993;22:489-497.
Spirito P, Rapezzi C, Bellone P.  et al.  Infective endocarditis in hypertrophic cardiomyopathy.  Circulation.1999;99:2132-2137.
Morrow AG, Reitz BA, Epstein SE.  et al.  Operative treatment in hypertrophic subaortic stenosiss.  Circulation.1975;52:88-102.
Williams WG, Wigle ED, Rakowski H.  et al.  Results of surgery for hypertrophic obstructive cardiomyopathy.  Circulation.1987;76(5 Pt 2):V104-V108.
McCully RB, Nishimura RA, Tajik AJ.  et al.  Extent of clinical improvement after surgical treatment of hypertrophic obstructive cardiomyopathy.  Circulation.1996;94:467-471.
Robbins RC, Stinson EB. Long-term results of left ventricular myotomy and myectomy for obstructive hypertrophic cardiomyopathy.  J Thorac Cardiovasc Surg.1996;111:586-594.
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(9 Suppl):II122-II127.
Krajcer Z, Leachman RD, Cooley DA, Coronado R. Septal myotomy-myectomy versus mitral valve replacement in hypertrophic cardiomyopathy.  Circulation.1989;80(3 Pt I):I57-I64.
McIntosh CL, Maron BJ. Current operative treatment of obstructive hypertrophic cardiomyopathy.  Circulation.1988;78:487-495.
Cohn LH, Trehan H, Collin Jr JJ. Long-term follow-up of patients undergoing myotomy-myectomy for obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1992;70:657-660.
ten Berg JM, Suttorp MJ, Knaepen PJ.  et al.  Hypertrophic obstructive cardiomyopathy: initial results and long-term follow-up after Morrow septal myectomy.  Circulation.1994;90:1781-1785.
Heric B, Lytle BW, Miller DP.  et al.  Surgical management of hypertrophic obstructive cardiomyopathy.  J Thorac Cardiovasc Surg.1995;110:195-208.
Theodoro DA, Danielson GK, Feldt RH, Anderson BJ. Hypertrophic cardiomyopathy in pediatric patients: results of surgical treatment.  J Thorac Cardiovasc Surg.1996;112:1589-1599.
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;98:2505-2508.
Ommen SR, Nishimura RA, Squires RW.  et al.  Comparison of dual-chamber pacing versus septal myectomy for the treatment of patients with hypertrophic obstructive cardiomyopathy.  J Am Coll Cardiol.1999;34:191-196.
Brunner-La Schonbeck MH, Rocca HP, Vogt PR.  et al.  Long-term follow-up in hypertrophic obstructive cardiomyopathy after septal myectomy.  Ann Thorac Surg.1998;65:1207-1214.
Yu EHC, Omran AS, Wigle ED.  et al.  Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy.  J Am Coll Cardiol.2000;36:2219-2225.
Schulte HD, Bircks WH, Loesse B.  et al.  Prognosis of patients with hypertrophic obstructive cardiomyopathy after transaortic myectomy.  J Thorac Cardiovasc Surg.1993;106:709-717.
Petrone RK, Klues HG, Panza JA.  et al.  Significance of the occurrence of mitral valve prolapse in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;20:55-61.
Cannon III RO, McIntosh CL, Schenke WH.  et al.  Effect of surgical reduction of left ventricular outflow obstruction on hemodynamics, coronary flow, and myocardial metabolism in hypertrophic cardiomyopathy.  Circulation.1989;79:766-775.
Kizilbash AM, Heinle SK, Grayburn PA. Spontaneous variability of left ventricular outflow tract gradient in hypertrophic obstructive cardiomyopathy.  Circulation.1998;97:461-466.
Klues HG, Leuner C, Kuhn H. Hypertrophic obstructive cardiomyopathy: no increase of the gradient during exercise.  J Am Coll Cardiol.1991;19:527-533.
Gilligan DM, Chan WL, Ang EL, Oakley CM. Effects of a meal on hemodynamic function at rest and during exercise in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1991;18:429-436.
Murgo J, Alter BR, Dorethy JF.  et al.  Dynamics of left ventricular ejection in obstructive and nonobstructive hypertrophic cardiomyopathy.  J Clin Invest.1980;66:1369-1382.
Maron BJ. Role of alcohol septal ablation in treatment of obstructive hypertrophic cardiomyopathy.  Lancet.2000;355:425-426.
Bryce M, Spielman SR, Greenspan AM, Kotler MN. Evolving indications for permanent pacemakers.  Ann Intern Med.2001;134:1130-1141.
Fananapazir L, Epstein ND, Curiel RV.  et al.  Long-term results of dual-chamber (DDD) pacing in obstructive hypertrophic cardiomyopathy.  Circulation.1994;90:2731-2742.
Slade AKB, Sadoul N, Shapiro L.  et al.  DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience.  Heart.1996;75:44-49.
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.  Circulation.1999;99:2927-2933.
Nishimura RA, Trusty JM, Hayes DL.  et al.  Dual-chamber pacing for hypertrophic cardiomyopathy.  J Am Coll Cardiol.1997;29:435-441.
Kappenberger L, Linde C, Daubert C.  et al.  Pacing in hypertrophic obstructive cardiomyopathy.  Eur Heart J.1997;18:1249-1256.
Linde C, Gadler F, Kappenberger L, Ryden L. Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1999;83:903-907.
Gadler F, Linde C, Daubert C.  et al.  Significant improvement of quality of life following atrioventricular synchronous pacing in patients with hypertrophic cardiomyopathy: data from 1 year of follow-up.  Eur Heart J.1999;20:1044-1050.
Knight C, Kurbaan AS, Seggewiss H.  et al.  Nonsurgical septal reduction for hypertrophic obstructive cardiomyopathy.  Circulation.1997;95:2075-2081.
Faber L, Meissner A, Ziemssen P.  et al.  Percutaneous transluminal septal myocardial ablation for hypertrophic obstructive cardiomyopathy.  Heart.2000;83:326-331.
Lakkis NM, Nagueh SF, Kleiman NS.  et al.  Echocardiography-guided ethanol septal reduction for hypertrophic obstructive cardiomyopathy.  Circulation.1998;98:1750-1755.
Geitzen FH, Leuner ChJ, Raute-Kreinsen U.  et al.  Acute and long-term results after transcoronary ablation of septal hypertrophy (TASH).  Eur Heart J.1999;20:1342-1354.
Mazur W, Nagueh SF, Lakkis NM.  et al.  Regression of left ventricular hypertrophy after nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy.  Circulation.2001;103:1492-1496.
Nagueh SF, Ommen SR, Lakkis NM.  et al.  Comparison of ethanol septal reduction therapy with surgical myectomy for the treatment of hypertrophic cardiomyopathy.  J Am Coll Cardiol.2001;38:1701-1706.
Wigle ED, Schwartz L, Woo A, Rakowski H. To ablate or operate? that is the question.  J Am Coll Cardiol.2001;15:1707-1710.
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.

Figures

Tables

References

Teare D. Asymmetrical hypertrophy of the heart in young adults.  Br Heart J.1958;20:1-18.
Braunwald E, Lambrew CT, Rockoff D.  et al.  Idiopathic hypertrophic subaortic stenosis, I: a description of the disease based upon an analysis of 64 patients.  Circulation.1964;30(suppl IV):3-217.
Maron BJ. Hypertrophic cardiomyopathy.  Lancet.1997;350:127-133.
Maron BJ, Bonow RO, Cannon III RO, Leon MB, Epstein SE. Hypertrophic cardiomyopathy: interrelation of clinical manifestations, pathophysiology, and therapy.  N Engl J Med.1987;316:780-789, 844-852.
Frank S, Braunwald E. Idiopathic hypertrophic subaortic stenosis: clinical analysis of 126 patients with emphasis on the natural history.  Circulation.1968;37:759-788.
Wigle ED, Sasson Z, Henderson MA.  et al.  Hypertrophic cardiomyopathy: the importance of the site and extent of hypertrophy.  Prog Cardiovasc Dis.1985;28:1-83.
Spirito P, Seidman CE, McKenna WJ, Maron BJ. Management of hypertrophic cardiomyopathy.  N Engl J Med.1997;336:775-785.
Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy: clinical spectrum and treatment.  Circulation.1995;92:1680-1692.
Maron BJ, Moller JH, Seidman CE.  et al.  Impact of laboratory molecular diagnosis on contemporary diagnostic criteria for genetically transmitted cardiovascular diseases: hypertrophic cardiomyopathy, long-QT syndrme, and Marfan syndrome. A statement for healthcare professions from the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association.  Circulation.1998;98:1460-1471.
Louie EK, Edwards LC. Hypertrophic cardiomyopathy.  Prog Cardiovasc Dis.1994;36:275-308.
Marian AJ, Roberts R. Recent advances in the molecular genetics of hypertrophic cardiomyopathy.  Circulation.1995;92:1336-1347.
Schwartz K, Carrier L, Guicheney P, Komajda M. Molecular basis of familial cardiomyopathies.  Circulation.1995;91:532-540.
Niimura H, Bachinski LL, Sangwatanaroj S.  et al.  Mutations in the gene for human cardiac myosin-binding protein C and late-onset familial hypertrophic cardiomyopathy.  N Engl J Med.1998;338:1248-1257.
Ciro E, Nichols III PF, Maron BJ. Heterogeneous morphologic expression of genetically transmitted hypertrophic cardiomyopathy.  Circulation.1983;67:1227-1233.
Maron BJ, Gottdiener JS, Epstein SE. Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy.  Am J Cardiol.1981;48:418-428.
Shapiro LM, McKenna WJ. Distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1983;2:437-444.
Klues HG, Roberts WC, Maron BJ. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in hypertrophic cardiomyopathy.  Circulation.1991;84:1188-1197.
Klues HG, Maron BJ, Dollar AL.  et al.  Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy.  Circulation.1992;85:1651-1660.
Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1995;26:1699-1708.
Maron BJ, Gardin JM, Flack JM.  et al.  Prevalence of hypertrophic cardiomyopathy in a general population of young adults.  Circulation.1995;92:785-789.
Maron BJ, Peterson EE, Maron MS, Peterson JE. Prevalence of hypertrophic cardiomyopathy in an outpatient population referred for echocardiographic study.  Am J Cardiol.1994;73:577-580.
Maron BJ, Epstein SE. Hypertrophic cardiomyopathy: a discussion of nomenclature.  Am J Cardiol.1979;43:1242-1244.
Watkins H, McKenna WJ, Thierfelder L.  et al.  The role of cardiac troponin T and α-tropomyosin mutations in hypertrophic cardiomyopathy.  N Engl J Med.1995;332:1058-1064.
Moolman JC, Corfield VA, Posen B.  et al.  Sudden death due to troponin T mutations.  J Am Coll Cardiol.1997;29:549-555.
Maron BJ, Niimura H, Casey SA.  et al.  Development of left ventricular hypertrophy in adults with hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C mutations.  J Am Coll Cardiol.2001;38:315-321.
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.
Anan R, Greve G, Thierfelder L.  et al.  Prognostic implications of novel β-cardiac myosin heavy chain gene mutations that cause familial hypertrophic cardiomyopathy.  J Clin Invest.1994;93:280-285.
Marian AJ. Pathogenesis of diverse clinical and pathological phenotypes in hypertrophic cardiomyopathy.  Lancet.2000;355:58-60.
Rosenzweig A, Watkins H, Hwang DS.  et al.  Preclinical diagnosis of familial hypertrophic cardiomyopathy by genetic analysis of blood lymphocytes.  N Engl J Med.1991;325:1753-1760.
Gollob MH, Green MS, Tang AS.  et al.  Identification of a gene responsible for familial Wolff-Parkinson-White syndrome.  N Engl J Med.2001;344:1823-1831.
Arad M, Benson DW, Perez-Atayde Not Available.  et al.  Constitutively active AMP kinase mutations cause glycogen storage disease mimicking hypertrophic cardiomyopathy.  J Clin Invest.2002;109:357-362.
Geisterfer-Lowrance AA, Christe M, Conner DA.  et al.  A mouse model of familial hypertrophic cardiomyopathy.  Science.1996;272:731-734.
Marian AJ, Wu Y, Lim DS.  et al.  A transgenic rabbit model for human hypertrophic cardiomyopathy.  J Clin Invest.1999;104:1683-1692.
Yang Q, Sanbe A, Osinska H, Hewett TE, Klevitsky R, Robbins J. A mouse model of myosin binding protein C human familial hypertrophic cardiomyopathy.  J Clin Invest.1998;102:1292-1300.
Lim DS, Lutucuta S, Bachireddy P.  et al.  Angiotensin II blockade reverses myocardial fibrosis in a transgenic mouse model of human hypertrophic cardiomyopathy.  Circulation.2001;103:789-791.
Fox PR, Liu S-K, Maron BJ. Echocardiographic assessment of spontaneously occurring feline hypertrophic cardiomyopathy.  Circulation.1995;92:2645-2651.
Webb JG, Sasson Z, Rakowski H.  et al.  Apical hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:83-90.
Louie EK, Maron BJ. Apical hypertrophic cardiomyopathy: clinical and two-dimensional echocardiographic assessment.  Ann Intern Med.1987;106:663-670.
Louie EK, Maron BJ. Hypertrophic cardiomyopathy with extreme increase in left ventricular wall thickness.  J Am Coll Cardiol.1986;8:57-65.
Maron BJ, Gross BW, Stark SI. Images in cardiovascular medicine: extreme left ventricular hypertrophy.  Circulation.1995;92:2748.
Spirito P, Bellone P, Harris KM.  et al.  Magnitude of left ventricular hypertrophy predicts the risk of sudden death in hypertrophic cardiomyopathy.  N Engl J Med.2000;342:1778-1785.
Elliott PM, Gimeno Blanes JR.  et al.  Relation between severity of left-ventricular hypertrophy and prognosis in patients with hypertrophic cardiomyopathy.  Lancet.2001;357:420-424.
Maron BJ, Shirani J, Poliac LC.  et al.  Sudden death in young competitive athletes: clinical, demographic and pathological profiles.  JAMA.1996;276:199-204.
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.
Corrado D, Basso C, Schiavon M, Thiene G. Screening for hypertrophic cardiomyopathy in young athletes.  N Engl J Med.1998;339:364-369.
Spirito P, Maron BJ, Bonow RO.  et al.  Severe functional limitation in patients with hypertrophic cardiomyopathy and only mild localized left ventricular hypertrophy.  J Am Coll Cardiol.1986;8:537-544.
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.
Pelliccia A, Maron BJ, Spataro A.  et al.  The upper limit of physiologic cardiac hypertrophy in highly trained elite athletes.  N Engl J Med.1991;324:295-301.
Maron BJ, Pelliccia A, Spirito P. Cardiac disease in young trained athletes: insights into methods for distinguishing athlete's heart from structural heart disease with particular emphasis on hypertrophic cardiomyopathy.  Circulation.1995;91:1596-1601.
Maron BJ. The electrocardiogram as a diagnostic tool for hypertrophic cardiomyopathy: revisited.  Ann Noninvasive Electrocardiol.2001;6:277-279.
Charron P, Dubourg O, Desnos M.  et al.  Diagnostic value of electrocardiography and echocardiography for familial hypertrophic cardiomyopathy in a genotyped adult population.  Circulation.1997;96:214-219.
Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy.  N Engl J Med.1986;315:610-614.
Charron P, Dubourg O, Desnos M.  et al.  Clinical features and prognostic implications of familial hypertrophic cardiomyopathy related to the cardiac myosin-binding protein C gene.  Circulation.1998;97:2230-2236.
Hagege AA, Dubourg O, Desnos M.  et al.  Familial hypertrophic cardiomyopathy.  Eur Heart J.1998;19:490-499.
Maron BJ, Nichols III PF, Pickle LW.  et al.  Patterns of inheritance in hypertrophic cardiomyopathy.  Am J Cardiol.1984;53:1087-1094.
Panza JA, Maron BJ. Relation of electrocardiographic abnormalities to evolving left ventricular hypertrophy in hypertrophic cardiomyopathy.  Am J Cardiol.1989;63:1258-1265.
Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;9:1013-1017.
Maron BJ, Spirito P. Implications of left ventricular remodeling in hypertrophic cardiomyopathy.  Am J Cardiol.1998;81:1339-1344.
Nagueh SF, Bachinski LL, Meyer D.  et al.  Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy.  Circulation.2001;104:128-130.
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.
Obeid AI, Maron BJ. Apical hypertrophic cardiomyopathy developing at a relatively advanced age.  Circulation.2001;103:1605.
Shirani J, Maron BJ, Cannon III RO.  et al.  Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation.  Am J Cardiol.1993;72:434-440.
Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and age in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:820-823.
Yamaguchi H, Ishimura T, Nishiyama S.  et al.  Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy).  Am J Cardiol.1979;44:401-412.
Klues HG, Roberts WC, Maron BJ. Morphologic determinants of echocardiographic patterns of mitral valve systolic anterior motion in obstructive hypertrophic cardiomyopathy.  Circulation.1993;87:1570-1579.
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 hypertrophic cardiomyopathy.  Circulation.1983;68:282-293.
Schwammenthal E, Block M, Schwartzkopff B.  et al.  Prediction of the site and severity of obstruction in hypertrophic cardiomyopathy by color flow mapping and continuous wave Doppler echocardiography.  J Am Coll Cardiol.1992;20:964-972.
Shah PM, Taylor RD, Wong M. Abnormal mitral valve coaptation in hypertrophic obstructive cardiomyopathy.  Am J Cardiol.1981;48:258-262.
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.
Pollick C, Rakowski H, Wigle ED. Muscular subaortic stenosis: the quantitative relationship between systolic anterior motion and pressure gradient.  Circulation.1984;69:43-49.
Ferrans VJ, Morrow AG, Roberts WC. Myocardial ultrastructure in idiopathic hypertrophic subaortic stenosis.  Circulation.1972;45:769-792.
Maron BJ, Roberts WC. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy.  Circulation.1979;59:689-706.
St. John Sutton MG, Lie JT, Anderson KR.  et al.  Histopathological specificity of hypertrophic obstructive cardiomyopathy.  Br Heart J.1980;44:433-443.
Varnava AM, Elliott PM, Mahon N.  et al.  Relation between myocyte disarray and outcome in hypertrophic cardiomyopathy.  Am J Cardiol.2001;88:275-279.
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.
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.
Tanaka M, Fujiwara H, Onodera T.  et al.  Quantitative analysis of narrowings of intramyocardial small arteries in normal hearts, hypertensive hearts, and hearts with hypertrophic cardiomyopathy.  Circulation.1987;75:1130-1139.
Cannon III RO, Rosing DR, Maron BJ.  et al.  Myocardial ischemia in patients with hypertrophic cardiomyopathy.  Circulation.1985;71:234-243.
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.
O'Gara PT, Bonow RO, Maron BJ.  et al.  Myocardial perfusion abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1987;76:1214-1223.
Cannon III 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 non-obstructive hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;10:53-62.
Nienaber CA, Gambhir SS, Mody FV.  et al.  Regional myocardial blood flow and glucose utilization in symptomatic patients with hypertrophic cardiomyopathy.  Circulation.1993;87:1580-1590.
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.
Tanaka M, Fujiwara H, Onodera T.  et al.  Quantitative analysis of myocardial fibrosis in normal, hypertensive hearts, and hypertrophic cardiomyopathy.  Br Heart J.1986;55:575-581.
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.
Camici P, Chiriatti G, Lorenzoni R.  et al.  Coronary vasodilation is impaired in both hypertrophied and nonhypertrophied myocardium of patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1991;17:879-886.
Hanrath P, Mathey D, Montz R.  et al.  Myocardial thallium-201 imaging in hypertrophic obstructive cardiomyopathy.  Eur Heart J.1981;2:177-185.
Suzuki Y, Kadota K, Nohara R.  et al.  Recognition of regional hypertrophy in hypertrophic cardiomyopathy using thallium-201 emission computed tomography.  Am J Cardiol.1984;53:1095-1102.
Grover-McKay M, Schwaiger M, Krivokapich J.  et al.  Regional myocardial blood flow and metabolism at rest in mildly symptomatic patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:317-324.
Basso C, Thiene G, Corrado D.  et al.  Hypertrophic cardiomyopathy and sudden death in the young: pathologic evidence of myocardial ischemia.  Hum Pathol.2000;31:988-998.
Takata J, Counihan PJ, Gane JN.  et al.  Regional thallium-201 washout and myocardial hypertrophy in hypertrophic cardiomyopathy and its relation to exertional chest pain.  Am J Cardiol.1993;72:211-217.
Pasternac A, Noble J, Streulens Y.  et al.  Pathophysiology of chest pain in patients with cardiomyopathies and normal coronary arteries.  Circulation.1982;65:778-789.
Elliott PM, Kaski JC, Prasad K.  et al.  Chest pain during daily life in patients with hypertrophic cardiomyopathy.  Eur Heart J.1996;17:1056-1064.
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.
Spirito P, Maron BJ, Chiarella F.  et al.  Diastolic abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1985;72:310-316.
Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and diastolic filling abnormalities in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:808-813.
Shirani J, Pick R, Roberts WC, Maron BJ. 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.
Maron BJ, Shen W-K, 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.
Silka MJ, Kron J, Dunnigan A, Dick II M. Sudden cardiac death and the use of implantable cardioverter-defibrillators in pediatric patients.  Circulation.1993;87:800-807.
Elliott PM, Sharma S, Varnava A.  et al.  Survival after cardiac arrest in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1999;33:1596-1601.
Nicod P, Polikar R, Peterson KL. Hypertrophic cardiomyopathy and sudden death.  N Engl J Med.1988;318:1255-1257.
Marian AJ, Yu QT, Workman R.  et al.  Angiotensin-converting enzyme polymorphism in hypertrophic cardiomyopathy and sudden cardiac death.  Lancet.1993;342:1085-1086.
Lechin M, Quiñones MA, Omran A.  et al.  Angiotensin-I converting enzyme genotypes and left ventricular hypertrophy in patients with hypertrophic cardiomyopathy.  Circulation.1995;92:1808-1812.
Grigg LE, Wigle ED, Williams WG.  et al.  Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;20:42-52.
Lewis JF, Maron BJ. Elderly patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:36-45.
Skinner JR, Manzoor A, Hayes AM.  et al.  A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants.  Heart.1997;77:229-233.
Maron BJ, Tajik AJ, Ruttenberg HD.  et al.  Hypertrophic cardiomyopathy in infants.  Circulation.1982;65:7-17.
McKenna WJ, Deanfield JE. Hypertrophic cardiomyopathy: an important cause of sudden death.  Arch Dis Child.1984;59:971-975.
Fiddler GI, Tajik AJ, Weidman WH.  et al.  Idiopathic hypertrophic subaortic stenosis in the young.  Am J Cardiol.1978;42:793-799.
Panza JA, Maris TJ, Maron BJ. Development and determinants of dynamic obstruction to left ventricular outflow in young patients with hypertrophic cardiomyopathy.  Circulation.1992;85:1398-1405.
Maron BJ, Casey SA, Poliac LC.  et al.  Clinical course of hypertrophic cardiomyopathy in a regional United States cohort.  JAMA.1999;281:650-655.
Lever HM, Karam RF, Currie PJ, Healy BP. Hypertrophic cardiomyopathy in the elderly.  Circulation.1989;79:580-589.
Fay WP, Taliercio CP, Ilstrup DM.  et al.  Natural history of hypertrophic cardiomyopathy in the elderly.  J Am Coll Cardiol.1990;16:821-826.
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.
Lewis JF, Maron BJ. Clinical and morphologic expression of hypertrophic cardiomyopathy in patients ≥ 65 years of age.  Am J Cardiol.1994;73:1105-1111.
McKenna WJ, England D, Doi YL.  et al.  Arrhythmia in hypertrophic cardiomyopathy.  Br Heart J.1981;46:168-172.
Maron BJ, Savage DD, Wolfson JK, Epstein SE. The prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy.  Am J Cardiol.1981;48:252-257.
Maron BJ, Roberts WC, Epstein SE. Sudden death in hypertrophic cardiomyopathy.  Circulation.1982;65:1388-1394.
Shah PM, Adelman AG, Wigle ED.  et al.  The natural (and unnatural) history of hypertrophic obstructive cardiomyopathy.  Circ Res.1974;35:suppl II:179-195.
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.
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.
Spirito P, Chiarella F, Carratino L.  et al.  Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatient population.  N Engl J Med.1989;320:749-755.
Shapiro LM, Zezulka A. Hypertrophic cardiomyopathy: a common disease with a good prognosis: five year experience of a district general hospital.  Br Heart J.1983;50:530-533.
Cannan CR, Reeder GS, Bailey KR, Melton III LJ, Gersh BJ. Natural history of hypertrophic cardiomyopathy.  Circulation.1995;92:2488-2495.
Spirito P, Rapezzi C, Autore C.  et al.  Prognosis in asymptomatic patients with hypertrophic cardiomyopathy and nonsustained ventricular tachycardia.  Circulation.1994;90:2743-2747.
Kofflard MJ, Waldstein DJ, Vos J, ten Cate FJ. Prognosis in hypertrophic cardiomyopathy.  Am J Cardiol.1993;72:939-943.
Maki S, Ikeda H, Muro A.  et al.  Predictors of sudden cardiac death in hypertrophic cardiomyopathy.  Am J Cardiol.1998;82:774-778.
Niimura H, Patton KK, Maron BJ.  et al.  Sarcomere protein gene mutations in hypertrophic cardiomyopathy of the elderly.  Circulation.2002;105:446-451.
Robinson KC, Frenneaux MP, Stockins B.  et al.  Atrial fibrillation in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1990;15:1279-1285.
Olivotto I, Cecchi F, Casey SA.  et al.  Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy.  Circulation.2001;104:2517-2524.
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.
Krikler DM, Davies MJ, Rowland E.  et al.  Sudden death in hypertrophic cardiomyopathy: associated accessory atrioventricular pathways.  Br Heart J.1980;43:245-251.
Stafford WJ, Trohman RG, Bilsker M.  et al.  Cardiac arrest in an adolescent with atrial fibrillation and hypertrophic cardiomyopathy.  J Am Coll Cardiol.1986;7:701-704.
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.
Sadoul N, Prasad K, Elliott PM.  et al.  Prospective diagnostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy.  Circulation.1997;96:2987-2991.
Cecchi F, Maron BJ, Epstein SE. Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest.  J Am Coll Cardiol.1989;13:1283-1288.
Romeo F, Pelliccia F, Cristofani R.  et al.  Hypertrophic cardiomyopathy: is a left ventricular outflow tract gradient a major prognostic determinant?  Eur Heart J.1990;11:233-240.
Yetman AT, McCrindle BW, MacDonald LC.  et al.  Myocardial bridging in children with hypertrophic cardiomyopathy.  N Engl J Med.1998;339:1201-1209.
Anan R, Shono H, Kisanuki A.  et al.  Patients with familial hypertrophic cardiomyopathy caused by a Phe110Ile missense mutation in the cardiac troponin T gene have variable cardiac morphologies and a favorable prognosis.  Circulation.1998;98:391-397.
Watkins H. Sudden death in hypertrophic cardiomyopathy.  N Engl J Med.2000;342:422-424.
Saumarez RC, Slade AK, Grace AA.  et al.  The significance of paced electrocardiogram fractionation in hypertrophic cardiomyopathy.  Circulation.1995;91:2762-2768.
Fananapazir L, Tracy CM, Leon MB.  et al.  Electrophysiologic abnormalities in patients with hypertrophic cardiomyopathy.  Circulation.1989;80:1259-1268.
McKenna WJ, Oakley CM, Krikler DM.  et al.  Improved survival with amiodarone in patients with hypertrophic cardiomyopathy and ventricular tachycardia.  Br Heart J.1985;53:412-416.
Ostman-Smith I, Wettrell G, Riesenfeld T. A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose β-adrenoceptor antagonist treatment.  J Am Coll Cardiol.1999;34:1813-1822.
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.  Heart.1998;79:331-336.
Camm AJ, Nisam S. The utilization of the implantable defibrillator.  Eur Heart J.2000;21:1998-2004.
Maron BJ, Isner JM, McKenna WJ. Hypertrophic cardiomyopathy, myocarditis and other myopericardial disease, and mitral valve prolapse. In: 26th Bethesda Conference. Recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities.  J Am Coll Cardiol.1994;24:880-885.
Maron BJ, Spirito P, Green KJ.  et al.  Noninvasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1987;10:733-742.
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.
Briguori C, Betocchi S, Romano M.  et al.  Exercise capacity in hypertrophic cardiomyopathy depends on left ventricular diastolic function.  Am J Cardiol.1999;84:309-315.
Lele SS, Thomson HL, Seo H.  et al.  Exercise capacity in hypertrophic cardiomyopathy.  Circulation.1995;92:2886-2894.
Frenneaux MP, Porter A, Caforio ALP.  et al.  Determinants of exercise capacity in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1989;13:1521-1526.
Chikamori T, Counihan PJ, Doi YL.  et al.  Mechanisms of exercise limitations in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;19:507-512.
Nihoyannopoulos P, Karatasakis G, Frenneaux M.  et al.  Diastolic function in hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;19:536-540.
Sharma S, Elliott P, Whyte G.  et al.  Utility of cardiopulmonary exercise in the assessment of clinical determinants of functional capacity in hypertrophic cardiomyopathy.  Am J Cardiol.2000;86:162-168.
Lazzeroni E, Picano E, Morozzi L.  et al.  Dipyridamole-induced ischemia as a prognostic marker of future adverse cardiac events in adult patients with hypertrophic cardiomyopathy.  Circulation.1997;96:4268-4272.
Dilsizian V, Bonow RO, Epstein SE.  et al.  Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1993;22:796-804.
Spicer RL, Rocchini AP, Crowley DC, Rosenthal A. Chronic verapamil therapy in pediatric and young adult patients with hypertrophic cardiomyopathy.  Am J Cardiol.1984;53:1614-1619.
Gistri R, Cecchi F, Choudhury L.  et al.  Effect of verapamil on absolute myocardial blood flow in hypertrophic cardiomyopathy.  Am J Cardiol.1994;74:363-368.
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.
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.
Rosing DR, Kent KM, Maron BJ, Epstein SE. 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.
Epstein SE, Rosing DR. Verapamil: its potential for causing serious complications in patients with hypertrophic cardiomyopathy.  Circulation.1981;64:437-441.
Sherrid M, Delia E, Dwyer E. Oral disopyramide therapy for obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1988;62:1085-1088.
Pollick C. Muscular subaortic stenosis: hemodynamic and clinical improvement after disopyramide.  N Engl J Med.1982;307:997-999.
Sherrid MV, Pearle G, Gunsburg DZ. Mechanism of benefit of negative inotropes in obstructive hypertrophic cardiomyopathy.  Circulation.1998;97:41-47.
Hecht GM, Klues HG, Roberts WC, Maron BJ. Coexistence of sudden cardiac death and end-stage heart failure in familial hypertrophic cardiomyopathy.  J Am Coll Cardiol.1993;22:489-497.
Spirito P, Rapezzi C, Bellone P.  et al.  Infective endocarditis in hypertrophic cardiomyopathy.  Circulation.1999;99:2132-2137.
Morrow AG, Reitz BA, Epstein SE.  et al.  Operative treatment in hypertrophic subaortic stenosiss.  Circulation.1975;52:88-102.
Williams WG, Wigle ED, Rakowski H.  et al.  Results of surgery for hypertrophic obstructive cardiomyopathy.  Circulation.1987;76(5 Pt 2):V104-V108.
McCully RB, Nishimura RA, Tajik AJ.  et al.  Extent of clinical improvement after surgical treatment of hypertrophic obstructive cardiomyopathy.  Circulation.1996;94:467-471.
Robbins RC, Stinson EB. Long-term results of left ventricular myotomy and myectomy for obstructive hypertrophic cardiomyopathy.  J Thorac Cardiovasc Surg.1996;111:586-594.
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(9 Suppl):II122-II127.
Krajcer Z, Leachman RD, Cooley DA, Coronado R. Septal myotomy-myectomy versus mitral valve replacement in hypertrophic cardiomyopathy.  Circulation.1989;80(3 Pt I):I57-I64.
McIntosh CL, Maron BJ. Current operative treatment of obstructive hypertrophic cardiomyopathy.  Circulation.1988;78:487-495.
Cohn LH, Trehan H, Collin Jr JJ. Long-term follow-up of patients undergoing myotomy-myectomy for obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1992;70:657-660.
ten Berg JM, Suttorp MJ, Knaepen PJ.  et al.  Hypertrophic obstructive cardiomyopathy: initial results and long-term follow-up after Morrow septal myectomy.  Circulation.1994;90:1781-1785.
Heric B, Lytle BW, Miller DP.  et al.  Surgical management of hypertrophic obstructive cardiomyopathy.  J Thorac Cardiovasc Surg.1995;110:195-208.
Theodoro DA, Danielson GK, Feldt RH, Anderson BJ. Hypertrophic cardiomyopathy in pediatric patients: results of surgical treatment.  J Thorac Cardiovasc Surg.1996;112:1589-1599.
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;98:2505-2508.
Ommen SR, Nishimura RA, Squires RW.  et al.  Comparison of dual-chamber pacing versus septal myectomy for the treatment of patients with hypertrophic obstructive cardiomyopathy.  J Am Coll Cardiol.1999;34:191-196.
Brunner-La Schonbeck MH, Rocca HP, Vogt PR.  et al.  Long-term follow-up in hypertrophic obstructive cardiomyopathy after septal myectomy.  Ann Thorac Surg.1998;65:1207-1214.
Yu EHC, Omran AS, Wigle ED.  et al.  Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy.  J Am Coll Cardiol.2000;36:2219-2225.
Schulte HD, Bircks WH, Loesse B.  et al.  Prognosis of patients with hypertrophic obstructive cardiomyopathy after transaortic myectomy.  J Thorac Cardiovasc Surg.1993;106:709-717.
Petrone RK, Klues HG, Panza JA.  et al.  Significance of the occurrence of mitral valve prolapse in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1992;20:55-61.
Cannon III RO, McIntosh CL, Schenke WH.  et al.  Effect of surgical reduction of left ventricular outflow obstruction on hemodynamics, coronary flow, and myocardial metabolism in hypertrophic cardiomyopathy.  Circulation.1989;79:766-775.
Kizilbash AM, Heinle SK, Grayburn PA. Spontaneous variability of left ventricular outflow tract gradient in hypertrophic obstructive cardiomyopathy.  Circulation.1998;97:461-466.
Klues HG, Leuner C, Kuhn H. Hypertrophic obstructive cardiomyopathy: no increase of the gradient during exercise.  J Am Coll Cardiol.1991;19:527-533.
Gilligan DM, Chan WL, Ang EL, Oakley CM. Effects of a meal on hemodynamic function at rest and during exercise in patients with hypertrophic cardiomyopathy.  J Am Coll Cardiol.1991;18:429-436.
Murgo J, Alter BR, Dorethy JF.  et al.  Dynamics of left ventricular ejection in obstructive and nonobstructive hypertrophic cardiomyopathy.  J Clin Invest.1980;66:1369-1382.
Maron BJ. Role of alcohol septal ablation in treatment of obstructive hypertrophic cardiomyopathy.  Lancet.2000;355:425-426.
Bryce M, Spielman SR, Greenspan AM, Kotler MN. Evolving indications for permanent pacemakers.  Ann Intern Med.2001;134:1130-1141.
Fananapazir L, Epstein ND, Curiel RV.  et al.  Long-term results of dual-chamber (DDD) pacing in obstructive hypertrophic cardiomyopathy.  Circulation.1994;90:2731-2742.
Slade AKB, Sadoul N, Shapiro L.  et al.  DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience.  Heart.1996;75:44-49.
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.  Circulation.1999;99:2927-2933.
Nishimura RA, Trusty JM, Hayes DL.  et al.  Dual-chamber pacing for hypertrophic cardiomyopathy.  J Am Coll Cardiol.1997;29:435-441.
Kappenberger L, Linde C, Daubert C.  et al.  Pacing in hypertrophic obstructive cardiomyopathy.  Eur Heart J.1997;18:1249-1256.
Linde C, Gadler F, Kappenberger L, Ryden L. Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy.  Am J Cardiol.1999;83:903-907.
Gadler F, Linde C, Daubert C.  et al.  Significant improvement of quality of life following atrioventricular synchronous pacing in patients with hypertrophic cardiomyopathy: data from 1 year of follow-up.  Eur Heart J.1999;20:1044-1050.
Knight C, Kurbaan AS, Seggewiss H.  et al.  Nonsurgical septal reduction for hypertrophic obstructive cardiomyopathy.  Circulation.1997;95:2075-2081.
Faber L, Meissner A, Ziemssen P.  et al.  Percutaneous transluminal septal myocardial ablation for hypertrophic obstructive cardiomyopathy.  Heart.2000;83:326-331.
Lakkis NM, Nagueh SF, Kleiman NS.  et al.  Echocardiography-guided ethanol septal reduction for hypertrophic obstructive cardiomyopathy.  Circulation.1998;98:1750-1755.
Geitzen FH, Leuner ChJ, Raute-Kreinsen U.  et al.  Acute and long-term results after transcoronary ablation of septal hypertrophy (TASH).  Eur Heart J.1999;20:1342-1354.
Mazur W, Nagueh SF, Lakkis NM.  et al.  Regression of left ventricular hypertrophy after nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy.  Circulation.2001;103:1492-1496.
Nagueh SF, Ommen SR, Lakkis NM.  et al.  Comparison of ethanol septal reduction therapy with surgical myectomy for the treatment of hypertrophic cardiomyopathy.  J Am Coll Cardiol.2001;38:1701-1706.
Wigle ED, Schwartz L, Woo A, Rakowski H. To ablate or operate? that is the question.  J Am Coll Cardiol.2001;15:1707-1710.
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.
CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 959

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
JAMAevidence.com

The Rational Clinical Examination
Hypertrophic Cardiomyopathy

The Rational Clinical Examination EDUCATION GUIDES
Murmur, Systolic