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*Cardiomyopathy

(Circulation. 1995;92:785-789.)
© 1995 American Heart Association, Inc.


Articles

Prevalence of Hypertrophic Cardiomyopathy in a General Population of Young Adults

Echocardiographic Analysis of 4111 Subjects in the CARDIA Study

Barry J. Maron, MD; Julius M. Gardin, MD; John M. Flack, MD, MPH; Samuel S. Gidding, MD; Tom T. Kurosaki, MS; Diane E. Bild, MD, MPH

From the Cardiovascular Research Division, Minneapolis Heart Institute Foundation; Divisions of Cardiology and Epidemiology, University of California, Irvine Medical Center, Orange; Division of General and Preventive Medicine, University of Minnesota School of Medicine, Minneapolis; Division of Pediatric Cardiology, Northwestern University Medical School, Chicago, Ill; and Division of Epidemiology and Clinical Applications, NHLBI, NIH, Bethesda, Md.

Correspondence to Dr Barry J. Maron, Cardiovascular Research Division, Minneapolis Heart Institute Foundation, 920 E 28th St, Ste 40, Minneapolis, MN 55407.

Background Hypertrophic cardiomyopathy (HCM) is a genetically transmitted disease and an important cause of morbidity and sudden cardiac death in young people, including competitive athletes. At present, however, few data exist to estimate the prevalence of this disease in large populations.

Methods and Results As part of the Coronary Artery Risk Development in (Young) Adults (CARDIA) Study, an epidemiological study of coronary risk factors, 4111 men and women 23 to 35 years of age selected from the general population of four urban centers had technically satisfactory echocardiographic studies during 1987 through 1988. Probable or definite echocardiographic evidence of HCM was present in 7 subjects (0.17%) on the basis of identification of a hypertrophied, nondilated left ventricle and maximal wall thickness >=15 mm that were not associated with systemic hypertension. Prevalence in men and women was 0.26:0.09%; in blacks and whites, 0.24:0.10%. Ventricular septal thickness was 15 to 21 mm (mean, 17 mm) in the 7 subjects. Only 1 of the 7 subjects had ever experienced important cardiac symptoms attributable to HCM, had previously been suspected of having cardiovascular disease, or had obstruction to left ventricular outflow; 4 other subjects had relatively mild systolic anterior motion of the mitral valve that was insufficient to produce dynamic basal outflow obstruction. ECGs were abnormal in 5 of the 7 subjects. Five other study subjects had left ventricular wall thicknesses of 15 to 21 mm that were a consequence of systemic hypertension.

Conclusions HCM was present in about 2 of 1000 young adults. These unique population-based data will aid in assessments of the impact of HCM-related mortality and morbidity in the general population and the practicality of screening large populations for HCM, including those comprising competitive athletes.


Key Words: cardiomyopathy • echocardiography • hypertrophy




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J. Biol. Chem.Home page
D. Szczesna-Cordary, G. Guzman, S.-S. Ng, and J. Zhao
Familial Hypertrophic Cardiomyopathy-linked Alterations in Ca2+ Binding of Human Cardiac Myosin Regulatory Light Chain Affect Cardiac Muscle Contraction
J. Biol. Chem., January 30, 2004; 279(5): 3535 - 3542.
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CirculationHome page
P. Sorajja, S. R. Ommen, R. A. Nishimura, B. J. Gersh, P. B. Berger, and A. J. Tajik
Adverse Prognosis of Patients With Hypertrophic Cardiomyopathy Who Have Epicardial Coronary Artery Disease
Circulation, November 11, 2003; 108(19): 2342 - 2348.
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J Am Coll CardiolHome page
B. J. Maron, W. J. McKenna, G. K. Danielson, L. J. Kappenberger, H. J. Kuhn, C. E. Seidman, P. M. Shah, W. H. Spencer III, P. Spirito, F. J. Ten Cate, 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., November 5, 2003; 42(9): 1687 - 1713.
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Eur Heart JHome page
Writing Committee Members, B. J. Maron, W. J. McKenna, G. K. Danielson, L. J. Kappenberger, H. J. Kuhn, C. E. Seidman, P. M. Shah, W. H. Spencer III, P. Spirito, 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
Eur. Heart J., November 1, 2003; 24(21): 1965 - 1991.
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Eur Heart JHome page
M. Alders, R. Jongbloed, W. Deelen, A. van den Wijngaard, P. Doevendans, F. Ten Cate, V. Regitz-Zagrosek, H.-P. Vosberg, I. van Langen, A. Wilde, et al.
The 2373insG mutation in the MYBPC3 gene is a founder mutation, which accounts for nearly one-fourth of the HCM cases in the Netherlands
Eur. Heart J., October 2, 2003; 24(20): 1848 - 1853.
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CirculationHome page
J. P. Schmitt, C. Semsarian, M. Arad, J. Gannon, F. Ahmad, C. Duffy, R. T. Lee, C. E. Seidman, and J.G. Seidman
Consequences of Pressure Overload on Sarcomere Protein Mutation-Induced Hypertrophic Cardiomyopathy
Circulation, September 2, 2003; 108(9): 1133 - 1138.
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Clin. Chem.Home page
M. Garcia-Castro, J. R. Reguero, A. Batalla, B. Diaz-Molina, P. Gonzalez, V. Alvarez, A. Cortina, G. I. Cubero, and E. Coto
Hypertrophic Cardiomyopathy: Low Frequency of Mutations in the {beta}-Myosin Heavy Chain (MYH7) and Cardiac Troponin T (TNNT2) Genes among Spanish Patients
Clin. Chem., August 1, 2003; 49(8): 1279 - 1285.
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CirculationHome page
S. L. Van Driest, E. G. Ellsworth, S. R. Ommen, A. J. Tajik, B. J. Gersh, and M. J. Ackerman
Prevalence and Spectrum of Thin Filament Mutations in an Outpatient Referral Population With Hypertrophic Cardiomyopathy * Note Added in Proof
Circulation, July 29, 2003; 108(4): 445 - 451.
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NEJMHome page
E. G. Nabel
Cardiovascular Disease
N. Engl. J. Med., July 3, 2003; 349(1): 60 - 72.
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CirculationHome page
C. G. Nebigil, F. Jaffre, N. Messaddeq, P. Hickel, L. Monassier, J.-M. Launay, and L. Maroteaux
Overexpression of the Serotonin 5-HT2B Receptor in Heart Leads to Abnormal Mitochondrial Function and Cardiac Hypertrophy
Circulation, July 1, 2003; 107(25): 3223 - 3229.
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J Am Coll CardiolHome page
J. G. Crilley, E. A. Boehm, E. Blair, B. Rajagopalan, A. M. Blamire, P. Styles, W. J. McKenna, I. Ostman-Smith, K. Clarke, and H. Watkins
Hypertrophic cardiomyopathy due to sarcomeric gene mutations is characterized by impaired energy metabolism irrespective of the degree of hypertrophy
J. Am. Coll. Cardiol., May 21, 2003; 41(10): 1776 - 1782.
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CirculationHome page
P. Richard, P. Charron, L. Carrier, C. Ledeuil, T. Cheav, C. Pichereau, A. Benaiche, R. Isnard, O. Dubourg, M. Burban, et al.
Hypertrophic Cardiomyopathy: Distribution of Disease Genes, Spectrum of Mutations, and Implications for a Molecular Diagnosis Strategy
Circulation, May 6, 2003; 107(17): 2227 - 2232.
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Eur Heart JHome page
D. J. Lips, L. J. deWindt, D. J.W. van Kraaij, and P. A. Doevendans
Molecular determinants of myocardial hypertrophy and failure: alternative pathways for beneficial and maladaptive hypertrophy
Eur. Heart J., May 2, 2003; 24(10): 883 - 896.
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J. Med. Genet.Home page
J Mogensen, A Bahl, T Kubo, N Elanko, R Taylor, and W J McKenna
Comparison of fluorescent SSCP and denaturing HPLC analysis with direct sequencing for mutation screening in hypertrophic cardiomyopathy
J. Med. Genet., May 1, 2003; 40(5): e59 - 59.
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J Am Coll CardiolHome page
A. J. Marian
On predictors of sudden cardiac death in hypertrophic cardiomyopathy
J. Am. Coll. Cardiol., March 19, 2003; 41(6): 994 - 996.
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Eur Heart JHome page
J. Mogensen, A. Bahl, and W. J McKenna
Hypertrophic cardiomyopathy--the clinical challenge of managing a hereditary heart condition
Eur. Heart J., March 2, 2003; 24(6): 496 - 498.
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