Hypertrophic cardiomyopathy has been investigated mainly at referral institutions. Thus, the clinical history of the disease that emerges from published studies could be influenced by a bias in patient selection. In the present study, we compared the clinical features of an outpatient population of 25 patients who had hypertrophic cardiomyopathy with those reported in 78 studies published during the past five years. In the 25 study patients, age, sex, and the extent of left ventricular hypertrophy, as well as the prevalence of diastolic filling abnormalities, subaortic obstruction, and ventricular arrhythmias, were similar to those in patients described in the literature. Cardiac symptoms, however, were much less severe in the study patients. Eighteen patients (72 percent) were asymptomatic, six (24 percent) had mild symptoms, and only one (4 percent) had moderate-to-severe symptoms. Of 24 patients followed for a mean period of 4.4 years (range, 2.9 to 5.7), none died or had clinical deterioration. Of 3404 patients described in the 78 studies we reviewed, 2483 (73 percent) came from only two referral institutions. Of the 1721 patients in whom severity of symptoms was reported, 757 (44 percent) had moderate-to-severe symptoms. However, 727 (96 percent) of these patients were studied at one of the same two referral institutions. We conclude that the natural history of hypertrophic cardiomyopathy may be more benign than can be inferred from published reports.
Source Information
Divisione di Cardiologia, Ente Ospedaliero Ospedali Galliera, Genoa, Italy.
This article has been cited by other articles:
Spirito, P., Autore, C., Rapezzi, C., Bernabo, P., Badagliacca, R., Maron, M. S., Bongioanni, S., Coccolo, F., Estes, N.A. M., Barilla, C. S., Biagini, E., Quarta, G., Conte, M. R., Bruzzi, P., Maron, B. J.
(2009). Syncope and Risk of Sudden Death in Hypertrophic Cardiomyopathy. Circulation
119: 1703-1710
[Abstract][Full Text]
Maron, M. S., Finley, J. J., Bos, J. M., Hauser, T. H., Manning, W. J., Haas, T. S., Lesser, J. R., Udelson, J. E., Ackerman, M. J., Maron, B. J.
(2008). Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy. Circulation
118: 1541-1549
[Abstract][Full Text]
Elliott, P, Spirito, P
(2008). Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice. Heart
94: 1269-1275
[Abstract][Full Text]
Viskin, S., Rogowski, O.
(2007). Asymptomatic Brugada syndrome: a cardiac ticking time-bomb?. Europace
9: 707-710
[Full Text]
Maron, B. J., Spirito, P., Shen, W.-K., Haas, T. S., Formisano, F., Link, M. S., Epstein, A. E., Almquist, A. K., Daubert, J. P., Lawrenz, T., Boriani, G., Estes, N. A. M. III, Favale, S., Piccininno, M., Winters, S. L., Santini, M., Betocchi, S., Arribas, F., Sherrid, M. V., Buja, G., Semsarian, C., Bruzzi, P.
(2007). Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy. JAMA
298: 405-412
[Abstract][Full Text]
Colan, S. D., Lipshultz, S. E., Lowe, A. M., Sleeper, L. A., Messere, J., Cox, G. F., Lurie, P. R., Orav, E. J., Towbin, J. A.
(2007). Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children: Findings From the Pediatric Cardiomyopathy Registry. Circulation
115: 773-781
[Abstract][Full Text]
Daubeney, P. E. F., Nugent, A. W., Chondros, P., Carlin, J. B., Colan, S. D., Cheung, M., Davis, A. M., Chow, C.W., Weintraub, R. G., on behalf of the National Australian Childhood Car,
(2006). Clinical Features and Outcomes of Childhood Dilated Cardiomyopathy: Results From a National Population-Based Study. Circulation
114: 2671-2678
[Abstract][Full Text]
Nishimura, R. A., Ommen, S. R.
(2006). Hypertrophic Cardiomyopathy: The Search for Obstruction. Circulation
114: 2200-2202
[Full Text]
Morita, H., Larson, M. G., Barr, S. C., Vasan, R. S., O'Donnell, C. J., Hirschhorn, J. N., Levy, D., Corey, D., Seidman, C. E., Seidman, J.G., Benjamin, E. J.
(2006). Single-Gene Mutations and Increased Left Ventricular Wall Thickness in the Community: The Framingham Heart Study. Circulation
113: 2697-2705
[Abstract][Full Text]
Elliott, P M, Gimeno, J R, Thaman, R, Shah, J, Ward, D, Dickie, S, Tome Esteban, M T, McKenna, W J
(2006). Historical trends in reported survival rates in patients with hypertrophic cardiomyopathy. Heart
92: 785-791
[Abstract][Full Text]
Spirito, P., Autore, C.
(2006). Management of hypertrophic cardiomyopathy.. BMJ
332: 1251-1255
[Full Text]
Kubo, T., Kitaoka, H., Okawa, M., Matsumura, Y., Hitomi, N., Yamasaki, N., Furuno, T., Takata, J., Nishinaga, M., Kimura, A., Doi, Y. L.
(2005). Lifelong Left Ventricular Remodeling of Hypertrophic Cardiomyopathy Caused by a Founder Frameshift Deletion Mutation in the Cardiac Myosin-Binding Protein C Gene Among Japanese. J Am Coll Cardiol
46: 1737-1743
[Abstract][Full Text]
Nugent, A. W., Daubeney, P. E.F., Chondros, P., Carlin, J. B., Colan, S. D., Cheung, M., Davis, A. M., Chow, C.W., Weintraub, R. G., for the National Australian Childhood Cardiomyopat,
(2005). Clinical Features and Outcomes of Childhood Hypertrophic Cardiomyopathy: Results From a National Population-Based Study. Circulation
112: 1332-1338
[Abstract][Full Text]
Isobe, S., Izawa, H., Iwase, M., Nanasato, M., Nonokawa, M., Ando, A., Ohshima, S., Nagata, K., Kato, K., Nishizawa, T., Murohara, T., Yokota, M.
(2005). Cardiac 123I-MIBG Reflects Left Ventricular Functional Reserve in Patients with Nonobstructive Hypertrophic Cardiomyopathy. JNM
46: 909-916
[Abstract][Full Text]
Maron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., Shah, P. M., Spencer, W. H. III, Spirito, P., Ten Cate, F. J., Wigle, E. D., Vogel, R. A., Abrams, J., Bates, E. R., Brodie, B. R., Danias, P. G., Gregoratos, G., Hlatky, M. A., Hochman, J. S., Kaul, S., Lichtenberg, R. C., Lindner, J. R., O'rourke, R. A., Pohost, G. M., Schofield, R. S., Tracy, C. M., Winters, W. L. Jr, Klein, W. W., Priori, S. G., Alonso-Garcia, A., Blomstrom-Lundqvist, C., De Backer, G., Deckers, J., Flather, M., Hradec, J., Oto, A., Parkhomenko, A., Silber, S., Torbicki, A.
(2003). 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
42: 1687-1713
[Full Text]
Writing Committee Members, , Maron, B. J., McKenna, W. J., Danielson, G. K., Kappenberger, L. J., Kuhn, H. J., Seidman, C. E., Shah, P. M., Spencer, W. H. III, Spirito, P., Ten Cate, F. J., Wigle, E. D., ACCF Task Force on Clinical Expert Consensus Docum, , Vogel, R. A., Abrams, J., Bates, E. R., Brodie, B. R., Danias, P. G., Gregoratos, G., Hlatky, M. A., Hochman, J. S., Kaul, S., Lichtenberg, R. C., Lindner, J. R., O'Rourke, R. A., Pohost, G. M., Schofield, R. S., Tracy, C. M., Winters, W. L. Jr, ESC Committee for Practice Guidelines Members, , Klein, W. W., Priori, S. G., Alonso-Garcia, A., Blomstrom-Lundqvist, C., De Backer, G., Deckers, J., Flather, M., Hradec, J., Oto, A., Parkhomenko, A., Silber, S., Torbicki, A.
(2003). 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
24: 1965-1991
[Full Text]
Nugent, A. W., Daubeney, P. E.F., Chondros, P., Carlin, J. B., Cheung, M., Wilkinson, L. C., Davis, A. M., Kahler, S. G., Chow, C.W., Wilkinson, J. L., Weintraub, R. G., the National Australian Childhood Cardiomyopathy S,
(2003). The Epidemiology of Childhood Cardiomyopathy in Australia. NEJM
348: 1639-1646
[Abstract][Full Text]
Kofflard, M. J. M., Ten Cate, F. J., van der Lee, C., van Domburg, R. T.
(2003). Hypertrophic cardiomyopathy in a large community-based population: clinical outcome and identification of risk factors for sudden cardiac death and clinical deterioration. J Am Coll Cardiol
41: 987-993
[Abstract][Full Text]
Marian, A. J.
(2003). On predictors of sudden cardiac death in hypertrophic cardiomyopathy. J Am Coll Cardiol
41: 994-996
[Full Text]
Choudhury, L., Mahrholdt, H., Wagner, A., Choi, K. M., Elliott, M. D., Klocke, F. J., Bonow, R. O., Judd, R. M., Kim, R. J.
(2002). Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. J Am Coll Cardiol
40: 2156-2164
[Abstract][Full Text]
Autore, C., Conte, M. R., Piccininno, M., Bernabo, P., Bonfiglio, G., Bruzzi, P., Spirito, P.
(2002). Risk associated with pregnancy in hypertrophic cardiomyopathy. J Am Coll Cardiol
40: 1864-1869
[Abstract][Full Text]
Freed, L. A., Benjamin, E. J., Levy, D., Larson, M. G., Evans, J. C., Fuller, D. L., Lehman, B., Levine, R. A.
(2002). Mitral valve prolapse in the general population: the benign nature of echocardiographic features in the Framingham Heart Study. J Am Coll Cardiol
40: 1298-1304
[Abstract][Full Text]
Braunwald, E., Seidman, C. E., Sigwart, U.
(2002). Contemporary Evaluation and Management of Hypertrophic Cardiomyopathy. Circulation
106: 1312-1316
[Full Text]
Ciampi, Q., Betocchi, S., Lombardi, R., Manganelli, F., Storto, G., Losi, M. A., Pezzella, E., Finizio, F., Cuocolo, A., Chiariello, M.
(2002). Hemodynamic determinants of exercise-induced abnormal blood pressure response in hypertrophic cardiomyopathy. J Am Coll Cardiol
40: 278-284
[Abstract][Full Text]
Epstein, J. A., Rader, D. J., Parmacek, M. S.
(2002). Perspective: Cardiovascular Disease in the Postgenomic Era--Lessons Learned and Challenges Ahead. Endocrinology
143: 2045-2050
[Full Text]
Maron, B. J.
(2002). Hypertrophic Cardiomyopathy: A Systematic Review. JAMA
287: 1308-1320
[Abstract][Full Text]
Maron, B. J., Olivotto, I., Bellone, P., Conte, M. R., Cecchi, F., Flygenring, B. P., Casey, S. A., Gohman, T. E., Bongioanni, S., Spirito, P.
(2002). Clinical profile of stroke in 900 patients with hypertrophic cardiomyopathy. J Am Coll Cardiol
39: 301-307
[Abstract][Full Text]
Jouven, X., Ducimetiere, P.
(2001). Exercise testing: do frequent premature ventricular depolarizations represent a new criterion of positivity?. Eur Heart J
22: 1759-1761
Ryden, L., Gadler, F.
(2001). Pharmacological therapy for hypertrophic cardiomyopathy: what is the evidence for success?. Eur Heart J Suppl
3: L21-L25
[Abstract]
Priori, S.G., Aliot, E., Blomstrom-Lundqvist, C., Bossaert, L., Breithardt, G., Brugada, P., Camm, A.J., Cappato, R., Cobbe, S.M., Di Mario, C., Maron, B.J., McKenna, W.J., Pedersen, A.K., Ravens, U., Schwartz, P.J., Trusz-Gluza, M., Vardas, P., Wellens, H.J.J., Zipes, D.P.
(2001). Task Force on Sudden Cardiac Death of the European Society of Cardiology. Eur Heart J
22: 1374-1450
Elliott, P. M., Poloniecki, J., Dickie, S., Sharma, S., Monserrat, L., Varnava, A., Mahon, N. G., McKenna, W. J.
(2000). Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol
36: 2212-2218
[Abstract][Full Text]
Jouven, X., Zureik, M., Desnos, M., Courbon, D., Ducimetiere, P.
(2000). Long-Term Outcome in Asymptomatic Men with Exercise-Induced Premature Ventricular Depolarizations. NEJM
343: 826-833
[Abstract][Full Text]
Maron, B. J., Olivotto, I., Spirito, P., Casey, S. A., Bellone, P., Gohman, T. E., Graham, K. J., Burton, D. A., Cecchi, F.
(2000). Epidemiology of Hypertrophic Cardiomyopathy-Related Death : Revisited in a Large Non-Referral-Based Patient Population. Circulation
102: 858-864
[Abstract][Full Text]
Spirito, P., Bellone, P., Harris, K. M., Bernabo, P., Bruzzi, P., Maron, B. J.
(2000). Magnitude of Left Ventricular Hypertrophy and Risk of Sudden Death in Hypertrophic Cardiomyopathy. NEJM
342: 1778-1785
[Abstract][Full Text]
Sorajja, P., Elliott, P.M., Mckenna, W.J.
(2000). The molecular genetics of hypertrophic cardiomyopathy: prognostic implications. Europace
2: 4-14
Jeresaty, R. M., Cheng, T. O., Freed, L. A., Levy, D., Levine, R. A., Evans, J. C., Benjamin, E. J.
(1999). Mitral-Valve Prolapse. NEJM
341: 1471-1472
[Full Text]
Freed, L. A., Levy, D., Levine, R. A., Larson, M. G., Evans, J. C., Fuller, D. L., Lehman, B., Benjamin, E. J.
(1999). Prevalence and Clinical Outcome of Mitral-Valve Prolapse. NEJM
341: 1-7
[Abstract][Full Text]
Olivotto, I., Maron, B. J., Montereggi, A., Mazzuoli, F., Dolara, A., Cecchi, F.
(1999). Prognostic value of systemic blood pressure response during exercise in a community-based patient population with hypertrophic cardiomyopathy. J Am Coll Cardiol
33: 2044-2051
[Abstract][Full Text]
Maron, B. J., Mathenge, R., Casey, S. A., Poliac, L. C., Longe, T. F.
(1999). Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities. J Am Coll Cardiol
33: 1590-1595
[Abstract][Full Text]
Elliott, P. M., Sharma, S., Varnava, A., Poloniecki, J., Rowland, E., McKenna, W. J.
(1999). Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol
33: 1596-1601
[Abstract][Full Text]
Spirito, P., Rapezzi, C., Bellone, P., Betocchi, S., Autore, C., Conte, M. R., Bezante, G. P., Bruzzi, P.
(1999). Infective Endocarditis in Hypertrophic Cardiomyopathy : Prevalence, Incidence, and Indications for Antibiotic Prophylaxis. Circulation
99: 2132-2137
[Abstract][Full Text]
Maron, B. J., Casey, S. A., Poliac, L. C., Gohman, T. E., Almquist, A. K., Aeppli, D. M.
(1999). Clinical Course of Hypertrophic Cardiomyopathy in a Regional United States Cohort. JAMA
281: 650-655
[Abstract][Full Text]
Takagi, E., Yamakado, T., Nakano, T.
(1999). Prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol
33: 206-211
[Abstract][Full Text]
Yetman, A. T., Hamilton, R. M., Benson, L. N., McCrindle, B. W.
(1998). Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy. J Am Coll Cardiol
32: 1943-1950
[Abstract][Full Text]
Jaaskelainen, P., Soranta, M., Miettinen, R., Saarinen, L., Pihlajamaki, J., Silvennoinen, K., Tikanoja, T., Laakso, M., Kuusisto, J.
(1998). The cardiac {beta}-myosin heavy chain gene is not the predominant gene for hypertrophic cardiomyopathy in the Finnish population. J Am Coll Cardiol
32: 1709-1716
[Abstract][Full Text]
Maron, B. J., Moller, J. H., Seidman, C. E., Vincent, G. M., Dietz, H. C., Moss, A. J., Towbin, J. A., Sondheimer, H. M., Pyeritz, R. E., McGee, G., Epstein, A. E.
(1998). Impact of Laboratory Molecular Diagnosis on Contemporary Diagnostic Criteria for Genetically Transmitted Cardiovascular Diseases: Hypertrophic Cardiomyopathy, Long-QT Syndrome, and Marfan Syndrome : A Statement for Healthcare Professionals From the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association. Circulation
98: 1460-1471
[Full Text]
PRASAD, K, FRENNEAUX, M P
(1998). Hypertrophic cardiomyopathy: is there a role for amiodarone?. Heart
79: 317-318
[Full Text]
Cecchi, F, Olivotto, I, Montereggi, A, Squillatini, G, Dolara, A, Maron, B J
(1998). 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
79: 331-336
[Abstract][Full Text]
Kizilbash, A. M., Heinle, S. K., Grayburn, P. A.
(1998). Spontaneous Variability of Left Ventricular Outflow Tract Gradient in Hypertrophic Obstructive Cardiomyopathy. Circulation
97: 461-466
[Abstract][Full Text]
Lazzeroni, E., Picano, E., Morozzi, L., Maurizio, A. R., Palma, G., Ceriati, R., Iori, E., Barilli, A.
(1997). Dipyridamole-Induced Ischemia as a Prognostic Marker of Future Adverse Cardiac Events in Adult Patients With Hypertrophic Cardiomyopathy. Circulation
96: 4268-4272
[Abstract][Full Text]
Sadoul, N., Prasad, K., Elliott, P. M., Bannerjee, S., Frenneaux, M. P., McKenna, W. J.
(1997). Prospective Prognostic Assessment of Blood Pressure Response During Exercise in Patients With Hypertrophic Cardiomyopathy. Circulation
96: 2987-2991
[Abstract][Full Text]
Spirito, P., Seidman, C. E., McKenna, W. J., Maron, B. J.
(1997). The Management of Hypertrophic Cardiomyopathy. NEJM
336: 775-785
[Full Text]
Cannan, C. R., Reeder, G. S., Bailey, K. R., Melton, L. J. III, Gersh, B. J.
(1995). Natural History of Hypertrophic Cardiomyopathy : A Population-Based Study, 1976 Through 1990. Circulation
92: 2488-2495
[Abstract][Full Text]
Wigle, E. D., Rakowski, H., Kimball, B. P., Williams, W. G.
(1995). Hypertrophic Cardiomyopathy : Clinical Spectrum and Treatment. Circulation
92: 1680-1692
[Full Text]
Maron, B. J., Gardin, J. M., Flack, J. M., Gidding, S. S., Kurosaki, T. T., Bild, D. E.
(1995). Prevalence of Hypertrophic Cardiomyopathy in a General Population of Young Adults : Echocardiographic Analysis of 4111 Subjects in the CARDIA Study. Circulation
92: 785-789
[Abstract][Full Text]
Maron, B. J., Pelliccia, A., Spirito, P.
(1995). 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
91: 1596-1601
[Full Text]
DiMarco, J. P., Philbrick, J. T.
(1990). Use of Ambulatory Electrocardiographic (Holter) Monitoring. ANN INTERN MED
113: 53-68
[Abstract]
