on July 10, 2006
Published online before print July 10, 2006, doi: 10.1161/CIRCULATIONAHA.105.583500
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Submitted on August 29, 2005
From the Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation (K.M.H., A.G.Z., J.R.L., B.J.M.), Minneapolis, Minn; Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts-New England Medical Center (M.S.M., J.E.U.), Boston, Mass; Ente Ospedaliero Ospedali Galliera (P.S., F.F.), Genoa, Italy; Jesse E. Edwards Cardiovascular Registry (S.M.-B.), St. Paul, Minn; and Department of Medicine (Cardiovascular Division), Beth Israel Hospital and Harvard Medical School (W.J.M.), Boston, Mass. * To whom correspondence should be addressed. E-mail: hcm.maron{at}mhif.org.
Background--End stage (ES) is a recognized part of the hypertrophic cardiomyopathy (HCM) disease spectrum. Frequency, clinical profile and course, and treatment strategies in these patients remain incompletely defined. Methods and Results--Three HCM cohorts comprised 1259 patients, including 44 (3.5%) characterized as ES with systolic dysfunction (ejection fraction <50% at rest; range 15% to 49%). ES developed at a wide age range (14 to 74 years), with 45% of patients Conclusions--ES of nonobstructive HCM has an expanded and more diverse clinical expression than previously appreciated, including occurrence in young patients, heterogeneous patterns of remodeling, frequent association with atrial fibrillation, and impaired LV contractility that precedes cavity dilatation, wall thinning, and heart failure symptoms. ES is an unfavorable complication (mortality rate 11% per year) and a sudden death risk factor; it requires vigilance to permit timely recognition and the necessity for defibrillator implantation and heart transplantation.
Revised on May 8, 2006
Accepted on May 10, 2006
Prevalence, Clinical Profile, and Significance of Left Ventricular Remodeling in the End-Stage Phase of Hypertrophic Cardiomyopathy
Kevin M. Harris MD,
40 years old. Although 29 patients (66%) died of progressive heart failure, had sudden death events, or underwent heart transplantation, 15 (34%) survived with medical management over 3±3 years. Duration from onset of HCM symptoms to ES identification was considerable (14±10 years), but ES onset to death/transplantation was brief (2.7±2 years). ES occurred with similar frequency in patients with or without prior myectomy (P=0.84). Appropriate defibrillator interventions were 10% per year in patients awaiting donor hearts. Most ES patients (n=23; 52%) showed substantial left ventricular (LV) remodeling with cavity dilatation. Less complete remodeling occurred in 21 patients (48%), including 5 with persistence of a nondilated and markedly hypertrophied LV. Pathology and magnetic resonance imaging showed extensive (transmural) fibrosis in 9 of 11 ES patients. At initial evaluation, patients who developed ES were younger with more severe symptoms, had a larger LV cavity, and more frequently had a family history of ES than other HCM patients.
Key words: cardiomyopathy • echocardiography • heart failure • heart transplantation • hypertrophy • magnetic resonance imaging
This article has been cited by other articles:
 |
|
 |
|
  L.K. Williams, M.P. Frenneaux, and R.P. Steeds Echocardiography in hypertrophic cardiomyopathy diagnosis, prognosis, and role in management Eur J Echocardiogr, December 1, 2009; 10(8): iii9 - iii14. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B J Maron Distinguishing hypertrophic cardiomyopathy from athlete's heart physiological remodelling: clinical significance, diagnostic strategies and implications for preparticipation screening Br. J. Sports Med., September 1, 2009; 43(9): 649 - 656. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Maron, I. Olivotto, B. J. Maron, S. K. Prasad, F. Cecchi, J. E. Udelson, and P. G. Camici The case for myocardial ischemia in hypertrophic cardiomyopathy. J. Am. Coll. Cardiol., August 25, 2009; 54(9): 866 - 875. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron, M. S. Maron, E. D. Wigle, and E. Braunwald The 50-year history, controversy, and clinical implications of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy: from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy. J. Am. Coll. Cardiol., July 14, 2009; 54(3): 191 - 200. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Decker, J. W. Rossano, E. O. Smith, B. Cannon, S. K. Clunie, C. Gates, J. L. Jefferies, J. J. Kim, J. F. Price, W. J. Dreyer, et al. Risk factors and mode of death in isolated hypertrophic cardiomyopathy in children. J. Am. Coll. Cardiol., July 14, 2009; 54(3): 250 - 254. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. L. Houlihan, J. J. Metzler, and J. S. Blum HSP90{alpha} and HSP90{beta} Isoforms Selectively Modulate MHC Class II Antigen Presentation in B Cells J. Immunol., June 15, 2009; 182(12): 7451 - 7458. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. J. Maron, W. C. Roberts, M. Arad, T. S. Haas, P. Spirito, G. B. Wright, A. K. Almquist, J. M. Baffa, J. P. Saul, C. Y. Ho, et al. Clinical Outcome and Phenotypic Expression in LAMP2 Cardiomyopathy JAMA, March 25, 2009; 301(12): 1253 - 1259. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G S Soor, A Luk, E Ahn, J R Abraham, A Woo, A Ralph-Edwards, and J Butany Hypertrophic cardiomyopathy: current understanding and treatment objectives J. Clin. Pathol., March 1, 2009; 62(3): 226 - 235. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. C. Afonso, J. Bernal, J. J. Bax, and T. P. Abraham Echocardiography in hypertrophic cardiomyopathy: the role of conventional and emerging technologies. J. Am. Coll. Cardiol. Img., November 1, 2008; 1(6): 787 - 800. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Maron, J. J. Finley, J. M. Bos, T. H. Hauser, W. J. Manning, T. S. Haas, J. R. Lesser, J. E. Udelson, M. J. Ackerman, and B. J. Maron Prevalence, Clinical Significance, and Natural History of Left Ventricular Apical Aneurysms in Hypertrophic Cardiomyopathy Circulation, October 7, 2008; 118(15): 1541 - 1549. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J P Kaski, M T Tome-Esteban, S Mead-Regan, A Pantazis, J Marek, J E Deanfield, W J McKenna, and P M Elliott B-type natriuretic peptide predicts disease severity in children with hypertrophic cardiomyopathy Heart, October 1, 2008; 94(10): 1307 - 1311. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P Elliott and P Spirito Prevention of hypertrophic cardiomyopathy-related deaths: theory and practice Heart, October 1, 2008; 94(10): 1269 - 1275. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. S. Maron, E. Appelbaum, C. J. Harrigan, J. Buros, C. M. Gibson, C. Hanna, J. R. Lesser, J. E. Udelson, W. J. Manning, and B. J. Maron Clinical Profile and Significance of Delayed Enhancement in Hypertrophic Cardiomyopathy Circ Heart Fail, September 1, 2008; 1(3): 184 - 191. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. Sotgia, R. Sciagra, I. Olivotto, G. Casolo, L. Rega, I. Betti, A. Pupi, P. G. Camici, and F. Cecchi Spatial Relationship Between Coronary Microvascular Dysfunction and Delayed Contrast Enhancement in Patients with Hypertrophic Cardiomyopathy J. Nucl. Med., July 1, 2008; 49(7): 1090 - 1096. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Basavarajaiah, A. Boraita, G. Whyte, M. Wilson, L. Carby, A. Shah, and S. Sharma Ethnic differences in left ventricular remodeling in highly-trained athletes relevance to differentiating physiologic left ventricular hypertrophy from hypertrophic cardiomyopathy. J. Am. Coll. Cardiol., June 10, 2008; 51(23): 2256 - 2262. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Elliott Relevance of platelet activation in obstructive hypertrophic cardiomyopathy Heart, June 1, 2008; 94(6): 688 - 689. [Full Text] [PDF]
|
|
|
|
 |
|
 I. Olivotto, F. Girolami, M. J. Ackerman, S. Nistri, J. M. Bos, E. Zachara, S. R. Ommen, J. L. Theis, R. A. Vaubel, F. Re, et al. Myofilament Protein Gene Mutation Screening and Outcome of Patients With Hypertrophic Cardiomyopathy Mayo Clin. Proc., June 1, 2008; 83(6): 630 - 638. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron The 2006 American Heart Association Classification of Cardiomyopathies Is the Gold Standard Circ Heart Fail, May 1, 2008; 1(1): 72 - 76. [Full Text] [PDF]
|
|
|
|
 |
|
 D. P.S. Rogers, S. Marazia, A. W. Chow, P. D. Lambiase, M. D. Lowe, M. Frenneaux, W. J. McKenna, and P. M. Elliott Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy Eur J Heart Fail, May 1, 2008; 10(5): 507 - 513. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Basavarajaiah, M. Wilson, G. Whyte, A. Shah, W. McKenna, and S. Sharma Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening. J. Am. Coll. Cardiol., March 11, 2008; 51(10): 1033 - 1039. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Weidemann, M. Niemann, S. Herrmann, M. Kung, S. Stork, C. Waller, M. Beer, F. Breunig, C. Wanner, W. Voelker, et al. A new echocardiographic approach for the detection of non-ischaemic fibrosis in hypertrophic myocardium Eur. Heart J., December 2, 2007; 28(24): 3020 - 3026. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. J. Paulus, C. Tschope, J. E. Sanderson, C. Rusconi, F. A. Flachskampf, F. E. Rademakers, P. Marino, O. A. Smiseth, G. De Keulenaer, A. F. Leite-Moreira, et al. How to diagnose diastolic heart failure: a consensus statement on the diagnosis of heart failure with normal left ventricular ejection fraction by the Heart Failure and Echocardiography Associations of the European Society of Cardiology Eur. Heart J., October 2, 2007; 28(20): 2539 - 2550. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. G Assomull, D. J Pennell, and S. K Prasad Cardiovascular magnetic resonance in the evaluation of heart failure Heart, August 1, 2007; 93(8): 985 - 992. [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron, P. Spirito, W.-K. Shen, T. S. Haas, F. Formisano, M. S. Link, A. E. Epstein, A. K. Almquist, J. P. Daubert, T. Lawrenz, et al. Implantable Cardioverter-Defibrillators and Prevention of Sudden Cardiac Death in Hypertrophic Cardiomyopathy JAMA, July 25, 2007; 298(4): 405 - 412. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron Surgical Myectomy Remains the Primary Treatment Option for Severely Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy Circulation, July 10, 2007; 116(2): 196 - 206. [Full Text] [PDF]
|
|
|
|
|
|
B. J. Maron and C. Basso Myocarditis in hypertrophic cardiomyopathy Eur. Heart J., July 1, 2007; 28(13): 1663 - 1664. [Full Text] [PDF]
|
|
|
|
 |
|
 J. B. Kim, G. J. Porreca, L. Song, S. C. Greenway, J. M. Gorham, G. M. Church, C. E. Seidman, and J. G. Seidman Polony Multiplex Analysis of Gene Expression (PMAGE) in Mouse Hypertrophic Cardiomyopathy Science, June 8, 2007; 316(5830): 1481 - 1484. [Abstract] [Full Text] [PDF]
|
|