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J Am Coll Cardiol, 2000; 36:2212-2218
© 2000 by the American College of Cardiology Foundation
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CLINICAL STUDY: HYPERTROPHIC CARDIOMYOPATHY

Sudden death in hypertrophic cardiomyopathy: identification of high risk patients

Perry M. Elliott, MRCPa, Jan Poloniecki, DPhila, Shaughan Dickiea, Sanjay Sharma, BSc, MRCPa, Lorenzo Monserrat, MDa, Amanda Varnava, MRCPa, Niall G. Mahon, MD, MRCPIa and William J. McKenna, MD, FRCP, FACC, FESCa

a Department of Cardiological Sciences, St. George’s Hospital Medical School, London, United Kingdom

Manuscript received February 4, 2000; revised manuscript received July 10, 2000, accepted August 18, 2000.

Reprint requests and correspondence: Dr. P. M. Elliott, Department of Cardiological Sciences, St. George’s Hospital Medical School, Cranmer Terrace, London SW17 0RE, United Kingdom
pelliott{at}sghms.ac.uk

OBJECTIVES

We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD).

BACKGROUND

Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers.

METHODS

We studied 368 patients (14 to 65 years old, 239 males) with HCM. There were five variables: nonsustained ventricular tachycardia (NSVT), syncope, exercise blood pressure response (BPR), family history of sudden death (FHSD) and left ventricular wall thickness (LVWT).

RESULTS

During follow-up (3.6 ± 2.5 years [range 2 days to 9.6 years]), 36 patients (9.8%) died, 22 of them suddenly. Two patients received heart transplants. The six-year SD-free survival rate was 91% (95% confidence interval [CI] 87% to 95%). In the Cox model, there was a significant pairwise interaction between FHSD and syncope (p = 0.01), and these were subsequently considered together. The multivariate SD risk ratios (with 95% CIs) were 1.8 for BPR (0.7 to 4.4) (p = 0.22); 5.3 for FHSD and syncope (1.9 to 14.9) (p = 0.002); 1.9 for NSVT (0.7 to 5.0) (p = 0.18) and 2.9 for LVWT (1.1 to 7.1) (p = 0.03). Patients with no risk factors (n = 203) had an estimated six-year SD-free survival rate of 95% (95% CI 91% to 99%). The corresponding six-year estimates (with 95% CIs) for one (n = 122), two (n = 36) and three (n = 7) risk factors were 93% (87% to 99%), 82% (67% to 96%) and 36% (0% to 75%), respectively. Patients with two or more risk factors had a lower six-year SD survival rate (95% CI) compared with patients with one or no risk factors (72% [56% to 88%] vs. 94% [91% to 98%]) (p = 0.0001).

CONCLUSIONS

This study demonstrates that patients with multiple risk factors have a substantially increased risk of SD sufficient to warrant consideration for prophylactic therapy.

Abbreviations and Acronyms
  BPR = blood pressure response
  CI = confidence interval
  ECG = electrocardiogram or electrocardiographic
  FHSD = family history of sudden death
  HCM = hypertrophic cardiomyopathy
  LVWT = left ventricular wall thickness
  NSVT = nonsustained ventricular tachycardia
  NYHA = New York Heart Association
  SD = sudden death




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NEJMHome page
W. D. Binder, M. A. Fifer, M. E. King, and J. R. Stone
Case 26-2005 - A 48-Year-Old Man with Sudden Loss of Consciousness while Jogging
N. Engl. J. Med., August 25, 2005; 353(8): 824 - 832.
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CirculationHome page
C. Rickers, N. M. Wilke, M. Jerosch-Herold, S. A. Casey, P. Panse, N. Panse, J. Weil, A. G. Zenovich, and B. J. Maron
Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy
Circulation, August 9, 2005; 112(6): 855 - 861.
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J Am Coll CardiolHome page
I. Olivotto, M. S. Maron, A. S. Adabag, S. A. Casey, D. Vargiu, M. S. Link, J. E. Udelson, F. Cecchi, and B. J. Maron
Gender-Related Differences in the Clinical Presentation and Outcome of Hypertrophic Cardiomyopathy
J. Am. Coll. Cardiol., August 2, 2005; 46(3): 480 - 487.
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HeartHome page
R Thaman, J R Gimeno, R T Murphy, T Kubo, B Sachdev, J Mogensen, P M Elliott, and W J McKenna
Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy
Heart, July 1, 2005; 91(7): 920 - 925.
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JNMHome page
S. Isobe, H. Izawa, M. Iwase, M. Nanasato, M. Nonokawa, A. Ando, S. Ohshima, K. Nagata, K. Kato, T. Nishizawa, et al.
Cardiac 123I-MIBG Reflects Left Ventricular Functional Reserve in Patients with Nonobstructive Hypertrophic Cardiomyopathy
J. Nucl. Med., June 1, 2005; 46(6): 909 - 916.
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J Am Coll CardiolHome page
A. S. Adabag, S. A. Casey, M. A. Kuskowski, A. G. Zenovich, and B. J. Maron
Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy
J. Am. Coll. Cardiol., March 1, 2005; 45(5): 697 - 704.
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JNMHome page
P. A. Kaufmann and P. G. Camici
Myocardial Blood Flow Measurement by PET: Technical Aspects and Clinical Applications
J. Nucl. Med., January 1, 2005; 46(1): 75 - 88.
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HeartHome page
P P Dimitrow and J S Dubiel
Echocardiographic risk factors predisposing to sudden cardiac death in hypertrophic cardiomyopathy
Heart, January 1, 2005; 91(1): 93 - 94.
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J Am Coll CardiolHome page
J. Mogensen, R. T. Murphy, T. Kubo, A. Bahl, J. C. Moon, I. C. Klausen, P. M. Elliott, and W. J. McKenna
Frequency and clinical expression of cardiac troponin I mutations in 748 consecutive families with hypertrophic cardiomyopathy
J. Am. Coll. Cardiol., December 21, 2004; 44(12): 2315 - 2325.
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J Am Coll CardiolHome page
B. J. Maron, J. A. Dearani, S. R. Ommen, M. S. Maron, H. V. Schaff, B. J. Gersh, and R. A. Nishimura
The case for surgery in obstructive hypertrophic cardiomyopathy
J. Am. Coll. Cardiol., November 16, 2004; 44(10): 2044 - 2053.
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CirculationHome page
G. Boriani, B. J. Maron, W.-K. Shen, and P. Spirito
Prevention of Sudden Death in Hypertrophic Cardiomyopathy: But Which Defibrillator for Which Patient?
Circulation, October 12, 2004; 110(15): e438 - e442.
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Eur J EchocardiogrHome page
L. Faber, H. Seggewiss, D. Welge, D. Fassbender, H. K. Schmidt, U. Gleichmann, and D. Horstkotte
Echo-guided percutaneous septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: 7 years of experience
Eur J Echocardiogr, October 1, 2004; 5(5): 347 - 355.
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J Am Coll CardiolHome page
R. Thaman, J. R. Gimeno, S. Reith, M. T. T. Esteban, G. Limongelli, R. T. Murphy, B. Mist, W. J. McKenna, and P. M. Elliott
Progressive left ventricular remodeling in patients with hypertrophic cardiomyopathy and severe left ventricular hypertrophy
J. Am. Coll. Cardiol., July 21, 2004; 44(2): 398 - 405.
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HeartHome page
M. P Frenneaux
Assessing the risk of sudden cardiac death in a patient with hypertrophic cardiomyopathy
Heart, May 1, 2004; 90(5): 570 - 575.
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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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JNMHome page
S. Isobe, H. Izawa, Y. Takeichi, M. Nonokawa, M. Nanasato, A. Ando, K. Kato, M. Ikeda, T. Murohara, and M. Yokota
Relationship Between Exercise-Induced Myocardial Ischemia and Reduced Left Ventricular Distensibility in Patients with Nonobstructive Hypertrophic Cardiomyopathy
J. Nucl. Med., November 1, 2003; 44(11): 1717 - 1724.
[Abstract] [Full Text] [PDF]


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HeartHome page
A Woo, H Rakowski, J C Liew, M-S Zhao, C-C Liew, T G Parker, M Zeller, E D Wigle, and M J Sole
Mutations of the {beta} myosin heavy chain gene in hypertrophic cardiomyopathy: critical functional sites determine prognosis
Heart, October 1, 2003; 89(10): 1179 - 1185.
[Abstract] [Full Text] [PDF]



 
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