Hypertrophic cardiomyopathy risk factors for sudden death

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Contents

Overview

Patients with hypertrophic cardiomyopathy are increased risk of sudden cardiac death. Identification of those patients at an increased risk can facilitate early treatment with an automatic implantable cardiac defibrillator.

Risk Factors for Sudden Death in Patients with Hypertrophic Cardiomyopathy

Risk factors for sudden death in individuals with HCM include[1]:

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy (DO NOT EDIT)[2]

SCD Risk Stratification (DO NOT EDIT)[2]

Class I
"1. All patients with HCM should undergo comprehensive SCD risk stratification at initial evaluation to determine the presence of the following[3][4][5][6][7][8][9][10][11][12][13][14]: (Level of Evidence: B)"
"a. A personal history for ventricular fibrillation, sustained VT, or SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias."
"b. A family history for SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias."
"c. Unexplained syncope."
"d. Documented NSVT defined as 3 or more beats at greater than or equal to 120 bpm on ambulatory (Holter) ECG."
"e. Maximal LV wall thickness greater than or equal to 30 mm."
Class III
"1. Invasive electrophysiologic testing as routine SCD risk stratification for patients with HCM should not be performed. (Level of Evidence: C)"
Class IIa
"1. It is reasonable to assess blood pressure response during exercise as part of SCD risk stratification in patients with HCM[3][15][11]. (Level of Evidence: B)"
"2. SCD risk stratification is reasonable on a periodic basis (every 12 to 24 months) for patients with HCM who have not undergone ICD implantation but would otherwise be eligible in the event that risk factors are identified (12 to 24 months). (Level of Evidence: C)"
Class IIb
"1. The usefulness of the following potential SCD risk modifiers is unclear but might be considered in selected patients with HCM for whom risk remains borderline after documentation of conventional risk factors:"
"a. CMR imaging with LGE[16][17]. (Level of Evidence: C)"
"b. Double and compound mutations (ie, >1). (Level of Evidence: C)"
"c. Marked LVOT obstruction[3][18][19][11]. (Level of Evidence: B)"

References

  1. Maron BJ, Cecchi F, McKenna WJ (Dec 1994). "Risk factors and stratification for sudden cardiac death in patients with hypertrophic cardiomyopathy". Br Heart J 72 (6 Suppl): S13–8. doi:10.1136/hrt.72.6_Suppl.S13. PMID 7873317. and the Task Force on Sudden Cardiac Death of the European Society of Cardiology link Note: Guideline withdraw
  2. 2.0 2.1 Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (December 2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Journal of the American College of Cardiology 58 (25): e212–60. doi:10.1016/j.jacc.2011.06.011. PMID 22075469. Retrieved on 2011-12-19.
  3. 3.0 3.1 3.2 Elliott PM, Gimeno JR, Tomé MT, et al. (August 2006). "Left ventricular outflow tract obstruction and sudden death risk in patients with hypertrophic cardiomyopathy". Eur. Heart J. 27 (16): 1933–41. doi:10.1093/eurheartj/ehl041. PMID 16754630.
  4. Maron BJ, Savage DD, Wolfson JK, Epstein SE (August 1981). "Prognostic significance of 24 hour ambulatory electrocardiographic monitoring in patients with hypertrophic cardiomyopathy: a prospective study". Am. J. Cardiol. 48 (2): 252–7. PMID 7196685.
  5. Maron BJ (November 2010). "Risk stratification and role of implantable defibrillators for prevention of sudden death in patients with hypertrophic cardiomyopathy". Circ. J. 74 (11): 2271–82. PMID 20962423.
  6. Maron BJ (January 2010). "Contemporary insights and strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy". Circulation 121 (3): 445–56. doi:10.1161/CIRCULATIONAHA.109.878579. PMID 20100987.
  7. Cecchi F, Maron BJ, Epstein SE (May 1989). "Long-term outcome of patients with hypertrophic cardiomyopathy successfully resuscitated after cardiac arrest". J. Am. Coll. Cardiol. 13 (6): 1283–8. PMID 2703610.
  8. Elliott PM, Sharma S, Varnava A, Poloniecki J, Rowland E, McKenna WJ (May 1999). "Survival after cardiac arrest or sustained ventricular tachycardia in patients with hypertrophic cardiomyopathy". J. Am. Coll. Cardiol. 33 (6): 1596–601. PMID 10334430.
  9. Elliott PM, Poloniecki J, Dickie S, et al. (December 2000). "Sudden death in hypertrophic cardiomyopathy: identification of high risk patients". J. Am. Coll. Cardiol. 36 (7): 2212–8. PMID 11127463.
  10. Fananapazir L, Chang AC, Epstein SE, McAreavey D (September 1992). "Prognostic determinants in hypertrophic cardiomyopathy. Prospective evaluation of a therapeutic strategy based on clinical, Holter, hemodynamic, and electrophysiological findings". Circulation 86 (3): 730–40. PMID 1516184.
  11. 11.0 11.1 11.2 Maki S, Ikeda H, Muro A, et al. (September 1998). "Predictors of sudden cardiac death in hypertrophic cardiomyopathy". Am. J. Cardiol. 82 (6): 774–8. PMID 9761089.
  12. Maron BJ, Spirito P, Shen WK, et al. (July 2007). "Implantable cardioverter-defibrillators and prevention of sudden cardiac death in hypertrophic cardiomyopathy". JAMA 298 (4): 405–12. doi:10.1001/jama.298.4.405. PMID 17652294.
  13. McKenna W, Deanfield J, Faruqui A, England D, Oakley C, Goodwin J (March 1981). "Prognosis in hypertrophic cardiomyopathy: role of age and clinical, electrocardiographic and hemodynamic features". Am. J. Cardiol. 47 (3): 532–8. PMID 7193406.
  14. Spirito P, Autore C, Rapezzi C, et al. (April 2009). "Syncope and risk of sudden death in hypertrophic cardiomyopathy". Circulation 119 (13): 1703–10. doi:10.1161/CIRCULATIONAHA.108.798314. PMID 19307481.
  15. Sadoul N, Prasad K, Elliott PM, Bannerjee S, Frenneaux MP, McKenna WJ (November 1997). "Prospective prognostic assessment of blood pressure response during exercise in patients with hypertrophic cardiomyopathy". Circulation 96 (9): 2987–91. PMID 9386166.
  16. Adabag AS, Maron BJ, Appelbaum E, et al. (April 2008). "Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance". J. Am. Coll. Cardiol. 51 (14): 1369–74. doi:10.1016/j.jacc.2007.11.071. PMID 18387438.
  17. Moon JC, McKenna WJ, McCrohon JA, Elliott PM, Smith GC, Pennell DJ (May 2003). "Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance". J. Am. Coll. Cardiol. 41 (9): 1561–7. PMID 12742298.
  18. Efthimiadis GK, Parcharidou DG, Giannakoulas G, et al. (September 2009). "Left ventricular outflow tract obstruction as a risk factor for sudden cardiac death in hypertrophic cardiomyopathy". Am. J. Cardiol. 104 (5): 695–9. doi:10.1016/j.amjcard.2009.04.039. PMID 19699347.
  19. Maron MS, Olivotto I, Betocchi S, et al. (January 2003). "Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy". N. Engl. J. Med. 348 (4): 295–303. doi:10.1056/NEJMoa021332. PMID 12540642.

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