Hypertrophic cardiomyopathy natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Lakshmi Gopalakrishnan, M.B.B.S. [2] Soroush Seifirad, M.D.[3]

Overview

The natural history of hypertrophic cardiomyopathy is quite variable. Signs and symptoms range from none, to atrial fibrillation, heart failure, embolic stroke and sudden cardiac death. Signs and symptoms are quite variable from individual to individual but are also quite variable within a given family (all of whom carry the same mutation). The disease is quite variable in the timing of its appearance and may appear anywhere from infancy to late in adult life. About 25% of HCM patients achieve normal longevity. The myosin-binding proteins C genetic variant carries a good prognosis. The presence of ventricular fibrillation/ ventricular tachycardia carries the poorest prognosis. The severity of the outflow gradient is also related to prognosis. Athletes should be screened for HOCM based upon a family history of sudden cardiac death and a murmur on physical examination. Electrocardiograms and echocardiograms are not cost-effective screening tools in this population with a low pre-test probability of disease.

Natural History, Complications and Prognosis

The natural history of hypertrophic cardiomyopathy is quite variable. Signs and symptoms range from none, to atrial fibrillation, heart failure, embolic stroke and sudden cardiac death.[1][2][3][4][5] Signs and symptoms are quite variable from individual to individual but are also quite variable within a given family (all of whom carry the same mutation). The disease is quite variable in the timing of its appearance and may appear anywhere from infancy to late in adult life. About 25% of HCM patients achieve normal longevity.[6][7][8][9]The myosin binding proteins C genetic variant carries a good prognosis. The presence of VT / VF carries the poorest prognosis. The severity of the outflow gradient is also related to prognosis. Athletes should be screened for HOCM based upon a family history of sudden cardiac death and a murmur on physical examination. Electrocardiograms and echocardiograms are not cost-effective screening tools in this population with a low pre-test probability of disease.

Time and Age-Dependent Appearance of Left Ventricular Hypertrophy

Left ventricular hypertrophy may be absent in childhood. It may then appear following the rapid growth of adolescence and may first appear at age 17 to 18.[10][11][12]

Sudden Cardiac Death

The incidence of sudden cardiac death (SCD) in patients with HCM is 2 to 4 percent per year in adults, and a 4 to 6 percent per year in children and adolescents.[13]

Among end stage patients with a left ventricular ejection fraction < 50%, the risk of SCD over 5 years may be as high as 47%. In this population, syncope has been identified as an independent correlate of sudden cardiac death (hazard ratio = 6.15; 95% confidence interval, 2.40-15.75; P < .001).[14]

A review of 78 patients with HCM who died suddenly or survived a cardiac arrest episode showed that 71 percent were younger than 30 years of age, 54 percent were without functional limitation, and 61 percent were performing a sedentary or minimal physical activity at the time of the cardiac arrest.

Predictors of Sudden Cardiac Death

There are few predictors of SCD in patients with HCM.

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death - Hypertrophic Cardiomyopathy (DO NOT EDIT) [17]

Class I
"1. ICD therapy should be used for treatment in patients with HCM who have sustained VT and/or VF and who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: B)"
Class IIa
"1. ICD implantation can be effective for primary prophylaxis against SCD in patients with HCM who have 1 or more major risk factor (see Table 7) for SCD and who are receiving chronic optimal medical therapy and in patients who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)"
"2. Amiodarone therapy can be effective for treatment in patients with HCM with a history of sustained VT and/or VF when an ICD is not feasible. (Level of Evidence: C)"
Class IIb
"1. EP testing may be considered for risk assessment for SCD in patients with HCM. (Level of Evidence: C)"
"2. Amiodarone may be considered for primary prophylaxis against SCD in patients with HCM who have 1 or more major risk factor for SCD (see Table 7) if ICD implantation is not feasible. (Level of Evidence: C)"

Prognosis in Survivors of Sudden Cardiac Death

Survivors of SCD have a poor prognosis. Event free survival at 1, 5, and 10 years was 83, 65, and 53 percent respectively.

2011 ACCF/AHA Guideline Recommendations: SCD Risk Stratification

[18][19]

Class I
"1. All patients with HCM should undergo comprehensive SCD risk stratification at initial evaluation to determine the presence of the following: "
"a. A personal history for ventricular fibrillation, sustained VT, or SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias.† (Level of Evidence: B)"
"b. A family history for SCD events, including appropriate ICD therapy for ventricular tachyarrhythmias.† (Level of Evidence: B)"
"c. Unexplained syncope. (Level of Evidence: B)"
"d. Documented NSVT defined as 3 or more beats at greater than or equal to 120 bpm on ambulatory (Holter) ECG. (Level of Evidence: B) "
"e. Maximal LV wall thickness greater than or equal to 30 mm. (Level of Evidence: B) "
Class III (Harm)
"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.(89,127,390) (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.(184,188) (Level of Evidence: C)"
"b. Double and compound mutations (i.e., >1). (Level of Evidence: C) "
"c. Marked LVOT obstruction.(45,127,143,390) (Level of Evidence: B) "

Guideline Resources

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy [18][19]

References

  1. Maron BJ. Hypertrophic cardiomyopathy. Lancet 1997;350:127–33.
  2. Maron BJ. Hypertrophic cardiomyopathy. A systematic review. JAMA 2002;287:1308–20.
  3. Maki S, Ikeda H, Muro A et al. Predictors of sudden cardiac death in hypertrophic cardiomyopathy. Am J Cardiol 1998;82:774–8.
  4. Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650–5.
  5. Maron BJ, Olivotto I, Bellone P et al. Clinical profile of stroke in 900 patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2002;39:301–7.
  6. Maron BJ. Hypertrophic cardiomyopathy. A systematic review. JAMA 2002;287:1308–20.
  7. Maron BJ, Casey SA, Poliac LC, Gohman TE, Almquist AK, Aeppli DM. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. JAMA 1999;281:650–5.
  8. Fay WP, Taliercio CP, Ilstrup DM, Tajik AJ, Gersh BJ. Natural history of hypertrophic cardiomyopathy in the elderly. J Am Coll Cardiol 1990;16:821–6.
  9. Takagi E, Yamakado T, Nakano T. Prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1999;33:206–11.
  10. Hagege AA, Dubourg O, Desnos M et al. Familial hypertrophic cardiomyopathy. Cardiac ultrasonic abnormalities in genetically affected subjects without echocardiographic evidence of left ventricular hypertrophy. Eur Heart J 1998;19:490–9.
  11. Maron BJ, Spirito P, Wesley Y, Arce J. Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med 1986;315:610–4.
  12. Spirito P, Maron BJ. Absence of progression of left ventricular hypertrophy in adult patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 1987;9:1013–7.
  13. Elliott PM, Poloniecki J, Dickie S, Sharma S, Monserrat L, Varnava A; et al. (2000). "Sudden death in hypertrophic cardiomyopathy: identification of high risk patients". J Am Coll Cardiol. 36 (7): 2212–8. PMID 11127463.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  14. Kawarai H, Kajimoto K, Minami Y, Hagiwara N, Kasanuki H (2011). "Risk of sudden death in end-stage hypertrophic cardiomyopathy". J Card Fail. 17 (6): 459–64. doi:10.1016/j.cardfail.2011.01.015. PMID 21624733.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  15. Maron BJ, Tajik AJ, Ruttenberg HD et al. Hypertrophic cardiomyopathy in infants. Clinical features and natural history. Circulation 1982; 65:7–17
  16. Skinner JR, Manzoor A, Hayes AM, Joffe HS, Martin RP. A regional study of presentation and outcome of hypertrophic cardiomyopathy in infants. Heart 1997;77:229–33.
  17. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  18. 18.0 18.1 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 (2011). "2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary 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): 2703–38. doi:10.1016/j.jacc.2011.10.825. PMID 22075468. Retrieved 2011-12-19. Unknown parameter |month= ignored (help)<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>
  19. 19.0 19.1 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 (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 2011-12-19. Unknown parameter |month= ignored (help)<templatestyles src="Module:Citation/CS1/styles.css"></templatestyles>


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