Hypertrophic cardiomyopathy primary prevention

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

Overview

There is no primary prevention for hypertrophic cardiomyopathy. This is a genetic familial disorder. But there are important approaches to decrease and prevent development of sudden death and heart attack in known cases of HCM (tertiary Prevention). Any activity, drug or circumstance that increases left ventricular outflow obstruction, reduced left ventricular filling, or increases left ventricular afterload should be avoided.

Activities That Increase Left Ventricular Outflow Tract Obstruction

Activities That Reduce Preload

The following activities increase left ventricular outflow tract obstruction and should be avoided:[1][2][3][4][5]

  • Nausea and vomiting
  • Dehydration
  • Hypovolemia (i.e., use diuretics with caution)
  • Medications that reduce preload and left ventricular filling such as nitrates
  • Alcohol ingestion may change outflow obstruction due to vaso and veno dilation. In one randomized trial, patients who ingested alcohol in amounts that mimic that ingested during social circumstances sustained rise in their gradient from 38 to 62 mm Hg, a drop in their systolic blood pressure from 132 to 122 mm Hg and an increase in systolic anterior motion (SAM) of the mitral valve.
  • Heavy meals may change the outflow obstruction for a wide variety of reasons including splanchnic pulling of blood

Activities That Increase Afterload

The following activities increase left ventricular afterload should be avoided:

  • Competitive endurance training
  • Burst activities (e.g., sprinting)
  • Intense isometric exercise (e.g., heavy weight lifting)

ICD

Indications for implantable cardioverter-defibrillator (primary prevention)

  • Family history of SCD
  • Non sustained VT on Holter monitoring
  • Syncope: recurrent or exercise-associated
  • Hypotension during exercise
  • Sever septal thickness (more than 3 cm)

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy A Report of the American College of Cardiology/American Heart  Association Joint Committee on Clinical Practice Guidelines[6]

Recommendations for Sports and Activity Referenced studies that support the recommendations are summarized in the Online Data Supplement

Class I
1. For most patients with HCM, mild to moderate-intensity recreational* exercise is beneficial to improve cardiorespiratory fitness, physical functioning, and quality of life, and for their overall health in keeping with physical activity guidelines for the general population(Level of Evidence: B-NR)

2. For athletes with HCM, a comprehensive evaluation and shared discussion of potential risks of sports participation by an expert provider is recommended(Level of Evidence: C-EO)

Class IIa
3. For most patients with HCM, participation in low-intensity competitive sports is reasonable.(Level of Evidence: C-EO)

4. In individuals who are genotype-positive, phenotype-negative for HCM, participation in competitive athletics of any intensity is reasonable(Level of Evidence: C-LD)

Class IIb
5. For patients with HCM, participation in high-intensity recreational activities or moderate- to high-intensity competitive sports activities may be considered after a comprehensive evaluation and shared discussion, repeated annually with an expert provider who conveys that the risk of sudden death and ICD shocks may be increased, and with the understanding that eligibility decisions for competitive sports participation often involve third parties (eg, team physicians, consultants, and other institutional leadership) acting on behalf of the schools or teams.(Level of Evidence: C-LD)

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

Participation in Competitive or Recreational Sports and Physical Activity (DO NOT EDIT)[7]

Class IIa
"1. It is reasonable for patients with HCM to participate in low-intensity competitive sports (eg, golf and bowling). (Level of Evidence: C) "
"2. It is reasonable for patients with HCM to participate in a range of recreational sporting activities. (Level of Evidence: C) "
Class III (Harm)
"1. Patients with HCM should not participate in intense competitive sports regardless of age, sex, race, presence or absence of LVOT obstruction, prior septal reduction therapy, or implantation of a cardioverter-defibrillator for high-risk status[8][9]. (Level of Evidence: C) "

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. Ommen SR, Mital S, Burke MA, Day SM, Deswal A, Elliott P; et al. (2020). "2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 142 (25): e558–e631. doi:10.1161/CIR.0000000000000937. PMID 33215931 Check |pmid= value (help).
  7. 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)
  8. Maron BJ, Ackerman MJ, Nishimura RA, Pyeritz RE, Towbin JA, Udelson JE (2005). "Task Force 4: HCM and other cardiomyopathies, mitral valve prolapse, myocarditis, and Marfan syndrome". J. Am. Coll. Cardiol. 45 (8): 1340–5. doi:10.1016/j.jacc.2005.02.011. PMID 15837284. Unknown parameter |month= ignored (help)
  9. Pelliccia A, Fagard R, Bjørnstad HH; et al. (2005). "Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology". Eur. Heart J. 26 (14): 1422–45. doi:10.1093/eurheartj/ehi325. PMID 15923204. Unknown parameter |month= ignored (help)


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