Treatment Guideline Chart

Hypertrophic cardiomyopathy is a genetically determined cardiac disorder characterized by hypertrophy, often of the left ventricle (LV), with nondilated ventricular chambers and no other cardiac or systemic disease (eg aortic stenosis, thyroid disease or hypertension) capable of producing the extent of hypertrophy manifested by the patient.

Symptoms can range from patients being asymptomatic to mildly or significantly symptomatic with pre-syncopal or syncopal episodes, palpitations, chest pain, decreased exercise tolerance, symptoms of pulmonary congestion (eg fatigue, dyspnea, orthopnea and paroxysmal nocturnal dyspnea) and symptoms of end-stage heart failure.

Approach to treatment is based on clinical experience and observational data as large randomized trials regarding medical therapy in hypertrophic cardiomyopathy are lacking.

An empiric therapy is often necessary to identify the best possible drug for the patient, starting at a low dose and titrating until clinical improvement or occurrence of side effects.

Treat comorbidities such as hypertension, diabetes, obesity and hyperlipidemia according to existing guidelines due to the significant influence of concomitant coronary artery disease on the survival of patients with hypertrophic cardiomyopathy.


Hypertrophic Cardiomyopathy References

  1. Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014 Oct 14;35(39):2733-2779. doi: 10.1093/eurheartj/ehu284. PMID: 25173338
  2. Gersh BJ, Maron BJ, Bonow RO, et al; American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. 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. J Am Coll Cardiol. 2011 Dec 13;58(25):e212-60. doi: 10.1016/j.jacc.2011.06.011. Accessed 09 Jan 2018. PMID: 22075469
  3. Maron MS. Hypertrophic cardiomyopathy: medical therapy. UpToDate. https://www.uptodate.com/. 01 Mar 2017.
  4. Naidu SS. Diagnosis and management of hypertrophic cardiomyopathy. American College of Cardiology. http://www.acc.org/. 11 Feb 2015.
  5. Semsarian C; Cardiovascular Genetics Working Group. Diagnosis and management of hypertrophic cardiomyopathy - position statement. Cardiac Society of Australia and New Zealand (CSANZ). http://www.csanz.edu.au/resources/#clinical-practice. 2016.
  6. Sen-Chowdhry S, Jacoby D, Moon JC, McKenna WJ. Update on hypertrophic cardiomyopathy and a guide to the guidelines. Nat Rev Cardiol. 2016 Nov;13(11):651-675. doi: 10.1038/nrcardio.2016.140. PMID: 27681577
  7. Maron MS. Hypertrophic cardiomyopathy: Clinical manifestations, diagnosis, and evaluation. UpToDate. https://www.uptodate.com/. 04 Oct 2022.
  8. Ho CY, Mealiffe ME, Bach RG, et al. Evaluation of Mavacamten in symptomatic patients with nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2020 Jun;75(21):2649-2660. doi: 10.1016/j.jacc.2020.03.064. PMID: 32466879
  9. Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC Guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: Executive summary: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2020 Dec;76(25):3022-3055. doi: 10.1016/j.jacc.2020.08.044. PMID: 33229115
  10. Keam SJ. Mavacamten: First Approval. Drugs. 2022 Jul;82(Suppl 10):1127-1135. doi: 10.1007/s40265-022-01739-7. PMID: 35802255
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