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Managing Hypertrophic
Cardiomyopathy

Current treatment guidelines focus on symptom management1

Pharmacological intervention can help1

In the absence of large, randomized clinical trials, the 2020 American Heart Association (AHA)/American College of Cardiology Foundation (ACCF) Guidelines for the Diagnosis and Treatment of Patients with HCM recommend pharmacological therapy to, in certain circumstances, help manage HCM symptoms solely on an empirical basis.1,2

Invasive procedures may help reduce symptoms1,3-5

The 2020 AHA/ACC Guidelines also outline that some obstructive HCM patients who are symptomatic despite pharmacological treatment should be considered for invasive procedures.3

Guidelines list various options to help manage symptoms in certain HCM patients1:

Pharmalogical Icon

Pharmacological1

  • Non-vasodilating beta blockers

  • Non-dihydropyridine calcium channel blockers

  • Disopyramide

Surgical Icon

Invasive Procedures1

  • Septal reduction therapy (SRT)

    • Alcohol septal ablation

    • Septal myectomy

Beta blockers (BBs) and calcium channel blockers (CCBs) are typically seen in the earlier stages of HCM symptom management. For persistently symptomatic obstructive HCM patients who do not respond to BBs or CCBs, the next step is often adding on disopyramide or septal reduction therapy.1 These pharmacological options are included in the guidelines, but most are not FDA approved for the treatment of HCM.1

For specific guideline recommendations and management algorithms, please refer to the 2020 AHA/ACC Guidelines for the Diagnosis and Treatment of Patients with HCM.

Dr. Owens and Dr. Silvestri discuss challenges in monitoring patients with HCM.

Watch a video on the hidden risks and challenges of HCM

Dr. Owens and Dr. Silvestri discuss challenges in monitoring patients with HCM.

Patients may be on a path to unmanaged symptoms6-8

Given that disease progression is generally slow in HCM, patients experiencing symptoms may adapt to their disease and accept limitations without thinking to inform their healthcare provider.6,7 This can lead to unmanaged symptoms and lifestyle changes that negatively affect their emotional state and social functioning.8

Discussing HCM symptoms and monitoring the impact HCM has on patients’ physical, emotional, and psychological functioning is an important part of clinical assessments of disease severity and progression.1 Given the multiple aspects of lifestyle HCM can impact, an open conversation with patients, continued over multiple visits, can be helpful in establishing appropriate management.1

HCM is difficult to monitor6

As patients subtly adapt their lifestyles, subjective assessment of worsening symptoms and function may be complicated.6 Therefore, objective monitoring over a patient’s lifetime is crucial.6

Test1 Guideline Recommended Frequency1
12–Lead ECG or TTE Every 1–2 years
24– to 48–hour ambulatory ECG monitoring Every 1–2 years
Cardiopulmonary exercise testing Every 2–3 years for patients whose functional capacity or symptom status is uncertain
CMR imaging Every 3–5 years

Maintaining an open dialogue with affected patients is important for management.1

REFERENCES:
  1. Ommen SR, Mital S, Burke MA, et al. 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. 2020;142(25):e533-e631.

  2. 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;35(39):2733-2779.

  3. Marian AJ, Braunwald E. Hypertrophic cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 2017;121(7):749-770.

  4. Liebregts M, Vriesendorp PA, Mahmoodi BK, Schinkel AF, Michels M, ten Berg JM. A systematic review and meta-analysis of long-term outcomes after septal reduction therapy in patients with hypertrophic cardiomyopathy. JACC Heart Fail. 2015;3(11):896-905.

  5. Minakata K, Dearani JA, Schaff HV, O’Leary PW, Ommen SR, Danielson GK. Mechanisms for recurrent left ventricular outflow tract obstruction after septal myectomy for obstructive hypertrophic cardiomyopathy. Ann Thorac Surg. 2005;80(3):851-856.

  6. Jacoby DL, DePasquale EC, McKenna WJ. Hypertrophic cardiomyopathy: diagnosis, risk stratification and treatment. CMAJ. 2013;185(2):127-134.

  7. Naidu SS, ed. Hypertrophic Cardiomyopathy. London, Eng: Springer-Verlag; 2015.

  8. Sweeting J, Ingles J, Timperio A, Patterson J, Ball K, Semsarian C. Physical activity in hypertrophic cardiomyopathy: prevalence of inactivity and perceived barriers. Open Heart. 2016;3(2):e000484.

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