In general, patients with cardiomyopathy require lifelong follow-up to evaluate changes in symptoms, risk of adverse events, ventricular function, and cardiac rhythm.[1]Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023 Oct 1;44(37):3503-626.
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http://www.ncbi.nlm.nih.gov/pubmed/37622657?tool=bestpractice.com
A shared approach between cardiomyopathy units and general cardiologists is strongly recommended.[1]Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023 Oct 1;44(37):3503-626.
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http://www.ncbi.nlm.nih.gov/pubmed/37622657?tool=bestpractice.com
All patients should undergo clinical assessment and risk stratification at the time of diagnosis and at 1-2 year intervals, or whenever there is a change in clinical status.[1]Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023 Oct 1;44(37):3503-626.
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http://www.ncbi.nlm.nih.gov/pubmed/37622657?tool=bestpractice.com
[2]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 Dec 22;142(25):e558-631.
https://www.ahajournals.org/doi/10.1161/CIR.0000000000000937
http://www.ncbi.nlm.nih.gov/pubmed/33215931?tool=bestpractice.com
Such evaluation should include: a detailed family history, with particular emphasis on whether other family members with hypertrophic cardiomyopathy experienced premature death; a detailed history with inquiry into symptoms of chest pain, palpitations, or loss of consciousness; echocardiography; Holter monitoring; ECG; and exercise testing.[2]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 Dec 22;142(25):e558-631.
https://www.ahajournals.org/doi/10.1161/CIR.0000000000000937
http://www.ncbi.nlm.nih.gov/pubmed/33215931?tool=bestpractice.com
Cardiac magnetic resonance evaluation should be considered every 2-5 years or more frequently in patients with progressive disease.[1]Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023 Oct 1;44(37):3503-626.
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http://www.ncbi.nlm.nih.gov/pubmed/37622657?tool=bestpractice.com
Patients with risk factors for sudden death, including nonsustained ventricular tachycardia on sequential Holter monitorings, previous cardiac arrest, extreme hypertrophy, and unexplained syncope, should be closely monitored; implantable cardioverter-defibrillator placement should be considered. See Diagnostic approach for more information on risk stratification.
Frequent follow-up at 6-month intervals is recommended for those with high-risk features for sudden cardiac death (SCD) and focused on mitigation of risk. It is important to note that the European Society of Cardiology risk score was developed from cohort studies of nonathletes and it is difficult to ascertain whether the risk of SCD is further exacerbated by the stressors associated with high-intensity activities.[38]O'Mahony C, Jichi F, Pavlou M, et al. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM risk-SCD). Eur Heart J. 2014 Aug 7;35(30):2010-20.
https://academic.oup.com/eurheartj/article/35/30/2010/467191
http://www.ncbi.nlm.nih.gov/pubmed/24126876?tool=bestpractice.com
Therefore, further or more frequent screening strategies may be indicated in those participating in high-intensity athletic activities, especially those that are highly dynamic start-stop type exercises. Patients over 65 years of age may have additional prevalent cardiac comorbidities such as hypertension and diabetes that may increase their risk.[112]Maron BJ, Doerer JJ, Haas TS, et al. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation. 2009 Mar 3;119(8):1085-92.
https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.108.804617
http://www.ncbi.nlm.nih.gov/pubmed/19221222?tool=bestpractice.com