Cardiomyopathy overview
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Cardiomyopathy Microchapters |
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Diagnosis |
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Treatment |
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Guidelines |
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2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy |
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Cardiomyopathy overview On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Edzel Lorraine Co, DMD, MD[2] Muhammad Saad, M.B.B.S.[3]
Overview
Cardiomyopathy refers to diseases that involve the heart muscle and leads to its abnormal function and/or structure, in the absence of coronary artery disease, hypertension, valvular disease or congenital heart disease that would explain the myocardium dysfunction.
Classification
Cardiomyopathies can generally be categorized into two groups (based on World Health Organization guidelines): extrinsic cardiomyopathies and intrinsic cardiomyopathies [1]. Cardiomyopathy can also be classified from a clinical standpoint, as to whether it is restrictive, dilated, or hypertrophic.
Causes
There is a very wide range of factors that can cause cardiomyopathy, ranging from infectious causes, toxins, genetic causes and other disease processes.
Risk Factors
Some of the risk factors for developing cardiomyopathy include: a family history of cardiomyopathy, heart failure or sudden death, a history of other diseases that may lead to cardiomyopathy, obesity, alcoholism, and long standing high blood pressure. Certain diseases such as hemochromatosis, amyloidosis, and sarcoidosis also increase the risk of developing cardiomyopathy.
Natural History, Complications and Prognosis
Cardiomyopathy will continue to progressively worsen, unless intervened on. Complications and sequelae of cardiomyopathy include heart failure, arrythmia, thromboembolic disease, and sudden cardiac death.
Diagnosis
History and Symptoms
Patients may be asymptomatic in early stages of disease. When symptoms occur, they include: shortness of breath, edema of the lower extremities, fatigue, loss of consciousness, and palpitations due to arrythmias.
Physical Examination
A thorough physical examination can help in diagnosing cardiomyopathy. Particular attention should be given to the cardiac exam, the lung exam, the extremities for edema, and in assessing for other signs of heart failure such as jugular venous distension.
Chest X Ray
A chest x ray also shows fluid build-up, or a pleural effusion in the lungs, which can be a sign of the complication of heart failure.
Treatment
Medical Therapy
Treatment is phenotype-directed and includes guideline-directed medical therapy (GDMT) for heart failure syndromes and targeted therapy for specific cardiomyopathies such as hypertrophic cardiomyopathy (HCM). In HCM, management includes symptom-directed pharmacotherapy (beta-blocker or nondihydropyridine calcium-channel blocker, with disopyramide or a cardiac myosin inhibitor in selected adults with obstructive physiology), risk-based use of ICDs, and septal reduction therapy at experienced centers when symptoms persist despite medical therapy.[2] [3]For cardiomyopathy with HFrEF, foundational GDMT consists of an ARNI/ACE inhibitor/ARB, evidence-based beta-blocker, mineralocorticoid receptor antagonist, and SGLT2 inhibitor, with additional therapies and device-based treatment individualized by EF, QRS, symptoms, and comorbidities.[3] Goals include symptom relief, prevention of sudden cardiac death (when relevant, e.g., HCM), and modification of heart-failure trajectory; patients with advanced heart failure despite GDMT should be referred to an advanced HF team for consideration of transplant and/or durable mechanical circulatory support.[4] In HCM with atrial fibrillation, oral anticoagulation is recommended irrespective of CHA₂DS₂-VASc, and rhythm and/or rate control should be individualized.[2]
Surgery
Procedural and surgical options are phenotype-specific and should be performed at experienced centers when indicated.[2] For symptomatic obstructive HCM refractory to medical therapy, septal reduction therapy includes surgical septal myectomy or (in selected patients) alcohol septal ablation. Advanced heart failure may require durable mechanical circulatory support and/or heart transplantation after evaluation by an advanced HF team.[2][4]
Primary Prevention
Primary prevention focuses on ‘at risk’ (Stage A) and ‘pre-HF’ (Stage B) management: control hypertension, diabetes, obesity, and other cardiovascular risk factors; avoid cardiotoxins (including excess alcohol and illicit drugs); and treat structural heart disease early to prevent progression to symptomatic HF.[4][5]
Preferences
- ↑ Richardson, P.; et al. (1996). "Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies". Circulation. 93 (5): 841–2. PMID 8598070. (Full text)
- ↑ 2.0 2.1 2.2 2.3 Ommen SR, Ho CY, Asif IM, Balaji S, Burke MA, Day SM, Dearani JA, Epps KC, Evanovich L, Ferrari VA, Joglar JA, Khan SS, Kim JJ, Kittleson MM, Krittanawong C, Martinez MW, Mital S, Naidu SS, Saberi S, Semsarian C, Times S, Waldman CB (June 2024). "2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines". Circulation. 149 (23): e1239–e1311. doi:10.1161/CIR.0000000000001250. PMID 38718139 Check
|pmid=value (help). - ↑ 3.0 3.1 Heidenreich PA, Bozkurt B, Aguilar D, Allen LA, Byun JJ, Colvin MM, Deswal A, Drazner MH, Dunlay SM, Evers LR, Fang JC, Fedson SE, Fonarow GC, Hayek SS, Hernandez AF, Khazanie P, Kittleson MM, Lee CS, Link MS, Milano CA, Nnacheta LC, Sandhu AT, Stevenson LW, Vardeny O, Vest AR, Yancy CW (May 2022). "2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 145 (18): e895–e1032. doi:10.1161/CIR.0000000000001063. PMID 35363499 Check
|pmid=value (help). - ↑ 4.0 4.1 4.2 Heidenreich PA, Bozkurt B, Aguilar D, Allen LA, Byun JJ, Colvin MM, Deswal A, Drazner MH, Dunlay SM, Evers LR, Fang JC, Fedson SE, Fonarow GC, Hayek SS, Hernandez AF, Khazanie P, Kittleson MM, Lee CS, Link MS, Milano CA, Nnacheta LC, Sandhu AT, Stevenson LW, Vardeny O, Vest AR, Yancy CW (May 2022). "2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 145 (18): e876–e894. doi:10.1161/CIR.0000000000001062. PMID 35363500 Check
|pmid=value (help). - ↑ Joglar JA, Chung MK, Armbruster AL, Benjamin EJ, Chyou JY, Cronin EM, Deswal A, Eckhardt LL, Goldberger ZD, Gopinathannair R, Gorenek B, Hess PL, Hlatky M, Hogan G, Ibeh C, Indik JH, Kido K, Kusumoto F, Link MS, Linta KT, Marcus GM, McCarthy PM, Patel N, Patton KK, Perez MV, Piccini JP, Russo AM, Sanders P, Streur MM, Thomas KL, Times S, Tisdale JE, Valente AM, Van Wagoner DR (January 2024). "2023 ACC/AHA/ACCP/HRS Guideline for the Diagnosis and Management of Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines". Circulation. 149 (1): e1–e156. doi:10.1161/CIR.0000000000001193. PMC 11095842 Check
|pmc=value (help). PMID 38033089 Check|pmid=value (help).