Dilated cardiomyopathy

Last updated: January 17, 2023

Summarytoggle arrow icon

Dilated cardiomyopathy (DCM) is the occurrence of ventricular dilatation and systolic dysfunction despite normal filling pressures, in the absence of coronary artery disease, abnormal loading pressures (e.g., valvular heart disease, hypertension), or congenital heart disease. It is the most common type of cardiomyopathy. Although most cases are idiopathic or inherited, DCM can also be caused by a number of conditions (e.g., endocrine disorders, autoimmune disease) and infections (e.g., Coxsackie B virus, Chagas disease) and by the use of certain substances (e.g., heavy drinking, cocaine). In DCM, decreased left ventricular contractility leads to left heart failure and eventually right heart failure with decreased ventricular output. Isolated dilation and subsequent decrease in right ventricular contractility is rare. Diagnosis is confirmed with echocardiography. Studies to evaluate the underlying etiology should be guided by clinical suspicion and include laboratory studies, genetic testing, advanced cardiac imaging, and, rarely, endomyocardial biopsy. Management involves treatment of the underlying condition and associated complications, e.g., congestive heart failure and arrhythmias. Severe or refractory disease may be managed with device implantation or cardiac transplantation.

Left or biventricular dilatation with structural and/or functional systolic dysfunction in the absence of coronary artery disease, abnormal loading pressures (e.g., valvular heart disease, hypertension), or congenital heart disease [1][2]

  • Incidence: ∼ 6/100,000 per year (most common cardiomyopathy) [3][4]
  • Sex: > (∼ 1.5:1) [5]
  • Age at presentation: most commonly between 30 and 40 years of age, but can occur at any age [2]
  • Ethnicity: more common in individuals of African descent [2]

Epidemiological data refers to the US, unless otherwise specified.

Primary causes

Secondary causes [8]

Volume and pressure overload secondary to conditions such as hypertension and valvular heart disease can cause dilation of the myocardium; however, these are not considered forms of DCM, as filling pressures are abnormal. [1]

To remember some high-yield secondary causes of dilated cardiomyopathy, think ABCCCDD: Alcohol use, Beriberi, Cocaine, Coxsackie B virus, Chagas, Doxorubicin/Daunorubicin

Symptoms [1]

Physical examination [11]

DCM is typically diagnosed during the workup for associated cardiac conditions (e.g., heart failure) or through screening of family members of patients with known or suspected familial DCM. [13]

Approach [8][14]

Echocardiography [14]

  • Indications
    • To confirm the diagnosis of suspected DCM
    • To screen first-degree relatives of patients with familial DCM [1][8][14]
  • Characteristic findings

Offer screening with a physical examination, ECG, and echocardiography (with assessment of left ventricular size and function) to first-degree relatives of patients with familial DCM. [7][14]

Initial diagnostic workup

Laboratory studies [8][14]

Chest x-ray

ECG [8][18]

Holter monitoring

Genetic testing [14]

Refer patients for genetic counseling prior to ordering genetic testing for DCM. [7]

Advanced studies [8][14]

Cardiac MRI

Endomyocardial biopsy

  • Indication: suspected underlying etiology that requires specific management (e.g., amyloidosis) and cannot be confirmed by other diagnostic methods
  • Findings vary based on the underlying etiology.

Overview [8][14]

Management of the underlying etiology [14]

Management of underlying disease in dilated cardiomyopathy [14]
Treatment
Substance use disorder
Endocrine disorders Hyperthyroidism
Hypothyroidism
  • Start levothyroxine replacement.
  • For older patients and those with known or suspected CAD: [14]
    • Start thyroid hormone replacement at 25–50% of the expected required dose.
    • Titrate up as tolerated every 6–8 weeks.
Acromegaly

Infections

Myocarditis
HIV
Chagas disease
Chemotherapy-related cardiomyopathy
  • All patients: Monitor cardiac function before, during, and after treatment with cardiotoxic chemotherapy agents.
  • For patients undergoing anthracycline-based chemotherapy, consider:
    • Selecting less cardiotoxic derivatives (e.g., epirubicin or idarubicin)
    • Using cardioprotective agents (e.g., dexrazoxane)
  • For patients with cardiac damage: Weigh the risks and benefits of continued treatment with cardiotoxic agents.
Peripartum cardiomyopathy
Autoimmune diseases

Avoid immunosuppressants with potential cardiotoxicity (e.g., etanercept, infliximab) in patients with DCM. [14]

Management of complications

Severe or refractory DCM [17][21]

DCM is the leading indication for heart transplantation. [25]

Dilated cardiomyopathy in pregnancy [15]

Planning pregnancy

During pregnancy

Avoid ACEIs, ARBs, and mineralocorticoid receptor antagonists during the preconception period and pregnancy. [15]

  1. Sinagra G, Merlo M, Pinamonti B. Dilated Cardiomyopathy. Springer ; 2019
  2. Schultheiss HP, Fairweather D, Caforio ALP, et al. Dilated cardiomyopathy. Nat Rev Dis Primers. 2019; 5 (1). doi: 10.1038/s41572-019-0084-1 . | Open in Read by QxMD
  3. P. Schild D, I. Ricciardi S, G. Hellige J, Vogel R, Arenja N. Current Pathophysiological and Genetic Aspects of Dilated Cardiomyopathy. IntechOpen ; 2019
  4. Seferović PM, Polovina M, Bauersachs J, et al. Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology. Eur J Heart Fail. 2019; 21 (5): p.553-576. doi: 10.1002/ejhf.1461 . | Open in Read by QxMD
  5. Rapezzi C, Arbustini E, Caforio ALP, et al. Diagnostic work-up in cardiomyopathies: bridging the gap between clinical phenotypes and final diagnosis. A position statement from the ESC Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2012; 34 (19): p.1448-1458. doi: 10.1093/eurheartj/ehs397 . | Open in Read by QxMD
  6. Japp AG, Gulati A, Cook SA, Cowie MR, Prasad SK. The Diagnosis and Evaluation of Dilated Cardiomyopathy. J Am Coll Cardiol. 2016; 67 (25): p.2996-3010. doi: 10.1016/j.jacc.2016.03.590 . | Open in Read by QxMD
  7. Bozkurt B, Colvin M, Cook J, et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016; 134 (23). doi: 10.1161/cir.0000000000000455 . | Open in Read by QxMD
  8. McNally EM, Mestroni L. Dilated Cardiomyopathy. Circ Res. 2017; 121 (7): p.731-748. doi: 10.1161/circresaha.116.309396 . | Open in Read by QxMD
  9. Zipes DP. Braunwald's Heart Disease. Mosby ; 2018
  10. Anand IS, Gupta P. Anemia and Iron Deficiency in Heart Failure. Circulation. 2018; 138 (1): p.80-98. doi: 10.1161/circulationaha.118.030099 . | Open in Read by QxMD
  11. Yancy CW, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA Guideline for the Management of Heart Failure. J Am Coll Cardiol. 2013; 62 (16): p.e147-e239. doi: 10.1016/j.jacc.2013.05.019 . | Open in Read by QxMD
  12. Finocchiaro G, Merlo M, Sheikh N, et al. The electrocardiogram in the diagnosis and management of patients with dilated cardiomyopathy. Eur J Heart Fail. 2020; 22 (7): p.1097-1107. doi: 10.1002/ejhf.1815 . | Open in Read by QxMD
  13. Bang V, Ganatra S, Shah SP, et al. Management of Patients With Giant Cell Myocarditis. J Am Coll Cardiol. 2021; 77 (8): p.1122-1134. doi: 10.1016/j.jacc.2020.11.074 . | Open in Read by QxMD
  14. Maddox TM, Januzzi JL, Allen LA, et al. 2021 Update to the 2017 ACC Expert Consensus Decision Pathway for Optimization of Heart Failure Treatment: Answers to 10 Pivotal Issues About Heart Failure With Reduced Ejection Fraction. J Am Coll Cardiol. 2021; 77 (6): p.772-810. doi: 10.1016/j.jacc.2020.11.022 . | Open in Read by QxMD
  15. Huizar JF, Ellenbogen KA, Tan AY, Kaszala K. Arrhythmia-Induced Cardiomyopathy. J Am Coll Cardiol. 2019; 73 (18): p.2328-2344. doi: 10.1016/j.jacc.2019.02.045 . | Open in Read by QxMD
  16. Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2020; 143 (5). doi: 10.1161/cir.0000000000000923 . | Open in Read by QxMD
  17. Larson EA, German DM, Shatzel J, DeLoughery TG. Anticoagulation in the cardiac patient: A concise review. Eur J Haematol. 2018; 102 (1): p.3-19. doi: 10.1111/ejh.13171 . | Open in Read by QxMD
  18. Verdonschot JAJ, Hazebroek MR, Ware JS, Prasad SK, Heymans SRB. Role of Targeted Therapy in Dilated Cardiomyopathy: The Challenging Road Toward a Personalized Approach. Am Heart J. 2019; 8 (11). doi: 10.1161/jaha.119.012514 . | Open in Read by QxMD
  19. Davies MJ, McKenna WJ. Dilated cardiomyopathy: an introduction to pathology and pathogenesis. Br Heart J. 1994; 72 (6 Suppl): p.S24-S24. doi: 10.1136/hrt.72.6_suppl.s24 . | Open in Read by QxMD
  20. Grewal J, Siu SC, Ross HJ, et al. Pregnancy Outcomes in Women With Dilated Cardiomyopathy. J Am Coll Cardiol. 2009; 55 (1): p.45-52. doi: 10.1016/j.jacc.2009.08.036 . | Open in Read by QxMD
  21. Babitt JL, Lin HY. Mechanisms of Anemia in CKD. Journal of the American Society of Nephrology. 2012; 23 (10): p.1631-1634. doi: 10.1681/asn.2011111078 . | Open in Read by QxMD
  22. Reichart D, Magnussen C, Zeller T, Blankenberg S. Dilated cardiomyopathy: from epidemiologic to genetic phenotypes. J Intern Med. 2019; 286 (4): p.362-372. doi: 10.1111/joim.12944 . | Open in Read by QxMD
  23. Wexler RK, Elton T, Pleister A, Feldman D. Cardiomyopathy: an overview. Am Fam Physician. 2009; 79 (9): p.778-84.
  24. Fairweather D, Cooper LT, Blauwet LA. Sex and Gender Differences in Myocarditis and Dilated Cardiomyopathy. Curr Probl Cardiol. 2013; 38 (1): p.7-46. doi: 10.1016/j.cpcardiol.2012.07.003 . | Open in Read by QxMD
  25. Lakdawala NK, Winterfield JR, Funke BH. Dilated Cardiomyopathy. Circulation: Arrhythmia and Electrophysiology. 2013; 6 (1): p.228-237. doi: 10.1161/circep.111.962050 . | Open in Read by QxMD
  26. Donnellan E, Phelan D, McCarthy CP, Collier P, Desai M, Griffin B. Radiation-induced heart disease: A practical guide to diagnosis and management. Cleve Clin J Med. 2016; 83 (12): p.914-922. doi: 10.3949/ccjm.83a.15104 . | Open in Read by QxMD

3 free articles remaining

You have 3 free member-only articles left this month. Sign up and get unlimited access.
 Evidence-based content, created and peer-reviewed by physicians. Read the disclaimer