Last updated: January 26, 2023

Summarytoggle arrow icon

Cardiomyopathies are diseases of the muscle tissue of the heart. Types of cardiomyopathies include dilated (most common), hypertrophic, restrictive, and arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) primarily affects the right ventricle and is characterized by fibrofatty replacement of myocardium, which causes myocardial thinning and subsequent ventricular dilation. Although the hallmark finding is arrhythmia, symptoms are highly variable. Because management depends greatly on individual factors, such as the extent of the disease, there is no single best course of treatment. All patients should avoid strenuous exercise.

Arrhythmia-induced cardiomyopathy is a very rare type of cardiomyopathy. It is caused by long-standing arrhythmia and typically affects the left ventricle. Features include palpitations, syncope, and signs of arrhythmia on ECG. Progression to left heart failure is possible in severe cases. Treatment involves antiarrhythmics such as beta blockers for rhythmic control.

Dilated cardiomyopathy”, “Hypertrophic cardiomyopathy,” “Restrictive cardiomyopathy,” and “Peripartum cardiomyopathy” are described in their respective articles in more detail.

Overview of major types of cardiomyopathytoggle arrow icon

Differential diagnosis of major cardiomyopathies
Types Dilated cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive cardiomyopathy


Distinctive clinical features
Echocardiography LV cavity size
  • Significantly increased
  • Decreased
  • Decreased
  • Significantly decreased
  • Normal
  • Normal or increased
Wall thickness
  • Normal or decreased
  • Significantly increased
  • Usually increased
Additional findings
Other characteristics
  • Most common cardiomyopathy
  • Second most common cardiomyopathy
  • Most common cause of sudden heart failure in athletes and teenagers

Arrhythmogenic right ventricular cardiomyopathy (ARVC)toggle arrow icon


  • Most common in young adults (mean age at diagnosis: ∼ 30 years) [2]
  • Prevalence: 1:1,000–2,000 [3]



Clinical features

Diagnostics [2][3][5]


ARVC is diagnosed based on the AHA criteria which include the following features:

  1. Dysfunction and structural abnormalities of RV (can be revealed by echocardiography, MRI, or RV angiography)
  2. Histological characteristics (require myocardial biopsy)
  3. Abnormal repolarization (diagnosed with ECG)
  4. Depolarization/conduction abnormalities (diagnosed with ECG)
  5. Arrhythmias (diagnosed with ECG)
  6. Family history (confirmation of ARVC in a relative either by criteria, pathological examination in surgery or autopsy, or by genetic testing)


Management [5]

Unclassified cardiomyopathiestoggle arrow icon

Left ventricular noncompaction [7]

Arrhythmia-induced cardiomyopathy [8][9]

Referencestoggle arrow icon

  1. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: June 6, 2019. Accessed: July 8, 2019.
  2. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: June 6, 2019. Accessed: July 5, 2019.
  3. Arrhythmogenic Right Ventricular Dysplasia (ARVD). Updated: September 14, 2014. Accessed: July 8, 2019.
  4. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: June 6, 2019. Accessed: July 8, 2019.
  5. Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: Executive Summary. Circulation. 2018; 138 (13): p.e210–e271.doi: 10.1161/cir.0000000000000548 . | Open in Read by QxMD
  6. Arman Arghami, Joseph A. Dearani, Sameh M. Said, Patrick W. O’Leary, and Hartzell V. Schaff. Hypertrophic cardiomyopathy in children. Annals of Cardiothoracic Surgery. 2017.
  7. Connolly HM, Attenhofer-Jost CH. Isolated left ventricular noncompaction. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: September 12, 2016. Accessed: March 30, 2017.
  8. Tracy CM. Arrhythmia-induced cardiomyopathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: July 6, 2016. Accessed: February 20, 2017.
  9. Gopinathannair R, Etheridge SP, Marchlinski FE, Spinale FG, Lakkireddy D, Olshansky B. Arrhythmia-Induced Cardiomyopathies Mechanisms, Recognition, and Management. J Am Coll Cardiol. 2015; 66 (15): p.1714-1728.doi: 10.1016/j.jacc.2015.08.038 . | Open in Read by QxMD
  10. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  11. Agabegi SS, Agabegi ED. Step-Up To Medicine. Wolters Kluwer Health ; 2015
  12. Le T, Bhushan V, Chen V, King M. First Aid for the USMLE Step 2 CK. McGraw-Hill Education ; 2015
  13. Le T, Bhushan V, Bagga HS. First Aid for the USMLE Step 2 CK. McGraw-Hill Medical ; 2009
  14. Fischer C. Master the Boards USMLE Step 2 CK. Kaplan Publishing ; 2015
  15. 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): p.2733-2779.doi: 10.1093/eurheartj/ehu284 . | Open in Read by QxMD
  16. Maron MS. Patient education: Hypertrophic cardiomyopathy (Beyond the Basics). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: November 18, 2015. Accessed: February 20, 2017.
  17. Gersh BJ, Maron BJ, Bonow RO et al. 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 . J Thorac Cardiovasc Surg. 2011; 142 (6): p.e153-203.doi: 10.1016/j.jtcvs.2011.10.020 . | Open in Read by QxMD
  18. Hypertrophic Cardiomyopathy. Updated: July 6, 2016. Accessed: February 20, 2017.
  19. Cisneros S, Duarte R, Fernandez-perez GC, et al. Left ventricular apical diseases. Insights Imaging. 2011; 2 (4): p.471-482.doi: 10.1007/s13244-011-0091-6 . | Open in Read by QxMD
  20. Le T, Bhushan V,‎ Sochat M, Chavda Y, Zureick A. First Aid for the USMLE Step 1 2018. McGraw-Hill Medical ; 2017
  21. Van Diepen S, Katz JN, Albert NM, et al. Contemporary Management of Cardiogenic Shock: A Scientific Statement From the American Heart Association. Circulation. 2017; 136 (16).doi: 10.1161/cir.0000000000000525 . | Open in Read by QxMD
  22. Nishimura RA, Seggewiss H, Schaff HV. Hypertrophic Obstructive Cardiomyopathy. Circ Res. 2017; 121 (7): p.771-783.doi: 10.1161/circresaha.116.309348 . | Open in Read by QxMD
  23. Maron MS, Hauser TH, Dubrow E, et al. Right Ventricular Involvement in Hypertrophic Cardiomyopathy. Am J Cardiol. 2007; 100 (8): p.1293-1298.doi: 10.1016/j.amjcard.2007.05.061 . | Open in Read by QxMD
  24. Christian Prinz, Dr. med., Martin Farr, Dr. rer. nat., Detlef Hering, Dr. med., Dieter Horstkotte, Prof. Dr. med, and Lothar Faber, Prof. Dr. med.. The Diagnosis and Treatment of Hypertrophic Cardiomyopathy. Deutsches Ärzteblatt. 2011.
  25. Martin S. Maron, MD; Ethan J. Rowin, MD; Barry J. Maron, MD. How to Image Hypertrophic Cardiomyopathy. Circulation: Cardiovascular Imaging. 2017.
  26. Dr. Irena Peovska Mitevksa. Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. ESC Council for Cardiology Practice. 2015.
  27. Veselka J, Anavekar NS, Charron P. Hypertrophic obstructive cardiomyopathy. Lancet. 2017; 389 (10075): p.1253-1267.doi: 10.1016/s0140-6736(16)31321-6 . | Open in Read by QxMD
  28. Diagnosis of Hypertrophic Cardiomyopathy: What Every Cardiologist Needs to Know. Updated: February 27, 2020. Accessed: March 22, 2020.
  29. Lee PT, Dweck MR, Prasher S, et al. Left Ventricular Wall Thickness and the Presence of Asymmetric Hypertrophy in Healthy Young Army Recruits. Circulation: Cardiovascular Imaging. 2013; 6 (2): p.262-267.doi: 10.1161/circimaging.112.979294 . | Open in Read by QxMD
  30. Afonso LC, Bernal J, Bax JJ, Abraham TP. Echocardiography in Hypertrophic Cardiomyopathy. J Am Coll Cardiol Img. 2008; 1 (6): p.787-800.doi: 10.1016/j.jcmg.2008.09.002 . | Open in Read by QxMD
  31. Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. N Engl J Med. 2018; 379 (7): p.655-668.doi: 10.1056/nejmra1710575 . | Open in Read by QxMD
  32. Ammirati E, Contri R, Coppini R, Cecchi F, Frigerio M, Olivotto I. Pharmacological treatment of hypertrophic cardiomyopathy: current practice and novel perspectives. European Journal of Heart Failure. 2016; 18 (9): p.1106-1118.doi: 10.1002/ejhf.541 . | Open in Read by QxMD
  33. Sherrid MV, Barac I, McKenna WJ, et al. Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2005; 45 (8): p.1251-1258.doi: 10.1016/j.jacc.2005.01.012 . | Open in Read by QxMD
  34. Houston BA, Stevens GR. Hypertrophic cardiomyopathy: a review.. Clin Med Insights Cardiol. 2014; 8 (Suppl 1): p.53-65.doi: 10.4137/CMC.S15717 . | Open in Read by QxMD

Icon of a lock3 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