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Cardiomyopathy

Last updated: January 20, 2021

Summary

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

Dilated cardiomyopathy (DCM) is the most common type of cardiomyopathy. Although most cases are idiopathic, a number of conditions (e.g., coronary artery disease, wet beriberi), infections (e.g., Coxsackie B virus, Chagas disease), and substances (e.g., heavy drinking, cocaine) have been identified as causes. In DCM, the decreased ventricular contractility of the dilated left ventricle (LV) leads to failure of the left and eventually right heart with decreased ventricular output. Isolated dilation and subsequent decrease in contractility of right ventricle (RV) is rare. Dilation can be seen on echocardiography, the most important diagnostic tool for all cardiomyopathies. Therapy involves management of congestive heart failure and treatment of the underlying condition.

Restrictive cardiomyopathy (RCM) is caused by the proliferation of connective tissue, with subsequent atrial enlargement (but normal ventricles). Like DCM, RCM causes left and right heart failure. The ejection fraction is usually normal, but diastolic filling is reduced on echocardiography. While a number of drugs offer symptomatic relief, the overall prognosis remains poor.

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.

See also “Hypertrophic cardiomyopathy” and “Peripartum cardiomyopathy.”

Overview of major types of cardiomyopathy

Dilated cardiomyopathy

Hypertrophic cardiomyopathy

 Restrictive cardiomyopathy
Etiology

Pathophysiology
Distinctive clinical features
Echocardiography LV cavity size
  • Significantly increased
  • Decreased
  • Decreased
EF
  • 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

Dilated cardiomyopathy

Epidemiology

  • Incidence: 6/100,000 per year (most common cardiomyopathy)
  • Sex: ♂ > ♀ (approx. 3:1)

Etiology [2]

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

Pathophysiology

Clinical features

Diagnostics

Approach

  • Diagnostic approach to DCM aims to:
    1. Investigate the underlying cause with confirming either the secondary cause or idiopathic disease
    2. Assess cardiac function
    3. Assess structural remodeling
  • Specific investigations are guided by suspected underlying cause or complications

Investigations [4]

Pathology

Treatment

Complications

Restrictive cardiomyopathy

Epidemiology

  • Least common cardiomyopathy (accounts for ∼ 5% of all cardiomyopathies) [6]

Etiology [2]

Remember the etiology of restrictive cardiomyopathy with “PLEASe Help!”: Postradiation/Postsurgery fibrosis, Löffler endocarditis, Endocardial fibroelastosis, Amyloidosis, Sarcoidosis, Hemochromatosis)

Pathophysiology

Clinical features

Diagnostics [12]

Treatment [12]

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

Epidemiology

  • Most common in young adults (mean age at diagnosis: ∼ 30 years) [14]
  • Prevalence: 1:1,000–2,000 [15]

Etiology

Pathophysiology

Clinical features

Diagnostics [14][15][17]

Approach

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)

Findings

Management [17]

Unclassified cardiomyopathies

Left ventricular noncompaction [19]

Arrhythmia-induced cardiomyopathy [20][21]

References

  1. Cooper LT. Definition and classification of the cardiomyopathies. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/definition-and-classification-of-the-cardiomyopathies?source=search_result&search=dilated%20cardiomyopathy&selectedTitle=4~150#H6.Last updated: June 12, 2015. Accessed: February 20, 2017.
  2. Sisakian H. Cardiomyopathies: Evolution of pathogenesis concepts and potential for new therapies. World Journal of Cardiology. 2014; 6 (6): p.478. doi: 10.4330/wjc.v6.i6.478 . | Open in Read by QxMD
  3. 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
  4. Colucci WS, Chen HH. Natriuretic peptide measurement in heart failure. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/natriuretic-peptide-measurement-in-heart-failure.Last updated: March 14, 2016. Accessed: February 20, 2017.
  5. Kristen N. Brown; Venkata Satish Pendela; Rene R. Diaz.. Restrictive (Infiltrative) Cardiomyopathy. StatPearls. 2020 .
  6. Amit Alam, Shankar Thampi, Shahryar G Saba, and Rita Jermyn. Loeffler Endocarditis: A Unique Presentation of Right-Sided Heart Failure Due to Eosinophil-Induced Endomyocardial Fibrosis. Clinical Medicine Insights: Case Reports. 2017 .
  7. Endocardial fibroelastosis. http://www.pathologyoutlines.com/topic/hearttumorendocardialfibroelastosis.html. Updated: November 1, 2013. Accessed: July 5, 2019.
  8. Mocumbi AO. Endomyocardial fibrosis: A form of endemic restrictive cardiomyopathy. Global Cardiology Science and Practice. 2012; 2012 (1): p.11. doi: 10.5339/gcsp.2012.11 . | Open in Read by QxMD
  9. Ana Olga Mocumbi. Endomyocardial fibrosis: A form of endemic restrictive cardiomyopathy. Global Cardiology Science and Practice. 2012 .
  10. Antonio Grimaldi, Ana Olga Mocumbi, Juergen Freers, Matthias Lachaud, Mariana Mirabel, Beatriz Ferreira, Kumar Narayanan, David S. Celermajer, Daniel Sidi, Xavier Jouven, and Eloi Marijon. Tropical Endomyocardial Fibrosis. AHA Journals. 2016 .
  11. Ammash NM, Tajik AJ. Idiopathic restrictive cardiomyopathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/idiopathic-restrictive-cardiomyopathy?source=search_result&search=restrictive%20cardiomyopathy&selectedTitle=1~82.Last updated: February 16, 2016. Accessed: February 20, 2017.
  12. Restrictive Cardiomyopathy. https://www.msdmanuals.com/professional/cardiovascular-disorders/cardiomyopathies/restrictive-cardiomyopathy. Updated: December 1, 2018. Accessed: July 5, 2019.
  13. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/arrhythmogenic-right-ventricular-cardiomyopathy-anatomy-histology-and-clinical-manifestations.Last updated: June 6, 2019. Accessed: July 8, 2019.
  14. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/arrhythmogenic-right-ventricular-cardiomyopathy-pathogenesis-and-genetics.Last updated: June 6, 2019. Accessed: July 5, 2019.
  15. Arrhythmogenic Right Ventricular Dysplasia (ARVD). https://emedicine.medscape.com/article/163856-overview#a4. Updated: September 14, 2014. Accessed: July 8, 2019.
  16. William J McKenna, MD. Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/arrhythmogenic-right-ventricular-cardiomyopathy-treatment-and-prognosis.Last updated: June 6, 2019. Accessed: July 8, 2019.
  17. 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
  18. 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 .
  19. Connolly HM, Attenhofer-Jost CH. Isolated left ventricular noncompaction. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/isolated-left-ventricular-noncompaction.Last updated: September 12, 2016. Accessed: March 30, 2017.
  20. Tracy CM. Arrhythmia-induced cardiomyopathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/arrhythmia-induced-cardiomyopathy.Last updated: July 6, 2016. Accessed: February 20, 2017.
  21. 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
  22. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  23. Agabegi SS, Agabegi ED. Step-Up To Medicine. Wolters Kluwer Health ; 2015
  24. Le T, Bhushan V, Chen V, King M. First Aid for the USMLE Step 2 CK. McGraw-Hill Education ; 2015
  25. Le T, Bhushan V, Bagga HS. First Aid for the USMLE Step 2 CK. McGraw-Hill Medical ; 2009
  26. Fischer C. Master the Boards USMLE Step 2 CK. Kaplan Publishing ; 2015
  27. Maron MS. Hypertrophic cardiomyopathy: Clinical manifestations, diagnosis, and evaluation. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-clinical-manifestations-diagnosis-and-evaluation?source=search_result&search=hypertrophic+cardiomyopathy&selectedTitle=1~150#H17.Last updated: December 15, 2016. Accessed: February 13, 2017.
  28. Nguyen VQ, Celebi MM, Suleman A, Sander GE. Dilated Cardiomyopathy. Dilated Cardiomyopathy. New York, NY: WebMD. http://emedicine.medscape.com/article/152696-overview. Updated: January 20, 2017. Accessed: February 20, 2017.
  29. 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
  30. Maron MS. Patient education: Hypertrophic cardiomyopathy (Beyond the Basics). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/hypertrophic-cardiomyopathy-beyond-the-basics.Last updated: November 18, 2015. Accessed: February 20, 2017.
  31. 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
  32. Shah SN. Hypertrophic Cardiomyopathy. Hypertrophic Cardiomyopathy. New York, NY: WebMD. http://emedicine.medscape.com/article/152913-overview#a4. Updated: January 5, 2016. Accessed: February 20, 2017.
  33. Maron MS. Hypertrophic cardiomyopathy: Gene mutations and clinical genetic testing. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/hypertrophic-cardiomyopathy-gene-mutations-and-clinical-genetic-testing?source=see_link.Last updated: December 14, 2016. Accessed: February 20, 2017.
  34. Hypertrophic Cardiomyopathy. http://www.heart.org/HEARTORG/Conditions/More/Cardiomyopathy/Hypertrophic-Cardiomyopathy_UCM_444317_Article.jsp#.WKreI7bhC8p. Updated: July 6, 2016. Accessed: February 20, 2017.
  35. Vainrib A, Viccellio AW, Goswami VJ. Restrictive Cardiomyopathy. Restrictive Cardiomyopathy. New York, NY: WebMD. http://emedicine.medscape.com/article/153062-overview. Updated: December 18, 2014. Accessed: February 20, 2017.
  36. Tomich EB. Takotsubo Cardiomyopathy. In: Schraga ED, Takotsubo Cardiomyopathy. New York, NY: WebMD. http://emedicine.medscape.com/article/1513631-overview. Updated: January 17, 2017. Accessed: March 30, 2017.
  37. Reeder GS, Prasad A. Clinical manifestations and diagnosis of stress (takotsubo) cardiomyopathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-stress-takotsubo-cardiomyopathy.Last updated: March 28, 2017. Accessed: March 30, 2017.
  38. Reeder GS, Prasad A. Management and prognosis of stress (takotsubo) cardiomyopathy. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/management-and-prognosis-of-stress-takotsubo-cardiomyopathy.Last updated: October 2, 2015. Accessed: March 30, 2017.
  39. Shavelle DM. Pathophysiology of stunned or hibernating myocardium. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/pathophysiology-of-stunned-or-hibernating-myocardium.Last updated: December 10, 2015. Accessed: March 30, 2017.
  40. 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
  41. Le T, Bhushan V,‎ Sochat M, Chavda Y, Zureick A. First Aid for the USMLE Step 1 2018. McGraw-Hill Medical ; 2017
  42. 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
  43. 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
  44. 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
  45. 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 .
  46. Martin S. Maron, MD; Ethan J. Rowin, MD; Barry J. Maron, MD. How to Image Hypertrophic Cardiomyopathy. Circulation: Cardiovascular Imaging. 2017 .
  47. Dr. Irena Peovska Mitevksa. Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. ESC Council for Cardiology Practice. 2015 .
  48. 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
  49. Diagnosis of Hypertrophic Cardiomyopathy: What Every Cardiologist Needs to Know. https://www.acc.org/latest-in-cardiology/articles/2020/02/25/06/34/diagnosis-of-hypertrophic-cardiomyopathy. Updated: February 27, 2020. Accessed: March 22, 2020.
  50. 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
  51. 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
  52. 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
  53. 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
  54. 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
  55. 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