Trusted medical expertise in seconds.

Access 1,000+ clinical and preclinical articles. Find answers fast with the high-powered search feature and clinical tools.

Try free for 5 days
Evidence-based content, created and peer-reviewed by physicians. Read the disclaimer.

Restrictive cardiomyopathy

Last updated: January 6, 2022

Summarytoggle arrow icon

Restrictive cardiomyopathy (RCM) is a rare type of cardiomyopathy characterized by marked diastolic dysfunction, normal (or near-normal) systolic function, and normal ventricular volumes. RCM occurs as a result of myocardium distortion due to proliferation of abnormal tissue or the deposition of abnormal compounds. Many diseases, both primary and acquired, cause RCM. Regardless of the etiology, RCM manifests with congestive heart failure (CHF), often with pronounced right-sided symptoms. Echocardiography is the first-line diagnostic test and is often followed by advanced imaging, including cardiac MRI. Initial management focuses on the treatment of CHF followed by identification and treatment of the underlying cause. In severe or refractory cases, heart transplantation may be necessary.

See also “Cardiomyopathy” for other cardiomyopathy types.

Epidemiological data refers to the US, unless otherwise specified.

RCM can be classified into four categories based on the underlying pathology; there is some overlap between the categories.

PLEASe Help!”: Cauases of restrictive cardiomyopathy include Postradiation/Postsurgery fibrosis, Löffler endocarditis, Endocardial fibroelastosis, Amyloidosis, Sarcoidosis, and Hemochromatosis.

Infiltrative cardiomyopathy [1][2][5]

Amyloidosis is the most common cause of restrictive cardiomyopathy in high-income countries. [6]

Storage disorders [1][2]

Some storage disorders, e.g., Gaucher disease, can also cause infiltrative cardiomyopathy. [1]

Endomyocardial disorders [5]

Noninfiltrative restrictive cardiomyopathy

Symptoms [1][4][5]

Physical examination [1]

The symptoms and signs of heart failure are the most common clinical features of RCM. [1]

Approach

Initial studies [1]

Echocardiography is the preferred first-line test for RCM because of its high sensitivity and specificity for this disease. [1][15]

Additional diagnostic studies

Assessment for associated disease states

Screening studies for the suspected underlying cause

Advanced studies for RCM

Approach

  • Start symptomatic treatment of heart failure, including education on lifestyle modification.
  • Screen for and treat associated diseases (e.g., arrhythmias, thromboembolism).
  • Treat the underlying cause, when possible.
  • Patients with severe or refractory symptoms:
    • Consider heart transplant.
    • If the patient is not a suitable candidate for transplant, refer early to palliative care, as symptoms can be challenging to manage.

RCM is the most challenging form of cardiomyopathy to manage because treatment options are limited and morbidity and mortality are high. [20]

Symptom management

Treatment of congestive heart failure [2][21]

Heart failure management in RCM [1][2][4]
Medication Treatment mechanism Additional considerations specific to RCM
Diuretics
  • First-line treatment for heart failure secondary to RCM [2]
  • Slowly decrease fluid overload with gentle diuresis and sodium restriction [1]
  • Avoid rapid and/or high volume diuresis: may cause hypotension. [2][4]
Beta blockers and calcium channel blockers (CCBs)
  • Introduce slowly: Profound hypotension may occur. [4]
Angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs)

Start at very low doses when prescribing beta blockers, CCBs, ACEIs, or ARBs for patients with RCM; life-threatening hypotension may occur.

Management of associated arrhythmias [1][5]

Digoxin should be avoided in patients with RCM because it can worsen symptoms of several of the underlying etiologies (e.g., amyloidosis, sarcoidosis). [2]

Anticoagulation

Disease-specific treatment

Definitive treatment depends on the specific etiology (see “Etiology” section).

Disease-specific treatment of RCM [1][4]]#19367][2]
Etiology Treatment
Infiltrative Amyloidosis: lIght chain
Amyloidosis: transthyretin amyloidosis (ATTR)
Sarcoidosis
Storage disorder Hereditary hemochromatosis
Iron overload
Endomyocardial disorder Hypereosinophilic syndrome
Radiotherapy
Noninfiltrative Systemic sclerosis

Management of severe or refractory RCM

  • RCM and constrictive pericarditis have similar clinical presentations. Distinguishing between the two diseases is critical to determining the appropriate management.
  • See also “Cardiomyopathy” article.

Echocardiography is the initial test of choice to distinguish RCM from constrictive pericarditis. [1]

Restrictive cardiomyopathy versus constrictive pericarditis [1][12][26]
RCM Constrictive pericarditis
Clinical presentation
Auscultation
X-ray chest
  • Atrial enlargement
Echocardiography
  • Decreased respiratory variation in Doppler flow
  • Ventricular septum shift with respiration [12]
  • Thickened pericardium may be seen.
CT or MRI chest
Cardiac catheterization
  • LVEDP similar to RVEDP
  • Normal RVSP

The differential diagnoses listed here are not exhaustive.

  • The prognosis for RCM is generally poor. [4]
  • Adult patients: The overall 5-year survival rate is approximately 50%.
  • Pediatric patients: over 50% experience sudden cardiac death shortly after diagnosis
  1. Pereira NL, Grogan M, Dec GW. Spectrum of Restrictive and Infiltrative Cardiomyopathies. J Am Coll Cardiol. 2018; 71 (10): p.1130-1148. doi: 10.1016/j.jacc.2018.01.016 . | Open in Read by QxMD
  2. Muchtar E, Blauwet LA, Gertz MA. Restrictive Cardiomyopathy. Circ Res. 2017; 121 (7): p.819-837. doi: 10.1161/circresaha.117.310982 . | Open in Read by QxMD
  3. Brieler J, Breeden MA, Tucker J. Cardiomyopathy: An Overview.. Am Fam Physician. 2017; 96 (10): p.640-646.
  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. Pereira NL, Grogan M, Dec GW. Spectrum of Restrictive and Infiltrative Cardiomyopathies. J Am Coll Cardiol. 2018; 71 (10): p.1149-1166. doi: 10.1016/j.jacc.2018.01.017 . | Open in Read by QxMD
  6. Nihoyannopoulos P, Dawson D. Restrictive cardiomyopathies. Eur J Echocardiograph. 2009; 10 (8): p.iii23-iii33. doi: 10.1093/ejechocard/jep156 . | Open in Read by QxMD
  7. 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
  8. Steger CM, Antretter H, Moser PL. Endocardial fibroelastosis of the heart. Lancet. 2012; 379 (9819): p.932. doi: 10.1016/s0140-6736(11)61418-9 . | Open in Read by QxMD
  9. Perez IE, Taveras Alam S, Hernandez GA, Sancassani R. Cancer Therapy-Related Cardiac Dysfunction: An Overview for the Clinician. Clin Med Insights Cardiol. 2019; 13 : p.117954681986644. doi: 10.1177/1179546819866445 . | Open in Read by QxMD
  10. Cyrille NB, Goldsmith J, Alvarez J, Maurer MS. Prevalence and Prognostic Significance of Low QRS Voltage Among the Three Main Types of Cardiac Amyloidosis. Am J Cardiol. 2014; 114 (7): p.1089-1093. doi: 10.1016/j.amjcard.2014.07.026 . | Open in Read by QxMD
  11. Burke MA, Cotts WG. Interpretation of B-type natriuretic peptide in cardiac disease and other comorbid conditions. Heart Fail Rev. 2007; 12 (1): p.23-36. doi: 10.1007/s10741-007-9002-9 . | Open in Read by QxMD
  12. Rammos A, Meladinis V, Vovas G, Patsouras D. Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment—A Systematic Review. Radiol Res Pract. 2017; 2017 : p.1-14. doi: 10.1155/2017/2874902 . | Open in Read by QxMD
  13. Nagueh SF. Diastology: 2020‐A practical guide. Echocardiography. 2020; 37 (11): p.1919-1925. doi: 10.1111/echo.14742 . | Open in Read by QxMD
  14. Wood MJ. Utility of echocardiography in the evaluation of individuals with cardiomyopathy. Heart. 2004; 90 (6): p.707-712. doi: 10.1136/hrt.2003.024778 . | Open in Read by QxMD
  15. Nagueh SF, Smiseth OA, Appleton CP, et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Journal of the American Society of Echocardiography. 2016; 29 (4): p.277-314. doi: 10.1016/j.echo.2016.01.011 . | Open in Read by QxMD
  16. Sekhri V, Sanal S, DeLorenzo LJ, Aronow WS, Maguire GP. Cardiac sarcoidosis: a comprehensive review. Arch Med Sci. 2011; 4 : p.546-554. doi: 10.5114/aoms.2011.24118 . | Open in Read by QxMD
  17. Galea N, Polizzi G, Gatti M, Cundari G, Figuera M, Faletti R. Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies. Radiol Med (Torino). 2020; 125 (11): p.1072-1086. doi: 10.1007/s11547-020-01287-8 . | Open in Read by QxMD
  18. Habib G, Bucciarelli-Ducci C, Caforio ALP, et al. Multimodality Imaging in Restrictive Cardiomyopathies: An EACVI expert consensus document In collaboration with the “Working Group on myocardial and pericardial diseases” of the European Society of Cardiology Endorsed by The Indian Academy of Echocardiography. Eur Heart J Cardiovasc Imaging. 2017; 18 (10): p.1090-1121. doi: 10.1093/ehjci/jex034 . | Open in Read by QxMD
  19. Bennett MK, Gilotra NA, Harrington C, et al. Evaluation of the Role of Endomyocardial Biopsy in 851 Patients With Unexplained Heart Failure From 2000–2009. Circ Heart Fail. 2013; 6 (4): p.676-684. doi: 10.1161/circheartfailure.112.000087 . | Open in Read by QxMD
  20. Artz G, Wynne J. Restrictive cardiomyopathy. Curr Treat Options Cardiovasc Med. 2000; 2 (5): p.431-438. doi: 10.1007/s11936-000-0038-6 . | Open in Read by QxMD
  21. Yancy CW, Jessup M, Bozkurt B, et al. 2017 ACC/AHA/HFSA Focused Update of the 2013 ACCF/AHA Guideline for the Management of Heart Failure. J Am Coll Cardiol. 2017; 70 (6): p.776-803. doi: 10.1016/j.jacc.2017.04.025 . | Open in Read by QxMD
  22. Aziz. Diastolic Heart Failure: A Concise Review. J Clin Med Res. 2013 . doi: 10.4021/jocmr1532w . | Open in Read by QxMD
  23. Brittenham GM. Iron-Chelating Therapy for Transfusional Iron Overload. N Engl J Med. 2011; 364 (2): p.146-156. doi: 10.1056/nejmct1004810 . | Open in Read by QxMD
  24. Lambova S. Cardiac manifestations in systemic sclerosis. World J Cardiol. 2014; 6 (9): p.993. doi: 10.4330/wjc.v6.i9.993 . | Open in Read by QxMD
  25. Grupper A, Park SJ, Pereira NL, et al. Role of ventricular assist therapy for patients with heart failure and restrictive physiology: Improving outcomes for a lethal disease. J Heart Lung Transplant. 2015; 34 (8): p.1042-1049. doi: 10.1016/j.healun.2015.03.012 . | Open in Read by QxMD
  26. Garcia MJ. Constrictive Pericarditis Versus Restrictive Cardiomyopathy?. J Am Coll Cardiol. 2016; 67 (17): p.2061-2076. doi: 10.1016/j.jacc.2016.01.076 . | Open in Read by QxMD