- Restrictive cardiomyopathy (RCM) is characterized by a rigid heart with poor ventricular filling but generally a nondilated LV and normal LVEF. Right HF symptoms often predominate.
- RCM may be primary, including conditions such as idiopathic RCM, endomyocardial fibrosis, and Löeffler endocarditis, or secondary to either infiltrative conditions (amyloidosis, sarcoidosis, hypereosinophilic syndrome) or storage diseases (Fabry disease, hemochromatosis, and the glycogen storage diseases).
- Constrictive pericarditis may present similarly to RCM but is a disease wherein the pericardium limits diastolic filling. Constriction carries a different prognosis and therapy, and the distinction between constriction and RCM is essential.
- In amyloidosis, misfolded protein (amyloid) deposits in the cardiac interstitium, interrupting the normal myocardial contractile units and causing restriction. Most commonly, the misfolded protein is either AL or TTR.
- In sarcoidosis, granulomatous infiltration of the myocardium is often subclinical and more commonly presents with arrhythmias or conduction system disease; however, in up to 5% of sarcoidosis cases, restriction occurs.
- In hemochromatosis, excess iron is deposited in the cardiomyocyte sarcoplasm, ultimately overcoming antioxidant capacity and resulting in lipid peroxidation and membrane permeability. Injury occurs initially in the epicardium and then later in the myocardium and endocardium, with systolic function initially preserved.
- Fabry disease, an X-linked genetic disorder, is characterized by deficient activity of the lysosomal enzyme α-galactosidase A, resulting in lysosomal accumulation of globotriaosylceramide in tissues. More than half of patients develop cardiomyopathy, typically with LV hypertrophy and RCM.
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Bhat, Pavat, et al., editors. "Restrictive Cardiomyopathy." Washington Manual of Medical Therapeutics, 35th ed., Wolters Kluwer Health, 2016. The Washington Manual, www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602059/all/Restrictive_Cardiomyopathy.
Restrictive Cardiomyopathy. In: Bhat PP, Dretler AA, Gdowski MM, et al, eds. Washington Manual of Medical Therapeutics. Wolters Kluwer Health; 2016. https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602059/all/Restrictive_Cardiomyopathy. Accessed May 30, 2023.
Restrictive Cardiomyopathy. (2016). In Bhat, P., Dretler, A., Gdowski, M., Ramgopal, R., & Williams, D. (Eds.), Washington Manual of Medical Therapeutics (35th ed.). Wolters Kluwer Health. https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602059/all/Restrictive_Cardiomyopathy
Restrictive Cardiomyopathy [Internet]. In: Bhat PP, Dretler AA, Gdowski MM, Ramgopal RR, Williams DD, editors. Washington Manual of Medical Therapeutics. Wolters Kluwer Health; 2016. [cited 2023 May 30]. Available from: https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602059/all/Restrictive_Cardiomyopathy.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Restrictive Cardiomyopathy ID - 602059 ED - Williams,Dominique, ED - Bhat,Pavat, ED - Dretler,Alexandra, ED - Gdowski,Mark, ED - Ramgopal,Rajeev, BT - Washington Manual of Medical Therapeutics UR - https://www.unboundmedicine.com/washingtonmanual/view/Washington-Manual-of-Medical-Therapeutics/602059/all/Restrictive_Cardiomyopathy PB - Wolters Kluwer Health ET - 35 DB - The Washington Manual DP - Unbound Medicine ER -