Cardiology Letters 2025, 34(1):41-49
Restrictive cardiomyopathy - an overview according to the latest guidelines
- Fakultná nemocnica s polikliniku J. A. Reimana, Prešov, Slovenská republika
Restrictive cardiomyopathy is still considered to be the least common form of cardiomyopathy in daily clinical practice. Various myocardial diseases are included, characterized by noncompliant, stiffened ventricular walls that resist diastolic filling, and leading to heart failure. In developed countries the most common etiology of restrictive cardiomyopathy is cardiac amyloidosis, cardiac sarcoidosis, cardiomyopathy developed in hemochromatosis, hypereosinophilia and radiation induced heart disease. Diagnosis of restrictive cardiomyopathy should include the patient´s anamnesis, ecg and echocardiography, laboratory tests, and cardiac magnetic resonance imaging. Sometimes it is necessary to continue with invasive hemodynamic examination and endomyocardial biopsy. The speckle-tracking echocardiography method is important because of the specific patterns in some types of restrictive cardiomyopathy. Therapy in restrictive cardiomyopathy is divided into the therapy of cardiac failure and specific therapy, differing in etiology of the restrictive cardiomyopathy.
Keywords: restrictive cardiomyopathy; cardiac amyloidosis; cardiac sarcoidosis; hemochromatosis; hypereosinophilia; echocardiography
Published: January 1, 2025 Show citation
References
- Arbelo E, Protonotarios A, Gimeno JR, Arbustini E, Barriales-Villa R, Basso C, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44: 3503-3626, doi: 10.1093/eurheartj/ehad194.
Go to original source... - Vojáček J, Kettner J, Dušek J. Klinická kardiologie. Piate vydanie. Praha: Maxdorf s.r.o.; 2022:1323.
- Táborský M, Kautzner J, Linhart A, Hatala R, Goncalvesová E, Hlivák P, et al. Kardiologie I. Praha: Mladá fronta a.s.; 2018:712.
- Habib G, Bucciarelli-Ducci Ch, Caforio LPA, Cardim N, Charron P, Cosyns B, 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;18:1090-1121, doi: 10.1093/ehjci/jex034.
Go to original source... - Goliash G, BInder T. Echofacts. Wien: Karin Dreher; 2014:252.
- Pereira NL, Grogan M, Dec GW. Spectrum of Restrictive and Infiltrative Cardiomyopathies: Part 1 of a 2-Part Series. J Am Coll Cardiol. 2018;71:1130-1148, doi: 10.1016/j.jacc.2018.01.016.
Go to original source... - Pieroni M, Moon J, Arbustini E, Barriales-Villa RB, Camporeale A, Vujkovac AC, et al. Cardiac Involvement in Fabry Disease. J Am Coll Cardiol. 2021;77:922-936, doi: 10.1016/j.jacc.2020.12.024.
Go to original source... - Yim J, Yau O, Yeung DF, Tsang TSM. Fabry Cardiomyopathy: Current Practice and Future Directions. Cells. 2021;10:1532, doi
Go to original source... - 3390/cells10061532. 9. Linhart A, Arad M, Elliott PM. Diagnosis and Management of Cardiac Manifestations in Anderson Fabry Disease and Glycogen Storage Diseases. In: ESC myocardial and pericardial work group. [online]. 2020;22:1076-1096 [cit.2024-06-09]. Dostupné na: <https://www.escardio.org/staticfile/Escardio/Subspecialty/Working%20Groups/Myocardial%20and%20Pericardial%20Diseases/y.%20Documents/BookletWG-Diseases-Fabry&Glycogen.pdf>.
Go to original source... - Germain DP, Linhart A. Pegunigalsidase alfa: a novel, pegylated recombinant alpha - galactosidase enzyme for the treatment of Fabry disease. Front Genet. 2024;15:1395287, doi: 10.3389/fgene.2024.1395287.
Go to original source... - Behl C. Breaking BAG: The Co-Chaperone BAG3 in Health and Disease. Trends Pharmacol Sci. 2016;37:672-688, doi: 10.1016/j.tips.2016.04.007.
Go to original source... - Aronow WS. Management of cardiac hemochromatosis. Arch Med Sci. 2018;14:560-568, doi: 10.5114/aoms.2017.68729.
Go to original source... - Kremastinos DT, Farmakis D. Iron overload cardiomyopathy in clinical practice. Circulation. 2011;124:2253-2263, doi: 10.1161/CIRCULATIONAHA.111.050773.
Go to original source... - Cheong B, Huber S, Muthupillai R, Flamm DS. Evaluation of myocardial iron overload by T2* cardiovascular magnetic resonance imaging. Texas Heart Institute Journal. 2005;32:448449, PMID: 16392242.
- Klion AD. Approach to the patient with suspected hypereosinophilic syndrome. Hematology Am Soc Hematol Educ Program. 2022;2022:47-54, doi: 10.1182/hematology.2022000367.
Go to original source... - Brener MI, Ravalli S. Left ventricular Cavity Obliteration From eosinophilic myocarditis in a Patient With Classic Hodgkin Lymphoma. JACC Case Rep. 2020;2:210-215, doi: 10.1016/j.jaccas.2019.10.046.
Go to original source... - Benezet-Mazuecos J, De La Fuente A, Marcos-Alberca P, Farre J. Loeffler endocarditis: what have we learned? Am J Hematol. 2007;82:861-862. Doi: 10.1002/ajh.20957.
Go to original source... - Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva JM, Sekijima Y, et al. Amyloid nomenclature recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2018;25:215-219, doi: 10.1080/13506129.2018.1549825.
Go to original source... - Papathanasiou M, Carpinteiro A, Rischpler C, Hagenacker T, Rassaf T, Luedike P. Diagnosing cardiac amyloidosis in every-day practice: A practical guide for the cardiologist. Int J Cardiol Heart Vasc. 2020;28:100519, doi: 10.1016/j.ijcha.2020.100519.
Go to original source... - Tanskanen M, Peuralinna T, Polvikoski T, Notkola I-L, Sulkava R, Hardy J, et al. Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2-macroglobulin and tau: a populationbased autopsy study. Ann Med. 2008;40:232-239, doi: 10.1080/07853890701842988.
Go to original source... - Garcia-Pavia P, Rapezzi C, Adler Y, Arad M, Basso C, Brucato A, et al. Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2021;42:1554-1568, doi: 10.1093/eurheartj/ehab072.
Go to original source... - Maurer MS, Fontana M, Berk JL, Gustafsson F, Simoes M, Grogan M, et al. Primary Results from APOLLO-B, A Phase 3 Study of Patisiran in Patients with Transthyretin-Mediated Amyloidosis with Cardiomyopathy. J CARD FAIL. 2023;389:1553-1565, doi: org/10.1016/j.cardfail.2022.10.013.
Go to original source... - Cheng RK, Kittleson MM, Beavers CJ, Birnie DH, Blankstein R, Bravo PE, et al. Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association Diagnosis and Management of Cardiac Sarcoidosis: A Scientific Statement From the American Heart Association. Circulation. 2024;149:1197-1216, doi: org./10.1161/CIR.00000000000012.
Go to original source... - Rojulpote C, Bhattaru A, Jean C, Adams SL, Patel V, Vidula MK, et al. Effect of immunosuppressive therapy and biopsy status in monitoring therapy response in suspected cardiac sarcoidosis. JACC Cardiovasc Imaging. 2022;15:1944-1955, doi: 10.1016/j.jcmg.2022.05.015.
Go to original source... - Wang H, Wei J, Zheng Q, Meng L, Xin Y, Yin X, et al. Radiationinduced heart disease: a review of classification, mechanism and prevention. Int J Biol Sci. 2019;15 2128-2138, doi: 10.7150/ijbs.35460.
Go to original source... - Belzile-Dugas E, Eisenberg MJ. Radiation-Induced Cardiovascular Disease: Review of an Underrecognized Pathology. J Am Heart Assoc. 2021;10:e021686. Doi: 10.1161/JAHA.121.021686.
Go to original source...

