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Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology
Univ Belgrade, Serbia; Serbian Acad Arts and Sci, Serbia.
Univ Belgrade, Serbia; Clin Ctr Serbia, Serbia.
Med Sch Hannover, Germany.
Tel Aviv Univ, Israel.
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2019 (English)In: European Journal of Heart Failure, ISSN 1388-9842, E-ISSN 1879-0844, Vol. 21, no 5, p. 553-576Article in journal (Refereed) Published
Abstract [en]

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and similar to 10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

Place, publisher, year, edition, pages
WILEY , 2019. Vol. 21, no 5, p. 553-576
Keywords [en]
Heart failure; Dilated cardiomyopathy; Hypertrophic cardiomyopathy; Restrictive cardiomyopathy; Peripartum cardiomyopathy; Epidemiology; Natural history; Pathophysiology; Management
National Category
Cardiac and Cardiovascular Systems
Identifiers
URN: urn:nbn:se:liu:diva-158844DOI: 10.1002/ejhf.1461ISI: 000471307000006PubMedID: 30989768OAI: oai:DiVA.org:liu-158844DiVA, id: diva2:1337657
Available from: 2019-07-16 Created: 2019-07-16 Last updated: 2019-07-16

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