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Circ Res. 2017 Sep 15;121(7):819-837. doi: 10.1161/CIRCRESAHA.117.310982.

Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy.

Author information

1
From the Division of Hematology (E.M., M.A.G.) and Department of Cardiovascular Medicine (L.A.B.), Mayo Clinic, Rochester, MN.
2
From the Division of Hematology (E.M., M.A.G.) and Department of Cardiovascular Medicine (L.A.B.), Mayo Clinic, Rochester, MN. gertz.morie@mayo.edu.

Abstract

Restrictive cardiomyopathy (RCM) is characterized by nondilated left or right ventricle with diastolic dysfunction. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Each of these 3 RCMs is challenging to diagnose, and recognition of each disease entity is frequently delayed. Clinical clues to promote recognition of cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis and imaging techniques used to facilitate diagnosis are discussed. Disease-specific therapies are reviewed. Early recognition remains a key barrier to improving survival in all RCMs.

KEYWORDS:

amyloidosis; cardiomyopathies; diagnosis; hemochromatosis; sarcoidosis

PMID:
28912185
DOI:
10.1161/CIRCRESAHA.117.310982
[Indexed for MEDLINE]

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