TY - JOUR
T1 - Restrictive cardiomyopathy
T2 - Genetics, pathogenesis, clinical manifestations, diagnosis, and therapy
AU - Muchtar, Eli
AU - Blauwet, Lori A.
AU - Gertz, Morie
PY - 2017/9/1
Y1 - 2017/9/1
N2 - 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.
AB - 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.
KW - Amyloidosis
KW - Cardiomyopathies
KW - Diagnosis
KW - Hemochromatosis
KW - Sarcoidosis
UR - http://www.scopus.com/inward/record.url?scp=85030620269&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85030620269&partnerID=8YFLogxK
U2 - 10.1161/CIRCRESAHA.117.310982
DO - 10.1161/CIRCRESAHA.117.310982
M3 - Article
C2 - 28912185
AN - SCOPUS:85030620269
VL - 121
SP - 819
EP - 837
JO - Circulation Research
JF - Circulation Research
SN - 0009-7330
IS - 7
ER -