TY - JOUR
T1 - Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), previously known as senile cardiac amyloidosis
T2 - Clinical presentation, diagnosis, management and emerging therapies
AU - Halatchev, Ilia G.
AU - Zheng, Jingsheng
AU - Ou, Jiafu
N1 - Publisher Copyright:
© Journal of Thoracic Disease.
PY - 2018/3/1
Y1 - 2018/3/1
N2 - Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies.
AB - Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies.
KW - ATTRwt cardiac amyloidosis (ATTRwt-CA)
KW - Heart failure with preserved ejection fraction (HFpEF)
KW - Left ventricular hypertrophy (LVH)
KW - Senile amyloidosis
KW - Transthyretin
UR - http://www.scopus.com/inward/record.url?scp=85045236338&partnerID=8YFLogxK
U2 - 10.21037/jtd.2018.03.134
DO - 10.21037/jtd.2018.03.134
M3 - Review article
C2 - 29707360
AN - SCOPUS:85045236338
SN - 2072-1439
VL - 10
SP - 2034
EP - 2045
JO - Journal of Thoracic Disease
JF - Journal of Thoracic Disease
IS - 3
ER -