Open AccessA review of the amyloidoses that infiltrate the heart
Author(s) -
MCCARTHY, III ROBERT E.,
Kasper EDWARD K.
Publication year - 1998
Publication title -
clinical cardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.263
H-Index - 72
eISSN - 1932-8737
pISSN - 0160-9289
DOI - 10.1002/clc.4960210804
Subject(s) - medicine , amyloidosis , cardiac amyloidosis , transthyretin , restrictive cardiomyopathy , heart failure , amyloid (mycology) , pathology , heart transplantation , amyloid fibril , infiltration (hvac) , primary systemic amyloidosis , cardiology , cardiomyopathy , systemic disease , disease , physics , amyloid β , thermodynamics
Primary amyloidosis, systemic senile amyloidosis, isolated atrial amyloidosis, and transthyretin isoleucine 122 amyloidosis frequently involve the heart. Amyloid fibrils infiltrate the myocardium, impairing ventricular contraction and relaxation. The clinical manifestations of cardiac infiltration in these disorders are protean, though congestive heart failure and arrhythmias are most common. Treatment of cardiac amyloidosis is directed at the underlying cause and at relief of symptoms. Heart transplantation is not a viable treatment option for patients with primary amyloidosis; its role in the other amyloidoses has not been established. The prognosis of patients with cardiac amyloidosis varies and is largely determined by the underlying disorder responsible for amyloid infiltration.