Žaneta Petrulionienė, Urtė Gargalskaitė, Agnė Skujaitė, Agnė Urbonienė, Karolina Lušaitė, Darius Palionis, Nomeda Valevičienė
Abstract
Amyloidosis – group of rare systemic diseases characterizedby abnormal protein structure formation and the formationof fibrils. Amyloidosis is caused by a large amount of amyloidprecursor in the body, the incomplete degradation of amyloidprecursor in the macrophage system and certain tissue characteristics.Although amyloidosis causes systemic changes, degree ofamyloid deposition varies in different organs and leads to differentclinical manifestations of amyloidosis. Cardiac amyloidosisis a clinical manifestation of the accumulation of amyloid in theheart. Main pathogenic element of cardiac amyloidosis is interstitialinfiltration of amyloid causing infiltrative cardiomyopathythat progresses to restrictive cardiomyopathy and heart failure.Heart damage in case of amyloidosis leads to extremely poor prognosisand high mortality rate, therefore it is very important toensure early diagnosis which would prevent amyloid depositionin various organs. In addition, cardiac amyloidosis is often diagnosedat an advanced process stage, because there is no specificclinical disease manifestation and this is why instrumental testsconfirming the diagnosis are carried out too late. The main treatmentof amyloidosis is directed to stopping infiltrative processwith intent to block amyloid manifestation in various organs aswell as treating complications which relieves the symptoms andimproves overall patient condition. This review aims to discusspathogenesis, manifestation and complex diagnostics of amyloidosiswith the main focus on amyloid cardiomyopathy illustratedby the clinical case of the disease.
Keyword(s): cardiac amyloidosis, cardiomyopathy, heart magnetic resonance imaging
DOI: 10.5200/sm-hs.2014.127
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