MEDIX, God. 26 Br. 144/145  •  Sponzorirani članak  •  Kardiologija HR ENG

Amiloidna transtiretinska kardiomiopatijaAmyloid transthyretin cardiomyopathy

Ivo Planinc

Amiloidoza je bolest nakupljanja, koja može zahvatiti različite organske sustave, no najlošije ishode imaju bolesnici s amiloidnom kardiomiopatijom. U transtiretinskoj amiloidozi nakuplja se tetramer transtiretin zbog mutacije gena ili kao posljedica starenja organizma. Nakupljanje transtiretina u srcu dovodi do zadebljanja stijenki miokarda, poremećaja provodnoga sustava te razvoja simptoma i znakova popuštanja srca. Posljednjih je godina sve veća pouzdanost i točnost neinvazivnih slikovnih metoda u dijagnosticiranju amiloidne transtiretinske kardiomiopatije, što znatno olakšava i ubrzava dijagnostički postupak. Velik je napredak učinjen i u liječenju bolesnika, razvojem lijekova koji utječu na ishode bolesnika s amiloidnom transtiretinskom kardiomiopatijom.

Ključne riječi:
amiloidoza; kardiomiopatija; popuštanje srca; scintigrafija kostiju; tafamidis

Članak u cijelosti pročitajte u tiskanom izdanju MEDIX, God. 26 Br. 144/145

Amyloidosis is an infiltrative disease affecting different organ systems, with worst outcomes in patients with amyloid cardiomyopathy. Transthyretin amyloidosis is characterized by deposition of transthyretin tetramer as a result of gene mutation or ageing process. Transthyretin deposits in the heart result in severe thickening of myocardium, conduction tissue disturbances, and lead to symptoms and signs of heart failure. Recently, the accuracy and efficacy of non-invasive imaging methods in diagnosis of transthyretin amyloid cardiomyopathy has been rising, allowing for easier and faster diagnosis. Great advancements have also been achieved in the development of disease-modifying agents for patients with transthyretin amyloid cardiomyopathy.

Key words:
amyloidosis; cardiomyopathy; heart failure; radionuclide imaging; tafamidis