Cor Vasa 2013, 55(1):e60-e75 | DOI: 10.1016/j.crvasa.2012.11.018

Cardiac amyloidosis: A comprehensive review

Michal Fikrle^a, Tomáš Paleček^a,b,*, Petr Kuchynka^a, Eduard Němeček^a, Lenka Bauerová^c, Jan Straub^d, Romana Ryšavá^e

^a II. interní klinika kardiologie a angiologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice, Praha, Česká republika

^b International Clinical Research Center, Lékařská fakulta Masarykovy univerzity a Fakultní nemocnice u sv. Anny, Brno, Česká republika

^c Ústav patologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice, Praha, Česká republika

^d I. interní klinika - klinika hematologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice, Praha, Česká republika

^e Klinika nefrologie, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice, Praha, Česká republika

Cardiac amyloidosis is characterized by clinically significant extracellular amyloid infiltration of the heart that is usually, but not always, associated with the involvement of other organs depending on the type of amyloid. Cardiac involvement represents the most important prognostic factor especially in AL amyloidosis and thus early diagnosis of amyloid heart disease is of utmost importance influencing further management of the patients. This review aims to broadly discuss pathogenesis, manifestation and complex diagnostics of amyloidosis with the main focus on amyloid cardiomyopathy. Also, the summary of current therapeutic options that have great potential to improve existing poor prognosis of affected individuals is given.

Keywords: Amyloidosis; Cardiomyopathy; Diagnosis; Heart failure; Imaging; Treatment

Received: November 6, 2012; Revised: November 23, 2012; Accepted: November 24, 2012; Published: February 1, 2013  Show citation