IJCS | Volume 32, Nº2, March/April 2019

DOI: 10.5935/2359-4802.20190005 177 REVIEW ARTICLE International Journal of Cardiovascular Sciences. 2019;32(2)177-189 Mailing Address: Marcelo Souto Nacif Departamento de Radiologia (MRD), Universidade Federal Fluminense - UFF, Hospital Universitária Antonio Pedro - HUAP, Rua Marques de Paraná, 303. Postal Code: 24033-900, 2º andar, Centro, Niterói, Rio de Janeiro - Brazil. E-mail: msnacif@gmail.com Cardiac Magnetic Resonance and amyloidosis: Review Vaneza Ferreira Ribeiro, 1 Diogo Costa Leandro de Oliveira, 1 D aniel Gama das Neves, 1, 2 Nágela Simão Vinhosa Nunes, 2 H umberto Villacorta Junior, 1 M arcelo Souto Nacif 1, 2 Universidade Federal Fluminense, 1 Niterói, RJ - Brazil Complexo Hospitalar de Niterói, 2 Niterói, RJ - Brazil Manuscript received December 20, 2017, revised manuscript March 27, 2018, accepted May 07, 2018. Amyloidosis;Myocarditis/pathology; Plaque,Amyloid/ diagnostic imaging; Magnetic Resonance Imaging. Keywords Abstract Amyloidosis is a disease caused by extracellular deposition of insoluble protein fibrils, that results in changes in tissue architecture and consequently modification of the organ structure. Cardiac involvement is common in amyloidosis. Two major types of systemic amyloidosis affect the myocardium – immunoglobulin light chain and transthyretin amyloidosis – each leading to different prognosis. Early detection and diagnosis of cardiac amyloidosis are the main objectives in the assessment of the disease. New techniques of magnetic resonance imaging have minimized the need for biopsies for the diagnosis. Late gadolinium enhancement technique, and more recently T1 mapping, have allowed a simplified evaluation of amyloid deposits and extracellular volume. The aim of this review was to describe basic concepts and updates of the use of magnetic resonance imaging for the diagnosis amyloidosis and evaluation of its severity. Introduction Amyloidosis is a group of diseases characterized by deposition of insoluble fibrils formed from soluble molecules that had suffered structural changes and become relatively insoluble. 1 Amyloid fibrils deposit either locally or systemically. Symptoms vary according to the content of amyloid deposits, which is directly related to the type of protein. Cardiac involvement is determinant for the choice of the therapy. 2 Immunoglobulin light chain amyloidosis is the most common form of amyloidosis in the USA, with 4,500 new cases diagnosed per year. It affects mostly older individuals, aged between 50 and 80 years, especially men (who account for one third of the cases). In the USA, wild-type transthyretin amyloidosis is more frequently diagnosed in older, African American patients, whereas patients with hereditary systemic amyloidosis are generally younger white women. Survival of transthyretin amyloidosis patients is higher than that of light-chain amyloidosis patients. 3 Several non-invasivemethodshavebeenused topredict the presence of amyloid deposits in myocardial tissue, including electrocardiogram (ECG), echocardiogram (ECHO), cardiac biomarkers, scintigraphy (SPECT) and cardiac magnetic resonance (CMR) imaging. Among these techniques, CMR has been considered the reference standard for assessment of global and regionalmyocardial function and for detection and quantification of fibrosis areas and expansion of myocardial extracellular volume. 4 Cardiac involvement is decisive for the prognosis and treatment of systemic amyloidosis. Although ECG, ECHO and scintigraphy are the main imaging tests used in cardiology, CMR can provide a new perspective, especially on the analysis of amyloid deposits. The use of the late gadolinium enhancement technique reveals more specific, and sometimes pathognomonic imaging features. Also, CMR enables the assessment of the extension of the cardiac area affected by amyloidosis. T1 mapping technique measures myocardial amyloid load and myocyte response to infiltration, thereby allowing monitoring and eventual change of therapy, even when cardiac function is normal. These techniques are very promising for the development of treatment and prognosis of this condition. 5-8