IJCS | Volume 32, Nº2, March/April 2019

185 Figure 7 - Postcontrast T1 mapping short axis, showing the myocardium with high T1 value (approximately 500 ms) in green (*). Inferoseptal, anteroseptal and anterior subendocardial fibrosis, (approximately 307 ms) in blue (***). Ribeiro et al. CMR and amyloidosis Int J Cardiovasc Sci. 2019;32(2)177-189 Review Article Prognosis In 2008, Maceira et al., 11 have evaluated the capacity of CMR in characterizingmortality, survival with therapeutic response and development of diastolic function. In 2015, the study by Fontana et al., 7 made clear the importance of detecting delayed enhancement in immunoglobulin light chain and transthyretin amyloidosis and differentiating subendocardial from transmural pattern. Regardless of the type of amyloidosis, transmural delayed enhancement is an important indicator of mortality, which seems to occur earlier in immunoglobulin light chain amyloidosis. In 2016, Boynton et al., 55 have demonstrated that delayed enhancement technique provides additional prognostic information to serum biomarkers in patients with cardiac amyloidosis. An important finding was the definition of disease severity by the method in case of inability to null the myocardium by delayed enhancement or in case of diffuse or transmural enhancement. This study had a long editorial highlighting the importance of the delayed enhancement technique in patients with amyloidosis, 5 corroborated by the study by Raina et al. 43 CMR can also help in the characterization of severe patients by assessment of left ventricular function, as proposed by Mohty et al. 56 Recently, Martinez-Naharro et al., 57 in a study published in JACC in 2017, demonstrated the role of the ECV as an independent factor of survival in amyloidosis patients. We believe that, in combination with technological updates of imaging systems, ECV can provide incremental prognostic information in patients with amyloidosis over myocardial delayed enhancement. Treatment In general, cardiac amyloidosis has a poor prognosis, depending on the type of amyloidosis, therapy availability and response to treatment. The treatment can be classified as – supportive therapy (specific treatment for heart failure, including pacemakers and automated defibrillators); therapies to suppress the synthesis of amyloid precursor protein (e.g. chemotherapy in immunoglobulin light chain amyloidosis); and new strategies to inhibit the formation of amyloid fibrils, as those targeting amyloid deposits or those including promisingmedications (such as tafamidis and diflunisal)