Cardiac amyloidosis in Latin America: Gaps and opportunities to increase awareness of the disease. Findings from the AMILO-LATAM research group.

BACKGROUND: Cardiac amyloidosis (CA) is an under-diagnosed disease presenting as a restrictive cardiomyopathy with high morbidity and mortality. Wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) is mostly seen in elderly patients, with increasing prevalence as life expectancy is growing. New diagnostic imaging techniques and treatments allow for a better prognosis, but lack of clinical awareness delays timely diagnosis and appropriate management. Our purpose was to investigate the knowledge of clinicians regarding ATTR-CM and to assess the availability of imaging resources in the Latin-American region. METHODS AND RESULTS: Two online surveys were distributed among clinicians and nuclear medicine professionals, respectively: one asking about awareness of CA in different clinical scenarios, and the other about the availability of diagnostic resources and studies performed. 406 responses were received for the first survey and 82 for the second, representing 17 and 14 countries, respectively. A significant lack of awareness was identified among clinicians, although appropriate diagnostic resources are generally available. Survey data showed that very few patients are evaluated for ATTR-CM in most Latin-American countries. CONCLUSIONS: The surveys demonstrated the need for educational programs and other measures to increase clinical awareness and early detection of CA, so patients receive timely treatment and management of the disease.

MIBG cardiac imaging compared to ejection fraction in evaluation of cardiotoxicity: a systematic review.

BACKGROUND: Advances in diagnosis and treatment of cancer has improved survival but resulted in increased cardiotoxic effects. The decrease in left ventricular ejection fraction (EF), one of the pillars of diagnosis of cardiotoxicity, seems to be a late process in the evolution of the disease, so 123I-metaiodobenzylguanidine (MIBG) cardiac imaging has been proposed to detect early cardiac impairment. The aim of this systematic review was to evaluate the performance of MIBG cardiac scan in this scenario. METHODS AND RESULTS: A systematic search was conducted in five international databases comparing MIBG parameters with EF for evaluation of cardiotoxicity. Twelve studies were included and separated in three groups. First, studies evaluating patients with established cardiotoxicity, in which EF was reduced and MIBG parameters were abnormal. Second, studies analyzing patients during or after treatment compared to controls, with MIBG parameters significantly different between groups in most studies, even when EF remained normal. Finally, studies analyzing anthracycline (ATC) dose-related changes, with alteration in MIBG parameters occurring even when EF was preserved. CONCLUSION: Although studies had high methodological variability, cardiac sympathetic innervation imaging seems to be a promising tool for assessing early cardiotoxicity. Further studies are needed to analyze its diagnostic value in this scenario.