Open AccessPearls from the First Gulf Cardiac Amyloidosis Summit 2021
Author(s) -
Abdullah Shehab,
Албена Тодорова,
Aftab Ahmed,
Esther González-López,
Fabian Knebel,
Haluk Alibazoglu,
Hani Sabbour,
Ingy Romany,
Isabel Krsnik,
Juwairiah Al Ali,
Khaldoon Al Humood,
Marianna Fontana,
Matlooba Al Zidjali,
Nishant R. Shah
Publication year - 2022
Publication title -
emirates medical journal
Language(s) - English
Resource type - Journals
ISSN - 0250-6882
DOI - 10.2174/03666220117152535
Subject(s) - cardiac amyloidosis , medicine , amyloidosis , intensive care medicine , transthyretin , differential diagnosis , restrictive cardiomyopathy , cardiomyopathy , radiology , pathology , heart failure
These proceedings from the First Gulf Cardiac Amyloidosis Summit held in June 2021 aimed to increase awareness of cardiacamyloidosis among the wider medical community in the region. Although the clinical presentation of cardiac amyloidosis is highly variable, anumber of ‘red flags’ have been identified to raise suspicion of the disease and prompt further investigation. Accurate diagnosis of cardiacamyloidosis is challenging and relies on the integration of clinical, imaging and laboratory investigations. Recent imaging techniques, includingbone scintigraphy together with a complete serum and urine workup, allow, in the majority of patients, accurate non-invasive diagnosis without theneed for confirmatory endomyocardial biopsies. Early differential diagnosis between light-chain (AL) amyloidosis and amyloid transthyretinamyloidosis (ATTR) is critical for timely delivery of appropriate therapy. AL amyloidosis is a medical emergency requiring chemotherapy andsupportive care. Treatment for ATTR-amyloidosis is most effective when administered early, before development of significant symptoms orcardiac dysfunction. Optimal management of patients involves close collaboration between multidisciplinary specialists, which may includehematologists, cardiologists, and other subspecialists, ideally at a designated specialty center with interest and expertise in amyloidosis.