Pearls from the First Gulf Cardiac Amyloidosis Summit 2021

Abdullah      Shehab; Albena      Todorova; Aysha      Ahmed; Esther      Gonzalez-Lopez; Fabian      Knebel; Haluk      Alibazoglu; Hani      Sabbour; Ingy      Romany; Isabel      Krsnik; Juwairiah      Al Ali; Khaldoon      Al Humood; Marianna      Fontana; Matlooba      Al Zidjali; Nishant   R.   Shah

Abstract

These proceedings from the First Gulf Cardiac Amyloidosis Summit held in June 2021 aimed to increase awareness of cardiac amyloidosis among the wider medical community in the region. Although the clinical presentation of cardiac amyloidosis is highly variable, a number of ‘red flags’ have been identified to raise suspicion of the disease and prompt further investigation. Accurate diagnosis of cardiac amyloidosis is challenging and relies on the integration of clinical, imaging and laboratory investigations. Recent imaging techniques, including bone scintigraphy together with a complete serum and urine workup, allow, in the majority of patients, accurate non-invasive diagnosis without the need for confirmatory endomyocardial biopsies. Early differential diagnosis between light-chain (AL) amyloidosis and transthyretin amyloidosis (ATTR) is critical for timely delivery of appropriate therapy. AL amyloidosis is a medical emergency requiring chemotherapy and supportive care. Treatment for ATTR-amyloidosis is most effective when administered early, before development of significant symptoms or cardiac dysfunction. Optimal management of patients involves close collaboration between multidisciplinary specialists, which may include hematologists, cardiologists, and other subspecialists, ideally at a designated specialty center with interest and expertise in amyloidosis.

Keywords: Amyloidosis, Transthyretin, Diagnosis, Cardiomyopathy, Scintigraphy, Genetic testing.

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