Title:Pearls from the First Gulf Cardiac Amyloidosis Summit 2021
Volume: 3
Author(s): 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 and Nishant R. Shah
Affiliation:
- Emirates Cardiac Society, Emirates Medical Association, Dubai, UAE
Keywords:
Amyloidosis, Transthyretin, Diagnosis, Cardiomyopathy, Scintigraphy, Genetic testing.
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.
