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New Emirates Medical Journal

Volume 3, 2 Issues, 2022
ISSN: 0250-6882 (Online)
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Open Access Article

Pearls from the First Gulf Cardiac Amyloidosis Summit 2021



Abdullah Shehab1, *0000-0001-8533-9174, Albena Todorova2, Aysha Ahmed3, Esther Gonzalez-Lopez4, Fabian Knebel5, Haluk Alibazoglu6, Hani Sabbour6, Ingy Romany7, Isabel Krsnik8, Juwairiah Al Ali9, Khaldoon Al Humood10, Marianna Fontana11, Matlooba Al Zidjali12, Nishant R. Shah13
1 Emirates Cardiac Society, Emirates Medical Association, Dubai, UAE
2 Genetic & Medico-Diagnostic Laboratory, Sofia, Bulgaria
3 Salmaniya Medical Complex, Manama, Bahrain
4 Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain
5 Campus Mitte, Berlin, Germany
6 Cleveland Clinic, Abu Dhabi, UAE
7 Pfizer Gulf FZ LLC, Dubai, UAE
8 Hospital Universitario Puerta de Hierro, Majadahonda, Madrid, Spain
9 Rashid Hospital, Dubai, UAE
10 Chest Diseases Hospital, Kuwait City, Kuwait
11 Royal Free Hospital, London, United Kingdom
12 National Heart Center, Royal Hospital, Muscat, Oman
13 Warren Alpert Medical School of Brown University, Providence, RI, USA

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.


Article Information


Identifiers and Pagination:

Year: 2022
Volume: 3
Issue: 1
First Page: 1
Last Page: 11
Publisher Id: nemj-3-1
DOI: 10.2174/03666220117152535

Article History:

Received Date: 05/01/2022
Revision Received Date: 06/01/2022
Acceptance Date: 06/01/2022
Electronic publication date: 21/01/2022
Collection year: 2022

© 2022 Shehab et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


* Address correspondence to this author at the Emirates Cardiac Society, Emirates Medical Association, Dubai, UAE; Tel: +971 4 255 6655; Fax: +971 4 255 6363; Tel Phone: 0502430220; E-mail:heartdr123@googlemail.com





Editor-in-Chief

Abdullah Shehab
Emirates Cardiac Society
Emirates Medical Association
Dubai
(United Arab Emirates)
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Acceptance rate = 40%

Average review speed: 45 days average

18 days from acceptance to publication