Kourek C, Briasoulis A, Magouliotis DE, Georgoulias P, Giamouzis G, Triposkiadis F, Skoularigis J, Xanthopoulos A. Recent advances in the diagnostic methods and therapeutic strategies of transthyretin cardiac amyloidosis. World J Cardiol 2024; 16(7): 370-379 [DOI: 10.4330/wjc.v16.i7.370]
Corresponding Author of This Article
Andrew Xanthopoulos, MD, PhD, Postdoctoral Fellow, Department of Cardiology, School of Medicine, University Hospital of Larissa, Mezourlo, Larissa 41110, Greece. andrewvxanth@gmail.com
Research Domain of This Article
Cardiac & Cardiovascular Systems
Article-Type of This Article
Editorial
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Cardiol. Jul 26, 2024; 16(7): 370-379 Published online Jul 26, 2024. doi: 10.4330/wjc.v16.i7.370
Recent advances in the diagnostic methods and therapeutic strategies of transthyretin cardiac amyloidosis
Christos Kourek, Alexandros Briasoulis, Dimitrios E Magouliotis, Panagiotis Georgoulias, Grigorios Giamouzis, Filippos Triposkiadis, John Skoularigis, Andrew Xanthopoulos
Christos Kourek, Clinical Ergospirometry, Exercise and Rehabilitation Laboratory, 1st Critical Care Medicine Department, Evangelismos Hospital, National and Kapodistrian University of Athens, Athens 10676, Greece
Alexandros Briasoulis, Department of Clinical Therapeutics, Alexandra Hospital, Faculty of Medicine, National and Kapodistrian University of Athens, Athens 11528, Greece
Dimitrios E Magouliotis, Department of Cardiothoracic Surgery, University of Thessaly, Larissa Biopolis, Larissa 41110, Greece
Panagiotis Georgoulias, Department of Nuclear Medicine, University Hospital of Larissa, Larissa 41110, Greece
Grigorios Giamouzis, Filippos Triposkiadis, John Skoularigis, Andrew Xanthopoulos, Department of Cardiology, School of Medicine, University Hospital of Larissa, Larissa 41110, Greece
Author contributions: Briasoulis A, Magouliotis DE, Triposkiadis F and Xanthopoulos A designed the overall concept and outline of the manuscript; Kourek C, Georgoulias P, Giamouzis G and Skoularigis J contributed to the discussion and design of the manuscript; All authors contributed to the writing, editing of the manuscript, illustrations, and review of the literature.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Andrew Xanthopoulos, MD, PhD, Postdoctoral Fellow, Department of Cardiology, School of Medicine, University Hospital of Larissa, Mezourlo, Larissa 41110, Greece. andrewvxanth@gmail.com
Received: March 27, 2024 Revised: June 6, 2024 Accepted: June 25, 2024 Published online: July 26, 2024 Processing time: 118 Days and 21 Hours
Abstract
Cardiac amyloidosis is a progressive disease characterized by the buildup of amyloid fibrils in the extracellular space of the heart. It is divided in 2 main types, immunoglobulin light chain amyloidosis and transthyretin amyloidosis (ATTR), and ATTR amyloidosis is further divided in 2 subtypes, non-hereditary wild type ATTR and hereditary mutant variant amyloidosis. Incidence and prevalence of ATTR cardiac amyloidosis is increasing over the last years due to the improvements in diagnostic methods. Survival rates are improving due to the development of novel therapeutic strategies. Tafamidis is the only disease-modifying approved therapy in ATTR amyloidosis so far. However, the most recent advances in medical therapies have added more options with the potential to become part of the therapeutic armamentarium of the disease. Agents including acoramidis, eplontersen, vutrisiran, patisiran and anti-monoclonal antibody NI006 are being investigated on cardiac function in large, multicenter controlled trials which are expected to be completed within the next 2-3 years, providing promising results in patients with ATTR cardiac amyloidosis. However, further and ongoing research is required in order to improve diagnostic methods that could provide an early diagnosis, as well as survival and quality of life of these patients.
Core Tip: Transthyretin amyloidosis (ATTR) cardiac amyloidosis is a progressive disease, affecting patients’ functional capacity, survival and quality of life. Incidence and prevalence of ATTR cardiac amyloidosis is increasing over the last years while survival rates are improving. Tafamidis is the only disease-modifying approved therapy so far. Novel medical therapies including acoramidis, eplontersen, vutrisiran, patisiran and anti-monoclonal antibody NI006 are being investigated on cardiac function in large, multicenter controlled trials, with the potential to become part of the therapeutic armamentarium of the disease.
