Prof. Livi Ugolino
Department of Cardiothoracic Surgery, Azienda Sanitaria Universitaria Integrata, Udine, Italy; Department of Medical Area, University of Udine, Udine, Italy.
Prof. Gianfranco Sinagra
Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste, Italy.
Special Issue Introduction
Systemic amyloidosis is a rare haematologic disorder where fibrils of misfolded proteins acquire a b-pleated sheet conformation, form amyloid fibril proteins, and begin to infiltrate tissues leading to organ failure. At least 30 different proteins have been reported to produce amyloid fibrils, but two types are more capable of affecting the heart, such as light-chain (AL) amyloidosis and transthyretin-related (TTR) amyloidosis, as hereditary or acquired form. The heart involvement due to the extracellular protein fibril deposition in the myocardium could appear with several manifestations, ranging from conduction-system disease to atrial fibrillation, sudden cardiac death or restrictive cardiomyopathy and heart failure, thus conditioning the stage and prognosis of the entire disease. Recently, significant progress has been made in the treatment of amyloid cardiomyopathy, and these promising results have already opened new chances for these patients. This Special Issue has two main objectives: (1) to provide the readers, through thoughtful review articles, a comprehensive state-of-art of the current knowledge about cardiac amyloidosis; (2) to stimulate researchers to share their experience in this field, since the overall contributions could open new borders in such rare diseases, as cardiac amyloidosis.
Cardiac amyloidosis, light chain cardiac amyloidosis, transthyretin amyloid cardiomyopathy, wild type TTR amyloidosis, hereditary TTR amyloidosis, heart failure, heart transplantation
30 Apr 2021