Cardiac amyloidosis (CA) is caused by the accumulation of amyloid fibrils in the extracellular space of the myocardium, with clinical manifestations including conduction disturbances and heart failure. CA has traditionally been considered a rare disease, lacking effective therapies and with a poor prognosis, but in recent years, increased possibilities for non-invasive diagnosis and greater awareness of the disease have led to the identification of a growing number of cases, challenging the concept of CA as a rare disease. This is why the ability to recognise and manage patients with CA should…mehr
Cardiac amyloidosis (CA) is caused by the accumulation of amyloid fibrils in the extracellular space of the myocardium, with clinical manifestations including conduction disturbances and heart failure. CA has traditionally been considered a rare disease, lacking effective therapies and with a poor prognosis, but in recent years, increased possibilities for non-invasive diagnosis and greater awareness of the disease have led to the identification of a growing number of cases, challenging the concept of CA as a rare disease. This is why the ability to recognise and manage patients with CA should become part of the core curriculum of cardiologists, but also of internal medicine specialists, general practitioners, neurologists, nephrologists and haematologists. This book, written by leading experts in the field, will provide the state-of-the-art in the diagnosis and management of CA and will also discuss the latest research developments and future research directions.
Thevolume appears to be a timely one, given the great interest of clinicians and researchers in CA and the exponential increase in publications in recent year, and will be of interest to cardiologists, internal medicine and other disciplines specialists who may encounter patients with CA in their clinical practice, as well as general practitioners.
Michele Emdin is Full Professor of Cardiology and Coordinator of the Health Science Interdisciplinary Center at the Sant'Anna School of Advanced Studies, Pisa, Italy, as well as Director of the Cardio-Thoracic Department at the Fondazione Toscana Gabriele Monasterio, Pisa and Massa, Italy. His clinical and research interest covers the whole spectrum of pathophysiology, diagnostics and medical management of cardiovascular diseases, with a focus on biomarkers, cardiomyopathies, namely of cardiac amyloidosis, and heart failure. Giuseppe Vergaro is Consultant Cardiologist at the Fondazione Toscana Gabriele Monasterio, where he is in charge of the AmyLab, an outpatient facility dedicated to patients with cardiac amyloidosis, and Assistant Professor at the Sant'Anna School of Advanced Studies. His main research interests include the investigation of the epidemiology, novel diagnostic and therapeutic strategies in cardiac amyloidosis. Alberto Aimo is a Cardiologist at the Fondazione Toscana Gabriele Monasterio (a section of the Italian National Research Council) and a Research Fellow at the Sant'Anna School of Advanced Studies. His research activity has mostly focused on heart failure and cardiomyopathies, particularly idiopathic dilated cardiomyopathy and cardiac amyloidosis, but also cardiac involvement in mitochondrial disease or muscular dystrophies. His main fields of interest are the use of biomarkers or imaging findings as tools for diagnosis and management of heart failure and cardiomyopathies. Marianna Fontana is the Director of the UCL CMR unit at the Royal Free Hospital. She is Professor of Cardiology and Honorary Consultant Cardiologist at the National Amyloidosis Centre, Division of Medicine, University College London, where she is Deputy Head of Center and Deputy Clinical Lead. She is the recipient of numerous awards, including the Michael Davies Early Career Award in 2021and the BHF Fellow of the year award in 2022. Her major clinical and research interests are in the delivery of efficient and effective care for patients with amyloidosis, with a particular focus on new technologies: imaging and drugs.
Inhaltsangabe
1. Tribute to Claudio Rapezzi.- 2. Giampaolo Merlini and the Pavia School.- 3. A brief history of amyloidosis.- 4. Pathophysiology, classification and epidemiology of amyloidosis.- 5. Amyloid light chain (AL) amyloidosis.- 6. Hereditary transthyretin amyloidosis.- 7. Wild-type ATTR amyloidosis.- 8. Electrocardiographic patterns.- 9. Echocardiography: a gatekeeper to diagnosis.- 10. Cardiovascular magnetic resonance: characterization of myocardial involvement.- 11. Biomarkers: monoclonal protein and indicators of cardiac damage.- 12. Plasma transthyretin and its ligands.- 13. Cardiac Scintigraphy with Bone-Avid Tracers - Old and New Applications.- 14. PET-CT: a tool for etiological diagnosis.- 15. The role of tissue biopsy: identification of the amyloid precursor and beyond.- 16. From red flags to diagnosis.- 17. Risk prediction and follow-up.- 18. Differential diagnoses in clinical mimics.- 19. Applications of Artificial Intelligence in Amyloidosis.- 20. Treatment of amyloid light-chain amyloidosis.- 21. Treatment of ATTR amyloidosis: from stabilizers to gene-editing.- 22. Treatment of cardiac complications.- 23. Monitoring disease progression and response to disease-modifying treatments.- 24. Cardiac amyloidosis: open issues and future perspectives.
1. Tribute to Claudio Rapezzi.- 2. Giampaolo Merlini and the Pavia School.- 3. A brief history of amyloidosis.- 4. Pathophysiology, classification and epidemiology of amyloidosis.- 5. Amyloid light chain (AL) amyloidosis.- 6. Hereditary transthyretin amyloidosis.- 7. Wild-type ATTR amyloidosis.- 8. Electrocardiographic patterns.- 9. Echocardiography: a gatekeeper to diagnosis.- 10. Cardiovascular magnetic resonance: characterization of myocardial involvement.- 11. Biomarkers: monoclonal protein and indicators of cardiac damage.- 12. Plasma transthyretin and its ligands.- 13. Cardiac Scintigraphy with Bone-Avid Tracers - Old and New Applications.- 14. PET-CT: a tool for etiological diagnosis.- 15. The role of tissue biopsy: identification of the amyloid precursor and beyond.- 16. From red flags to diagnosis.- 17. Risk prediction and follow-up.- 18. Differential diagnoses in clinical mimics.- 19. Applications of Artificial Intelligence in Amyloidosis.- 20. Treatment of amyloid light-chain amyloidosis.- 21. Treatment of ATTR amyloidosis: from stabilizers to gene-editing.- 22. Treatment of cardiac complications.- 23. Monitoring disease progression and response to disease-modifying treatments.- 24. Cardiac amyloidosis: open issues and future perspectives.
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