Article ; Online: The importance of pathways to facilitate early diagnosis and treatment of patients with cardiac amyloidosis.
Therapeutic advances in cardiovascular disease
2023 Volume 17, Page(s) 17539447231216318
Abstract: Cardiac amyloidosis (CA) is a condition caused by extracellular deposition of amyloid fibrils in the heart. It is an underdiagnosed disease entity which can present with a variety of cardiac and non-cardiac manifestations. Diagnosis usually follows an ... ...
| Abstract | Cardiac amyloidosis (CA) is a condition caused by extracellular deposition of amyloid fibrils in the heart. It is an underdiagnosed disease entity which can present with a variety of cardiac and non-cardiac manifestations. Diagnosis usually follows an initial suspicion based on clinical evaluation or imaging findings before confirmation with subsequent imaging (echocardiography, cardiac magnetic resonance imaging, 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy) in combination with biochemical screening for monoclonal dyscrasia (serum free light chains and serum and urine electrophoresis) and/or histology (bone marrow trephine, fat or endomyocardial biopsy). More than 95% of CA can be classified as either amyloid light-chain (AL) CA or amyloid transthyretin (ATTR) CA; these two conditions have very different management strategies. AL-CA, which may be associated with multiple myeloma, can be managed with chemotherapy agents, autologous stem cell transplantation, cardiac transplant and supportive therapies. For ATTR-CA, there is increasing importance in making an early diagnosis because of novel treatments in development, which have transformed this once incurable disease to a potentially treatable disease. Timely diagnosis is crucial as there may only be a small window of opportunity where patients can benefit from treatment beyond which therapies may be less effective. Reviewing the existing patient pathway provides a basis to better understand the complexities of real-world activities which may be important to help reduce missed opportunities related to diagnosis and treatment for patients with CA. With healthcare provider interest in improving the care of patients with CA, the development of an optimal care pathway for the condition may help reduce delays in diagnosis and treatment and thus enhance patient outcomes. |
|---|---|
| MeSH term(s) | Humans ; Cardiomyopathies/diagnostic imaging ; Cardiomyopathies/therapy ; Hematopoietic Stem Cell Transplantation ; Transplantation, Autologous ; Amyloidosis/diagnosis ; Amyloidosis/therapy ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/therapy ; Echocardiography ; Amyloid ; Early Diagnosis ; Amyloidogenic Proteins |
| Chemical Substances | Amyloid ; Amyloidogenic Proteins |
| Language | English |
| Publishing date | 2023-12-15 |
| Publishing country | England |
| Document type | Journal Article ; Review |
| ZDB-ID | 2485062-7 |
| ISSN | 1753-9455 ; 1753-9447 |
| ISSN (online) | 1753-9455 |
| ISSN | 1753-9447 |
| DOI | 10.1177/17539447231216318 |
| Database | MEDical Literature Analysis and Retrieval System OnLINE |
More links
Kategorien
In stock of ZB MED Cologne/Königswinter
| Zs.A 6798: Show issues | Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG) |
Order via subito
This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.