Abstract
The amyloidoses represent a group of diseases characterized by extracellular deposition of abnormal protein, amyloid, which is formed by insoluble extracellular fibrils in β-pleated sheets. Although cardiac involvement may occur in all types of amyloidoses, clinically relevant amyloid cardiomyopathy is a typical feature of AL amyloidosis and transthyretin-related amyloidoses. Congestive heart failure represents the commonest manifestation of amyloid heart disease. Noninvasive imaging techniques, especially echocardiography and cardiac magnetic resonance, play a major role in the diagnosis of amyloid cardiomyopathy; however, histological confirmation and exact typing of amyloid deposits is necessary whether in extracardiac location or directly in the myocardium. Early diagnosis of amyloid heart disease is of utmost importance as the presence and especially the severity of cardiac involvement generally drives the prognosis of affected subjects and plays a major role in determining the intensity of specific treatment, namely in AL amyloidosis. The management of patients with amyloid heart disease is complex. Loop diuretics together with aldosterone antagonists represent the basis for influencing signs of congestion. In AL amyloidosis, high-dose chemotherapy followed by autologous stem cell transplantation is generally considered to be a front-line treatment option, if the disease is diagnosed at its early stage. The combination of mephalan with dexamethasone has been the standard therapy for severely affected individuals; however, the combinations with several novel agents including immunomodulatory drugs and bortezomibe have been tested in clinical trials with promising results. New therapeutic substances with the potential to slow or even stop the progression of transthyretin-related amyloidosis are also extensively studied.
Keywords: Amyloid, cardiomyopathy, echocardiography, magnetic resonance imaging, pharmacological treatment, stem cell transplantation.
Current Pharmaceutical Design
Title:Contemporary Treatment of Amyloid Heart Disease
Volume: 21 Issue: 4
Author(s): Tomas Palecek, Michal Fikrle, Eduard Nemecek, Lenka Bauerova, Petr Kuchynka, William E. Louch and Romana Rysava
Affiliation:
Keywords: Amyloid, cardiomyopathy, echocardiography, magnetic resonance imaging, pharmacological treatment, stem cell transplantation.
Abstract: The amyloidoses represent a group of diseases characterized by extracellular deposition of abnormal protein, amyloid, which is formed by insoluble extracellular fibrils in β-pleated sheets. Although cardiac involvement may occur in all types of amyloidoses, clinically relevant amyloid cardiomyopathy is a typical feature of AL amyloidosis and transthyretin-related amyloidoses. Congestive heart failure represents the commonest manifestation of amyloid heart disease. Noninvasive imaging techniques, especially echocardiography and cardiac magnetic resonance, play a major role in the diagnosis of amyloid cardiomyopathy; however, histological confirmation and exact typing of amyloid deposits is necessary whether in extracardiac location or directly in the myocardium. Early diagnosis of amyloid heart disease is of utmost importance as the presence and especially the severity of cardiac involvement generally drives the prognosis of affected subjects and plays a major role in determining the intensity of specific treatment, namely in AL amyloidosis. The management of patients with amyloid heart disease is complex. Loop diuretics together with aldosterone antagonists represent the basis for influencing signs of congestion. In AL amyloidosis, high-dose chemotherapy followed by autologous stem cell transplantation is generally considered to be a front-line treatment option, if the disease is diagnosed at its early stage. The combination of mephalan with dexamethasone has been the standard therapy for severely affected individuals; however, the combinations with several novel agents including immunomodulatory drugs and bortezomibe have been tested in clinical trials with promising results. New therapeutic substances with the potential to slow or even stop the progression of transthyretin-related amyloidosis are also extensively studied.
Export Options
About this article
Cite this article as:
Palecek Tomas, Fikrle Michal, Nemecek Eduard, Bauerova Lenka, Kuchynka Petr, Louch E. William and Rysava Romana, Contemporary Treatment of Amyloid Heart Disease, Current Pharmaceutical Design 2015; 21 (4) . https://dx.doi.org/10.2174/138161282104141204143657
DOI https://dx.doi.org/10.2174/138161282104141204143657 |
Print ISSN 1381-6128 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-4286 |
Call for Papers in Thematic Issues
"Tuberculosis Prevention, Diagnosis and Drug Discovery"
The Nobel Prize-winning discoveries of Mycobacterium tuberculosis and streptomycin have enabled an appropriate diagnosis and an effective treatment of tuberculosis (TB). Since then, many newer diagnosis methods and drugs have been saving millions of lives. Despite advances in the past, TB is still a leading cause of infectious disease mortality ...read more
Current Pharmaceutical challenges in the treatment and diagnosis of neurological dysfunctions
Neurological dysfunctions (MND, ALS, MS, PD, AD, HD, ALS, Autism, OCD etc..) present significant challenges in both diagnosis and treatment, often necessitating innovative approaches and therapeutic interventions. This thematic issue aims to explore the current pharmaceutical landscape surrounding neurological disorders, shedding light on the challenges faced by researchers, clinicians, and ...read more
Emerging and re-emerging diseases
Faced with a possible endemic situation of COVID-19, the world has experienced two important phenomena, the emergence of new infectious diseases and/or the resurgence of previously eradicated infectious diseases. Furthermore, the geographic distribution of such diseases has also undergone changes. This context, in turn, may have a strong relationship with ...read more
Melanoma and Non-Melanoma Skin Cancer Treatment: Standard of Care and Recent Advances
In this thematic issue, we aim to provide a standard of care of the diagnosis and treatment of melanoma and non-melanoma skin cancer. The editor will invite authors from different countries who will write review articles of melanoma and non-melanoma skin cancers. The Diagnosis, Staging, Surgical Treatment, Non-Surgical Treatment all ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
- Announcements
Related Articles
-
Making the Most of Pathological Specimens: Molecular Diagnosis in Formalin-Fixed, Paraffin Embedded Tissue
Current Drug Targets Bioinformatics Tools for Mass Spectrometry-Based High-Throughput Quantitative Proteomics Platforms
Current Proteomics Transient Left Ventricular Acute Failure after Cocaine Use
Current Drug Research Reviews Amniotic Fluid Embolism: Review
Current Pharmaceutical Biotechnology Heart Failure Modulates the Muscle Reflex
Current Cardiology Reviews Therapeutic Angiogenesis using Hepatocyte Growth Factor (HGF)
Current Gene Therapy Dynamics of Toll-like Receptors Signaling in Skeletal Muscle Atrophy
Current Medicinal Chemistry Meet Our Editorial Board Member:
Current Vascular Pharmacology Human Myoblast Genome Therapies and Devices in Regenerative Medicine
Recent Patents on Regenerative Medicine Biomarker-Guided Strategy for Treatment of Autism Spectrum Disorder (ASD)
CNS & Neurological Disorders - Drug Targets Iodinated Contrast Media in Diagnostic Imaging: Cardiovascular Side Effects
Current Pharmacogenomics and Personalized Medicine Biology and Regulatory Roles of Nuclear Lamins in Cellular Function and Dysfunction
Recent Patents on Endocrine, Metabolic & Immune Drug Discovery Surrogates of Insulin Sensitivity and Indices of Cardiometabolic Profile in Obesity
Current Vascular Pharmacology Antipsychotic-Induced Electrocardiographic Changes: A Comparative Study Between Olanzapine and Risperidone
Current Psychopharmacology Heme Oxygenase: A Target Gene for Anti-Diabetic and Obesity
Current Pharmaceutical Design Cardiovascular disease management through restrained inflammatory responses
Current Pharmaceutical Design Exercise as Treatment for Neuropathy in the Setting of Diabetes and Prediabetic Metabolic Syndrome: A Review of Animal Models and Human Trials
Current Diabetes Reviews Endothelial Dysfunction in Morbid Obesity
Current Pharmaceutical Design Myocardial Energy Substrate Metabolism in Heart Failure : from Pathways to Therapeutic Targets
Current Pharmaceutical Design Common Pathways in Health Benefit Properties of RSV in Cardiovascular Diseases, Cancers and Degenerative Pathologies
Current Pharmaceutical Biotechnology