Related Experiment Video
Updated: Jul 6, 2026

Tissue Preparation Techniques for Contrast-Enhanced Micro Computed Tomography Imaging of Large Mammalian Cardiac Models with Chronic Disease
Published on: February 8, 2022
[Cardiac amyloidosis]
Caroline Hoyer1, Christiane E Angermann, Stefan Knop
1Medizinische Klinik und Poliklinik I, Universität Würzburg, Klinikstrasse 6-8, Würzburg. Hoyer_C@klinik.uni-wuerzburg.de
Insights
Amyloidosis involves amyloid fibril deposition in multiple organs, often affecting the heart and leading to severe complications. Treatment varies by type, including stem cell or liver transplantation for specific forms.
Area of Science:
- Cardiology
- Hematology
- Nephrology
- Neurology
Background:
- Amyloidoses are multisystem disorders characterized by extracellular amyloid fibril deposition.
- Cardiac involvement is common and a leading cause of mortality in amyloidosis patients.
- Symptoms include edema, dyspnea, chest pain, and autonomic dysfunction.
Purpose of the Study:
- To provide an overview of amyloidosis, focusing on cardiac involvement.
- To discuss the common types of amyloidosis, including AL and ATTR.
- To outline current and potential treatment strategies.
Main Methods:
- Literature review of amyloidosis, cardiac manifestations, and treatment options.
- Discussion of diagnostic criteria and therapeutic approaches for different amyloidosis subtypes.
- Synthesis of information on AL amyloidosis, ATTR amyloidosis, and other forms.
Main Results:
- AL amyloidosis, linked to monoclonal gammopathy, is the most common form, treatable with melphalan and stem cell transplantation.
- ATTR amyloidosis, an autosomal dominant disorder, is curable with liver transplantation.
- Cardiac amyloidosis management follows heart failure guidelines, with other types including senile systemic and secondary amyloidosis.
Conclusions:
- Amyloidosis is a serious condition with significant cardiac implications.
- Treatment selection is crucial, especially for high-mortality procedures like stem cell transplantation.
- Multidisciplinary approaches are essential for managing this complex group of diseases.
Abstract:
Amyloidoses are a heterogeneous group of multisystem disorders, which are characterized by an extracellular deposition of amyloid fibrils. Typically affected are the heart, liver, kidneys, and nervous system. More than half of the patients die due to cardiac involvement. Clinical signs of cardiac amyloidosis are edema of the lower limbs, hepatomegaly, ascites and elevated jugular vein pressure, frequently in combination with dyspnea. There can also be chest pain, probably due to microvessel disease. Dysfunction of the autonomous nervous system or arrhythmias may cause low blood pressure, dizziness, or recurrent syncope. The AL amyloidosis caused by the deposition of immunoglobulin light chains is the most common form. It can be performed by monoclonal gammopathy. The desirable treatment therapy consists of high-dose melphalan therapy twice followed by autologous stem cell transplantation. Due to the high peritransplantation mortality, selection of appropriate patients is mandatory. The ATTR amyloidosis is an autosomal dominant disorder caused by the amyloidogenic form of transthyretin, a plasmaprotein that is synthesized in the liver. Therefore, liver transplantation is the only curative therapy. The symptomatic treatment of cardiac amyloidosis is based on the current guidelines for chronic heart failure according to the patient's New York Heart Association (NYHA) state. Further types of amyloidosis with possible cardiac involvement comprise the senile systemic amyloidosis caused by the wild-type transthyretin, secondary amyloidosis after chronic systemic inflammation, and the beta(2)-microglobulin amyloidosis after long-term dialysis treatment.
Related Concept Videos
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy V: Interprofessional Care
Cardiomyopathy VI: Nursing Management
