Amyloidosis Cardiac, Cardiac MRI, Diagnosis, Heart Failure, Phenotyping, Spectroscopic Analysis
Conditions
Keywords
Cardiac MRI, Exercise MRI, Magnetic Resonace Spectroscopy, Spirometry, Cardiopulmonary Exercise Testing, Disease Progression, Treatment Monitoring, Longitudinal Study
Brief summary
Cardiac amyloidosis is a progressive disorder caused by extracellular deposition of amyloid fibrils in the heart, leading to heart failure and impaired cardiac function. Early diagnosis and targeted therapies are essential to improve patient outcomes. This prospective, single-center study aims to longitudinally follow patients with suspected cardiac amyloidosis to characterize disease progression and assess treatment effects. Participants will undergo cardiac magnetic resonance imaging (resting and exercise stress MRI), magnetic resonance spectroscopy, cardiopulmonary exercise testing (spiroergometry) and blood testing at baseline and at 6, 12, and 24 months
Detailed description
This study is designed to longitudinally evaluate patients with cardiac amyloidosis. Patients undergoing clinically indicated diagnostic work-up for amyloidosis prior to initiation of specific therapy. Participants will undergo comprehensive baseline assessments including resting cardiac MRI, exercise stress MRI, magnetic resonance spectroscopy, pulmonary function testing, cardiopulmonary exercise testing (spiroergometry) as well as blood testing including biomarkers relevant to cardiac amyloidosis (e.g., NT-proBNP, troponin, serum free light chains, immunofixation, and other routine laboratory parameters). Patients will be seen at 3 and 9 months as part of routine clinical care. Study-specific assessments, including imaging and functional testing, will be performed at 6, 12, and 24 months. Imaging, functional and laboratory findings will be correlated with clinical characteristics, laboratory parameters, and treatment over time to improve disease characterization, monitoring, and understanding of disease progression in cardiac amyloidosis. The study aims to systematically characterize cardiac structure and function, disease phenotype, and clinical status, and to assess their evolution over time. Additional data collected within the study will not influence clinical management and are intended solely for scientific analysis. The overall objective is to improve understanding of disease progression and treatment effects through comprehensive phenotyping and follow-up in a real-world clinical setting
Interventions
None listed
Sponsors
Study design
Eligibility
Inclusion criteria
* Age \> 18 years * Male and female patients undergoing clinically indicated diagnostic work-up for amyloidosis or with a previously confirmed diagnosis of cardiac amyloidosis prior to initiation of therapy * Presence of left ventricular wall thickness \> 12 mm on transthoracic echocardiography and at least one "red flag" suggestive of cardiac amyloidosis (according to ESC 2021 criteria) or an otherwise clinically established suspicion of amyloidosis * Written informed consent
Exclusion criteria
* Age \< 18 years * Contraindications to cardiac MRI (e.g., metallic foreign bodies, older-generation pacemakers, severe obesity, claustrophobia) * Lack of written informed consent for study participation * Inability to comply with the study procedures
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Changes in myocardial structural parameters | after 6 and 12 months | measured by Cardiac Magnetic Resonance Tomography |
| Changes in myocardial function | after 6 and 12 months | measured by cardiac magnetic resonance |
| Changes in myocardial metabolism under therapy | after 6 and 12 months | measured by MR spectroscopy |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Changes in heart rate reserve | after 6 and 12 months | measured by functional exercise testing |
Countries
Germany
Contacts
Division of Cardiology, Pulmonary Disease and Vascular Medicine at University Hospital Duesseldorf
Division of Cardiology, Pulmonary Disease and Vascular Medicine at University Hospital Duesseldorf