Cardiac manifestations in Finnish gelsolin amyloidosis patients
| Autor: | Miia Holmström, Arttu Holkeri, Sami Pakarinen, Sari Atula, Aapo L. Aro, Sari Kiuru-Enari, Lauri Lehmonen, Tuuli Mustonen |
|---|---|
| Přispěvatelé: | Neurologian yksikkö, HUS Neurocenter, HUS Heart and Lung Center, HUS Medical Imaging Center, Department of Diagnostics and Therapeutics, Clinicum |
| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Finnish gelsolin amyloidosis Contrast Media Gadolinium macromolecular substances 030204 cardiovascular system & hematology cardiac magnetic resonance s disease Cohort Studies 03 medical and health sciences Electrocardiography 0302 clinical medicine Internal Medicine medicine Meretoja’ Humans cardiovascular diseases Finland Gelsolin Conduction disease Aged conduction disease Amyloid Neuropathies Familial medicine.diagnostic_test business.industry Amyloidosis medicine.disease Systemic amyloidosis 3. Good health 3121 General medicine internal medicine and other clinical medicine cardiovascular system 1182 Biochemistry cell and molecular biology 3111 Biomedicine Cardiac magnetic resonance business 030217 neurology & neurosurgery |
| Zdroj: | Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis. 28(3) |
| ISSN: | 1744-2818 |
| Popis: | Introduction Finnish gelsolin amyloidosis (AGel amyloidosis) is an inherited systemic amyloidosis with well-known ophthalmological, neurological and cutaneous symptoms. Additionally, cardiomyopathies, conduction disorders and need of cardiac pacemakers occur in some patients. This study focuses on electrocardiographic (ECG) findings in AGel amyloidosis and their relation to cardiac magnetic resonance (CMR) changes. We also assessed whether ECG abnormalities were associated with pacemaker implantation and mortality. Materials and methods In this cohort study, 51 genetically verified AGel amyloidosis patients (mean age 66 years) without cardiac pacemakers underwent 12-lead ECG and CMR imaging with contrast agent in 2017. Patients were followed-up for 3 years. Results Conduction disturbances were found in 22 patients (43%). Nine (18%) presented with first-degree atrioventricular block, six (12%) with left anterior hemiblock, seven (14%) with left or right bundle branch block and two (4%) with non-specific intraventricular conduction delay. Low QRS voltage was present in two (4%) patients. Late gadolinium enhancement (LGE) concentrating on the interventricular septum and inferior parts of the heart was present in 19 (86%) patients with conduction abnormalities. During the follow-up, only one patient received a pacemaker, and one patient died. Discussion Conduction disorders and septal LGE are common in AGel amyloidosis, whereas other ECG and CMR findings typically observed in most common cardiac amyloidosis types were rare. Septal pathology seen in CMR may interfere with the cardiac conduction system in AGel amyloidosis, explaining conduction disorders, although pacemaker therapy is rarely required. |
| Databáze: | OpenAIRE |
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