P354 REMOVAL OF CARDIAC AL–AMYLOID LEADS TO CARDIOMYOCYTE POSITIVE REMODELING AND RESTRICTIVE PATTERN REVERSAL
| Autor: | E Maggio, F Ballatore, F Ajmone, G Manguso, R Verardo, P Ciaramella, A Vernile, M Alfarano, C Vizza, C Chimenti |
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| Rok vydání: | 2023 |
| Předmět: | |
| Zdroj: | European Heart Journal Supplements. 25:D182-D182 |
| ISSN: | 1554-2815 1520-765X |
| DOI: | 10.1093/eurheartjsupp/suad111.427 |
| Popis: | Background Properly treated AL cardiac amyloidosis can have a long survival. However, the mechanism involved in arresting or reversing cardiomyopathy, such as toxic suppression of the light chain or removal of the amyloid substance, is still unclear. We describe the case of a patient with AL amyloidosis treated with chemotherapy in whom serial endomyocardial biopsies were performed at diagnosis and after 17 years. Clinical case A 71–year–old lady onset with a hypertrophic phenotype cardiomyopathy (maximum thickness LV 18.2 mm, diastolic dysfunction with E/A ratio 2.45. preserved contractility (EF 55%) and NYHA class 3) received a diagnosis in 2004 of AL amyloidosis and underwent several cycles of chemotherapy. At clinical onset, cardiac amyloidosis was documented by endomyocardial biopsy. After 17 years, in 2021, the patient was hospitalized for palpitations and chest pain and documented intermittent phases of atrial fibrillation. The patient was reevaluated with immunoelectrophoresis, echocardiogram, cardiac magnetic resonance, coronary angiography, and left ventricular endomyocardial biopsy. Serum monoclonal chain AL–K was reduced from initial 233 mg/L to 18.2 mg/L, vn Conclusions Remission of monoclonal gammopathy is associated with a removal of cardiac amyloid deposits and with a regression of cardiac hypertrophy and restrictive pattern. Histologically, the degradation of amyloid fibrils determines a better trophism of cardiomyocytes. |
| Databáze: | OpenAIRE |
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