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Trifascicular block as primary presentation of the cardiac amyloidosis; A rare case report

BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected pres...

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Detalles Bibliográficos
Autores principales: Yaghubi, Mohsen, Dinpanah, Hossein, Ghanei-Motlagh, Fahimeh, Kakhki, Samaneh, Ghasemi, Reza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Isfahan Cardiovascular Research Center, Isfahan University of Medical Sciences 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6087624/
https://www.ncbi.nlm.nih.gov/pubmed/30108642
http://dx.doi.org/10.22122/arya.v14i2.1676
Descripción
Sumario:BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation. CASE REPORT: A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block. Echocardiography study showed a moderately increased thickness of left ventricular wall with concentric pattern as well. Laboratory investigations including serum and urine electrophoresis, and serum free light chain examination as immunofixation assay revealed that κ chains predominated over λ chains in a ratio of 3:2. Our patient with final diagnosis of amyloid light-chain (AL) amyloidosis underwent chemotherapy with melphalan combined with high-dose dexamethasone, CPHPC and monoclonal antibodies for 2 weeks. CONCLUSION: It shows that rapid diagnosis of AL amyloidosis can enhance the prognosis. Applying an optimal strategy for the treatment leads to effective therapy, too.