TITLE:
Extensive Bi-Atrial Remodeling on Hypertensive Patient with Permanent Atrial Fibrillation Delayed Diagnosis of Fatal Cardiac Lambda Amyloidosis
AUTHORS:
Houzéiph Abdou Lassissi, Ngardjibem Djita, Ibrahima Sory Sylla, Thome Masset, Abdelrhani Moumen, Sedjolo Emmanuelle Bibiane Kpomalegni
KEYWORDS:
Myeloma, Cardiac Lambda Light Chain Amyloidosis, Monoclonal Gammopathy, Heart Failure
JOURNAL NAME:
World Journal of Cardiovascular Diseases,
Vol.14 No.6,
June
28,
2024
ABSTRACT: Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits of transthyretin and immunoglobulin light chains. Treatment depends on the type of amyloidosis. Cardiac light chain amyloidosis is a medical emergency. Aim: To highlight the importance of an early diagnosis of cardiac light chain amyloidosis. Case Presentation: We report the case of an 88-year-old hypertensive female patient with sustained atrial fibrillation and recurrent heart failure, in whom echocardiography showed concentric left ventricle hypertrophy with mildly reduced left ventricle ejection fraction (LVEF) to 45%. Bone scintigraphy was normal. Serum analysis showed increased lambda free light chains. Accessory salivary gland biopsy revealed weak Kappa light chain staining and clear overexpression of lambda light chain deposits. The diagnosis of stage 3B cardiac amyloidosis secondary to lambda light chain myeloma was made. After a multidisciplinary meeting, it was decided to start treatment with DARATUMUMAB + LENALIDOMIDE. Patient’s general condition deteriorated with the occurrence of febrile pancytopenia. Chemotherapy was stopped and management was limited to comfort care until the patient’s death. Conclusion: Cardiac light-chain amyloidosis must be diagnosed early as it can be rapidly fatal.