Cardiac amyloidosis as a rare cause of heart failure

Rationale: Cardiac amyloidosis is a rare condition that is difficult to diagnose, because the clinical manifestations are often varied and nonspecific. The presence and degree of cardiac involvement are the main prognosis determinants, with a median survival of 6 months expected when presented with heart failure. Moreover, the optimal treatment for cardiac amyloidosis is still unclear. Patient concerns: We report a case of a 50-year-old man who was admitted with evolution of progressive dyspnea. Two months before the present admission, the patient was diagnosed with bacterial pneumonia complicated by bilateral parapneumonic effusion that required drainage. Diagnosis: Electrocardiography demonstrated poor R-wave progression in leads V1-V3 with right axis deviation and low voltage criteria. Echocardiography revealed diffuse left ventricular hypertrophy with normal ventricular cavity size, severe diastolic dysfunction, and sparkling and granular texture of the ventricle wall. Serum free light-chain analysis showed an altered kappa/lambda ratio of 0.01 with lambda light chains greatly elevated. A periumbilical fat aspirate sample confirmed amyloidosis. Bone marrow examination confirmed benign monoclonal gammopathy with 8.5% plasma cells, and biopsy stained for Congo red was negative. Intervention: A combination of bortezomib with cyclophosphamide and dexamethasone treatment was initiated. Outcome: Unfortunately, 5 days after the second therapy with bortezomib, the patient died. Lessons: Cardiac amyloidosis should be seriously considered in any adult with signs or nonspecific symptoms of cardiac distress, most notably congestive heart failure due to underlying restrictive cardiomyopathy.