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Cardiac amyloidosis as a rare cause of heart failure: A case report
Ming Gao1, Quan Liu1, Liping Chen2
1Department of Cardiology.
Insights
Cardiac amyloidosis, a rare condition, presents with varied symptoms. Early consideration is crucial for patients with unexplained heart failure, as diagnosis and treatment remain challenging, impacting prognosis.
Area of Science:
- Cardiology
- Hematology
- Oncology
Background:
- Cardiac amyloidosis is a rare, challenging diagnosis due to nonspecific symptoms.
- Prognosis is determined by cardiac involvement; heart failure indicates poor survival.
- Optimal treatment strategies for cardiac amyloidosis are not yet established.
Observation:
- A 50-year-old male presented with progressive dyspnea following pneumonia.
- ECG showed poor R-wave progression and low voltage; echocardiography revealed left ventricular hypertrophy and diastolic dysfunction.
- Fat aspirate confirmed amyloidosis; bone marrow showed benign monoclonal gammopathy.
Findings:
- Serum free light-chain analysis revealed elevated lambda chains.
- A Congo red stain on bone marrow biopsy was negative.
- Treatment with bortezomib, cyclophosphamide, and dexamethasone was initiated.
Implications:
- Cardiac amyloidosis must be suspected in adults with cardiac distress or heart failure.
- Prompt diagnosis and consideration of underlying restrictive cardiomyopathy are vital.
- This case highlights the diagnostic difficulties and poor outcomes associated with advanced cardiac amyloidosis.
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.
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