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[Primary systemic amyloidosis with cardiac manifestations]
H Fischer1, F Woitinas, R Morell
12. Medizinische Klinik, Städtisches Krankenhaus München-Schwabing.
Deutsche Medizinische Wochenschrift (1946)
|August 18, 1995
Summary
A 68-year-old woman with lambda light-chain gammopathy developed heart failure due to primary systemic amyloidosis. Diagnosis was confirmed by echocardiogram and rectal biopsy, showing restrictive cardiomyopathy.
Area of Science:
- Cardiology
- Hematology
- Pathology
Background:
- A 68-year-old Vietnamese woman had a known monoclonal lambda light-chain gammopathy for 10 years.
- Previous ECG showed abnormalities, but cardiac catheterization and initial echocardiogram were normal.
Observation:
- The patient presented with progressive dyspnea and signs of heart failure.
- Current echocardiogram revealed left atrial enlargement, left ventricular hypertrophy, and restrictive filling patterns.
- Myocardial echogenicity was increased, and rectal biopsy confirmed amyloid fibrillae deposition.
Findings:
- The findings indicated restrictive cardiomyopathy secondary to primary systemic amyloidosis.
- The patient's gammopathy was confirmed without evidence of plasmacytoma.
Implications:
- This case highlights the importance of considering systemic amyloidosis in patients with monoclonal gammopathies and unexplained heart failure.
- Early diagnosis and management of restrictive cardiomyopathy are crucial for improving patient outcomes.