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[Echocardiographic image of cardial amyloidosis]
1Klinika Choroby Wieńcowej Instytutu Kardiologii, Warszawa.
Insights
Cardiac amyloidosis, a serious heart condition, frequently occurs with plasma cell disorders like plasmacytoma. Echocardiography revealed characteristic heart changes, confirming the diagnosis in a patient with plasmacytoma, hepatomegaly, and nephropathy.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Systemic amyloidosis commonly affects the heart, particularly in patients with plasma cell dyscrasias (e.g., plasmacytoma, monoclonal gammopathy, Waldenstrom's disease) and chronic inflammatory conditions.
- Cardiac involvement in acquired systemic amyloidosis ranges from 54% in inflammatory states to nearly 90% in immunological disorders associated with plasma cell neoplasms.
Observation:
- A case study presented a patient with cardiac amyloidosis, hepatomegaly, and nephropathy secondary to plasmacytoma.
- The patient was initially referred for an echocardiogram due to unstable postinfarction angina.
Findings:
- Echocardiography demonstrated findings characteristic of cardiac amyloidosis, including concentric biventricular hypertrophy.
- Additional echocardiographic findings included impaired global contractility, thickened intra-atrial septum, and pericardial thickening with minimal effusion.
- The echocardiographic diagnosis was subsequently confirmed by anatomopathological examination.
Implications:
- This case highlights the importance of echocardiography in diagnosing cardiac amyloidosis, even when cardiac symptoms are initially attributed to other conditions like coronary artery disease.
- Early and accurate diagnosis of cardiac amyloidosis is crucial for appropriate management and can be facilitated by characteristic echocardiographic findings.
- Coexistence of cardiac amyloidosis with other organ involvement (hepatomegaly, nephropathy) in plasmacytoma underscores the systemic nature of the disease.
Abstract:
Heart is involved nearly in 90% of acquired, systemic amyloidosis in a course of plasmocytoma, monoclonal gammapathy, Waldenstrom's disease and others immunological disorders. In classical acquired systemic amyloidosis due to chronic bacterial infection and other inflammatory states heart involvement is observed in 54% of patients. Authors presented a case of cardiac amyloidosis coexisting with hepatomegaly and nephropathy due to plasmocytoma. Diagnosis was based on the echocardiographic examination to which the patient was sent as the unstable postinfarction angina. Echocardiographic image typical of amyloidosis revealed such changes as: concentric both ventricular hypertrophy, generalized contractility impairment, thickness of intraatrial septum and pericardium with minimal effusion. Echocardiographic diagnosis was proved by the anatomopathologic examination.