Journal article
AL type cardiac amyloidosis: a devastating fatal disease
Journal of community hospital internal medicine perspectives, v 11(3), pp 407-412
04 May 2021
PMID: 34234917
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Cardiac amyloidosis is a rare entity with a grave prognosis. Due to the low index of suspicion secondary to non-specific symptoms, it is often diagnosed at an advanced stage with multi-organ involvement.
We report a case of systemic AL amyloidosis with predominant cardiac and renal involvement associated with multiple myeloma.
A 60-year-old male presented with progressive anasarca, orthopnea and weight gain over 8 months. On clinical examination, 3+ pitting edema was found in bilateral extremities and scrotum. Serum N-type proBNP and troponin T were elevated, and EKG showed diffuse low voltage QRS, right axis deviation, and 1
st
degree AV block. Echocardiography revealed granular myocardium, biventricular hypertrophy, bi-atrial dilation and apical sparing pattern on global longitudinal strain which was suggestive of cardiac amyloidosis. Light chain assessment showed elevated kappa and lambda chains with kappa to lambda ratio of 16.2. Endomyocardial biopsy revealed AL type cardiac amyloidosis, and bone marrow biopsy confirmed the diagnosis of multiple myeloma. He received six cycles of bortezomib, cyclophosphamide, and dexamethasone but continued to deteriorate. He experienced an episode of cardiac arrest following which he had a return of spontaneous circulation but due to poor prognosis, the family opted for pursuing comfort measures only.
Cardiac involvement in AL type amyloidosis imparts significant morbidity and mortality. The management of cardiac amyloidosis entails a multidisciplinary approach with an emphasis on cardiology and oncology. Despite the novel diagnostic modalities and treatment regimens, the outcome for AL-type cardiac amyloidosis remains poor.
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Details
- Title
- AL type cardiac amyloidosis: a devastating fatal disease
- Creators
- Adeel Nasrullah - Allegheny Health NetworkAnam Javed - Allegheny Health NetworkThejus T Jayakrishnan - Allegheny Health NetworkAaron Brumbaugh - Allegheny Health NetworkAriel Sandhu - Allegheny Health NetworkBrent Hardman - Allegheny Health Network
- Publication Details
- Journal of community hospital internal medicine perspectives, v 11(3), pp 407-412
- Publisher
- Taylor & Francis
- Number of pages
- 6
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Radiology (Radiologic Sciences); Emergency Medicine; General Internal Medicine
- Web of Science ID
- WOS:000648740100025
- Other Identifier
- 991022084053904721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Cardiac & Cardiovascular Systems