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Timely Intervention in Light Chain Cardiac Amyloidosis
Annas Rahman1, Firas Anaya1, Bala Pushparaji1
1Division of Cardiovascular Medicine, Heart and Vascular Institute, MetroHealth Medical Center/Case Western Reserve University, Cleveland, Ohio, USA.
JACC. Case Reports
|August 15, 2025
Summary
Light-chain cardiac amyloidosis (AL-CA) is often misdiagnosed, delaying treatment. Prompt diagnosis and tailored therapy, like Dara-CyBorD, can lead to remission and improved heart function in AL-CA patients.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Light-chain cardiac amyloidosis (AL-CA) is frequently misdiagnosed as diastolic dysfunction, potentially worsening patient outcomes.
- Delayed diagnosis of AL-CA can lead to a poor prognosis in heart failure with preserved ejection fraction cases.
Observation:
- A 70-year-old male with suspected diastolic dysfunction was evaluated.
- Multimodality imaging and endomyocardial biopsy were crucial for diagnosing AL-CA.
- A multidisciplinary team approach facilitated prompt diagnosis and treatment.
Findings:
- The patient received Dara-CyBorD (daratumumab, cyclophosphamide, bortezomib, and dexamethasone) therapy.
- Complete remission was achieved within 5 months.
- Stabilization and potential reversal of myocardial dysfunction were observed.
Implications:
- Early recognition of AL-CA's clinical features is vital for differentiating it from other diastolic dysfunction causes.
- Timely diagnosis and assessment of infiltrative burden guide individualized hematologic treatment.
- Multidisciplinary management and intensive therapies improve AL-CA prognosis.
Keywords:
cardiomyopathydiastolic heart failureechocardiographyimagingrestrictiveright-sided catheterizationstrain rate imagingMore Related Videos
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