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Diastolic Dysfunction Unveiling Cardiac Light-Chain Amyloidosis: A Case Report
Somesh Saha1, Ritwick Mondal2, Shramana Deb3
1Department of Critical Care Medicine, Belle Vue Clinic, Kolkata, India.
Insights
Cardiac light-chain amyloidosis significantly impacts survival, with deposition extent being key. Early diagnosis and treatment are crucial for halting progression and improving patient outcomes in this serious condition.
Area of Science:
- Cardiology
- Internal Medicine
- Nephrology
Background:
- Cardiac light-chain amyloidosis is a critical, multi-systemic disease.
- The extent of cardiac free light-chain deposition is the primary determinant of survival.
Purpose of the Study:
- To highlight the importance of early diagnosis and prompt treatment for cardiac amyloidosis.
- To present a case illustrating the diagnostic process and clinical presentation of cardiac amyloidosis.
Main Methods:
- Case report of a 67-year-old male with relevant medical history.
- Diagnostic workup included 12-lead electrocardiogram, 2D echocardiography, cardiac magnetic resonance imaging, nuclear imaging, and biopsy.
Main Results:
- The patient presented with symptoms suggestive of cardiac compromise.
- Diagnostic imaging and biopsy confirmed cardiac light-chain amyloidosis.
- Key findings included left ventricular hypertrophy, restrictive filling, and pericardial effusion.
Conclusions:
- Timely recognition and a high index of suspicion are essential for early diagnosis.
- Prompt diagnosis allows for initiation of definitive therapy.
- Effective treatment can halt disease progression and significantly improve prognosis.
Background:
Cardiac light-chain amyloidosis represents a critical component of this multi-systemic disease, significantly impacting prognosis. The extent of cardiac free light-chain deposition is the primary determinant of survival.
Case Presentation:
We report the case of a 67-year-old male with a 10-year history of diabetes mellitus and arterial hypertension who presented with a two-day history of chest discomfort and difficulty lying down or sleeping, along with a two-month history of progressively worsening exertional dyspnea. On examination, the patient exhibited low blood pressure. A 12-lead electrocardiogram revealed poor R-wave progression and left ventricular hypertrophy. Further evaluation using 2D echocardiography demonstrated significant concentric left ventricular hypertrophy, a restrictive filling pattern, and mild pericardial effusion. Cardiac magnetic resonance imaging, nuclear imaging, and biopsy confirmed the diagnosis of cardiac light-chain amyloidosis.
Conclusion:
Timely recognition and a high index of suspicion are essential for the early diagnosis of cardiac amyloidosis. Prompt diagnosis enables the initiation of definitive therapy, which may halt disease progression and significantly improve prognosis.
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