Key result
Thickened ventricular walls on echo and low QRS voltage characterize cardiac AL amyloidosis in CHF.
Why the study?
Cardiac involvement is the most common cause of death in immunoglobulin light-chain amyloidosis, but diagnosis is often delayed and frequently established only postmortem.
Case Report (n=4)
No
Echocardiography and electrocardiography can effectively identify cardiac involvement in AL amyloidosis, which should be considered in patients presenting with congestive heart failure.
May prompt consideration of cardiac AL amyloidosis in heart failure with thick walls and low voltage; case series leaves open need for validation.
In immunoglobulin light-chain (L) amyloidosis the cause of death is most commonly due to cardiac involvement. Cardiac amyloidosis is frequently diagnosed by postmortem examination. There are many reports of diagnoses of cardiac amyloidosis established by autopsy of cardiac tissue. We report four patients whom we could diagnose as having cardiac amyloidosis by echocardiogram and electrocardiogram. They were referred to our hospital because of congestive heart failure. All patients had thickened ventricular walls, increased myocardial echogenicity, and normal ventricular chamber size on echocardiogram. Our 4 cases showed low voltage and pseudoinfarction pattern on electrocardiogram. Biopsies from the myocardium, rectal mucosa and/or abdominal wall revealed extracellular deposition of amyloid in all cases. Two cases were associated with myeloma and the other two cases were primary amyloidosis. Cardiac involvement from amyloid light-chain (AL) amyloidosis is rapidly fatal. Unfortunately, the diagnosis of AL amyloidosis is often delayed. AL amyloidosis should be considered as a cause of congestive heart failure and requires further investigation.
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A 1999 study conducted a case report in Immunoglobulin Light-Chain Systemic Amyloidosis with cardiac involvement (n=4). Melphalan and prednisolone was evaluated. Four patients presenting with congestive heart failure were diagnosed with cardiac AL amyloidosis characterized by thickened ventricular walls on echocardiogram and low QRS voltage on electrocardiogram.
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