BACKGROUND: Patients with AL amyloidosis and congestive heart failure have a very poor prognosis. To date, the recovery of these patients has not been described in detail. OBJECTIVE: To determine the frequency and characteristics regression of disease in patients with congestive heart failure due to AL amyloidosis. DESIGN: Review of patients with systemic AL amyloidosis. SETTING: An international referral center for amyloidosis in the United States. PATIENTS: 140 patients with congestive heart failure due to Al amyloidosis who were seen between 1983 and 1994. MEASUREMENTS: Functional status, Doppler echocardiography, and objective measurements of disease activity. RESULTS: 3 of 140 patients (2.1%) had marked resolution of congestive heart failure and evidence for remission of disease activity. All 3 had been treated with melphalan. CONCLUSIONS: Melphalan appears to have had a favorable effect in 3 patients with AL amyloidosis and heart failure. The abolition of light chains that was seen in these 3 patients suggests that light-chain toxicity may play a role in the genesis of heart failure in patients with AL amyloidosis.
BACKGROUND:Patients with AL amyloidosis and congestive heart failure have a very poor prognosis. To date, the recovery of these patients has not been described in detail. OBJECTIVE: To determine the frequency and characteristics regression of disease in patients with congestive heart failure due to AL amyloidosis. DESIGN: Review of patients with systemic AL amyloidosis. SETTING: An international referral center for amyloidosis in the United States. PATIENTS: 140 patients with congestive heart failure due to Al amyloidosis who were seen between 1983 and 1994. MEASUREMENTS: Functional status, Doppler echocardiography, and objective measurements of disease activity. RESULTS: 3 of 140 patients (2.1%) had marked resolution of congestive heart failure and evidence for remission of disease activity. All 3 had been treated with melphalan. CONCLUSIONS:Melphalan appears to have had a favorable effect in 3 patients with AL amyloidosis and heart failure. The abolition of light chains that was seen in these 3 patients suggests that light-chain toxicity may play a role in the genesis of heart failure in patients with AL amyloidosis.
Authors: Raymond Q Migrino; Seth Truran; David D Gutterman; Daniel A Franco; Megan Bright; Brittany Schlundt; Mitchell Timmons; Angelica Motta; Shane A Phillips; Parameswaran Hari Journal: Am J Physiol Heart Circ Physiol Date: 2011-09-30 Impact factor: 4.733