Regression of Cardiac Amyloidosis After Stem Cell Transplantation Assessed by Cardiovascular Magnetic Resonance Imaging
A previously fit 52-year-old man presented with severe progressive exertional dyspnea. He was in heart-failure with an elevated jugular-venous-pressure, edema, and increased plasma-NTpro-BNP levels of 4285 μg/mL (upper limit of normal <900 μg/mL). His ECG demonstrated sinus-rhythm with low limb and chest lead voltages (Figure 1). He had significant proteinuria with renal and bone marrow biopsies confirming light-chain amyloidosis. Cardiac magnetic resonance imaging revealed concentric left-ventricular hypertrophy with an ejection fraction of 65%, left-ventricular end-diastolic volume of 146 mL, left-ventricular end-systolic volume of 51 mL, left-ventricular mass of 245 g, and left-atrial volume of 144 mL (Figure 2, Movie I in the online-only Data Supplement). Late gadolinium-enhanced imaging showed extensive diffuse subendocardial hyperenhancement in both ventricles (Figures 3 and 4, arrows), consistent with amyloid infiltration. He subsequently underwent successful autologous stem cell transplantation.
At follow-up, 2.5 years later, his functional status had markedly improved and he was exercising regularly. His cardiovascular examination and plasma-NTpro-BNP level (117 μg/mL) was normal. His ECG showed some recovery of voltages in the limb leads (Figure 5). Repeat cardiac magnetic resonance imaging showed minimal change in left-ventricular volumes, function, and mass (left-ventricular end-diastolic volume, 138 mL; left-ventricular end-systolic volume, 43mL; ejection fraction, 69%; left-ventricular mass, 235 g), although left atrial volume was significantly reduced (105 mL; Figure 6, Movie I in the online-only Data Supplement). Late gadolinium-enhanced imaging demonstrated marked regression of the subendocardial hyperenhancement (Figures 7 and 8).
Prognosis for patients with light-chain amyloid and cardiac infiltration has historically been dismal, and extensive cardiac involvement has generally been regarded as a contraindication to stem cell transplantation.1–3 This case suggests that stem cell transplantation can lead to regression of cardiac amyloid and may be considered in selected patients.
Sources of Funding
Dr Brahmanandam was supported by National Institutes of Health grant T32HL072742.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.114.009135/-/DC1.
- © 2014 American Heart Association, Inc.