Abstract 3206: Prognostic Utility of Delayed Enhancement Magnetic Resonance Imaging in Patients With Systemic Amyloidosis and Suspected Cardiac Involvement
Background: In patients with systemic amyloidosis cardiac involvement is associated with high morbidity and mortality. We hypothesized delayed enhancement cardiac magnetic resonance (DE-CMR) would identify patients with cardiac involvement and those at risk of death or transplantation.
Methods: 45 consecutive patients with biopsy proven systemic amyloidosis and suspected cardiac involvement had DE-CMR performed (1.5T scanner). TI (time from inversion) scouts were performed to identify myocardial and blood pool null points. Standard DE-MR imaging was performed 10 minutes after injection of 0.15 mmol/kg gadolinium using the longest TI that nulled a myocardial region or the blood pool, which-ever was longest. Scans were interpreted by consensus of 2 investigators masked to all clinical data for the presence of diffuse DE (DDE) consistent with an infiltrative pattern. Patients were clinically followed for the occurrence of death or heart failure leading to transplantation. Results: Mean age was 63.0 +/− 11.1 years (32 male, 13 female). Thirty-one (68.8%) patients had evidence of DDE on DE-CMR imaging. At a mean follow-up of 339 days 20 (44.4%) patients died and 2 (4.4%) underwent cardiac transplantation due to heart failure. Patients with DDE had a 64.5% risk of death or transplantation during follow up compared to 14.3% in those without DDE (HR 6.71, 95% CI: 1.58, 8.79, p=0.0027). Endomyocardial tissue was obtained in 13 patients and was positive for amyloid in 10. DDE was present in all 10 patients.
Conclusion: In patients with systemic amyloidosis and suspected cardiac amyloid DE-MR identifies a group of patients at a high risk of death or transplantation.