Abstract 1971: Delayed Gadolinium Enhancement as a Novel Prognostic Marker in Patients with Cardiac Amyloidosis
Introduction: Cardiac magnetic resonance (CMR) has recently proven valuable for the diagnosis of cardiac amyloidosis (CA). The purpose of this study was to determine the prognostic significance of late gadolinium enhancement (LGE) in pts with amyloid light chain (AL) and transthyretin (ATTR) amyloidosis.
Methods: CMR was performed on a 1.5 T clinical scanner (Intera Achieva®, Philips Medical Systems) in 34 pts with biopsy proven AL (n= 27) or ATTR (n=7) amyloidosis. Intensity of LGE was graded visually on a four point scale by two blinded observers using the AHA 17 segment model. A LGE severity score (“LGE score”) was calculated for each patient by dividing the sum of intensities by the number of evaluated segments. All pts were followed for the combined endpoint of cardiac death, heart transplantation and ICD implantation for mean of 338 days.
Results: LGE was present in 81% of patients. Pts with CA displayed a higher number of segments with LGE (10.76± 1.53 vs. 2.17± 0.87, p= 0.007) and a higher LGE score (1.29± 0.21 vs. 0.19± 0.69, p< 0.001). While there was no correlation of LGE score and left ventricular EF, EDV and EDV, a strong relation between LGE score and LV mass (R=0.55, p= 0.005) and serum levels of NT-proBNP ( R= 0.46, p= 0.02) could be found. Pts who reached the combined endpoint (n=7) had a higher LGE score (1.59± 0.42 vs. 0.61± 0.21, p= 0.02). ROC analysis identified an LGE score of < 0.58 as best discriminator for event free survival (Fig 1⇓).
Conclusions: Our results demonstrate that severity and extent of late gadolinium enhancement have prognostic implications in pts with cardiac amyloidosis. This novel finding may aid to identify high risk patients and guide treatment decisions.