Abstract 19388: Cardiovascular Magnetic Resonance in Cardiac Amyloidosis: A 242 Patient Prospective Study
Introduction: Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement technique (LGE) is a candidate reference standard for non invasive diagnosis of light chain and transthyretin cardiac amyloidosis (AL, ATTR). However, conflicting results on morphology and LGE have been reported in small retrospective studies.
Hypothesis: that morphology and LGE can guide differential diagnosis and predict survival in AL and ATTR amyloidosis.
Methods: 242 patients were recruited: 116 with AL, 126 with ATTR (8 of which were mutations carriers). All subjects underwent CMR with standard cine imaging, LGE (with and without phase sensitive inversion recovery, PSIR) and T1 mapping with Extracellular Volume (ECV) measurement.
Results: Compared to AL, ATTR had more LVH, more asymmetry, more LV impairment and more RVH. Using the ECV as truth standard, in 43% initial non-PSIR had incorrect initial regional or global nulling with the highest ECV myocardium displayed as nulled. PSIR always displayed areas of greatest interstitial expansion as bright (P<0.0001), even when pan-myocardial (Figure 1A). PSIR LGE in AL and ATTR could be divided into three patterns: none (34% vs 13%, p<0.05), subendocardial (40% vs 25%, p<0.05) and transmural (26% vs 62%, p<0.0001). This tracked increasing amyloid burden measured as ECV (and DPD grade in ATTR), p<0.0001. During follow-up, 40 deaths occurred, 29 in AL and 11 in ATTR. Transmural LGE predicted death in AL (HR 3.7, 95% CI: 1.7-8.1, P<0.001) (Figure 1B) and remained independent after adjusting for NT-proBNP, EF and native T1 (HR = 4.0, 95% CI: 1.2-12.8). In ATTR, all cardiac deaths happened in transmural patients except for one that was classified discordantly by the two observers (subendocardial and transmural).
Conclusion: AL and ATTR have different morphology and prevalence of LGE pattern. PSIR LGE is essential. A transmural PSIR LGE pattern predicts death.
Author Disclosures: M. Fontana: None. S. Pica: None. P. Reant: None. A. Abdel-Gadir: None. T.A. Treibel: None. S.M. Banypersad: None. V. Maestrini: None. T. Lane: None. H.J. Lachmann: None. C.J. Whelan: None. A.D. Wechalekar: None. C.H. Manisty: None. A.S. Herrey: None. J.D. Gillmore: None. P.N. Hawkins: None. J.C. Moon: None.
- © 2014 by American Heart Association, Inc.