Diagnostic and Prognostic Value of Non-late Gadolinium Enhancement Cardiac Magnetic Resonance Parameters in Cardiac Amyloidosis

Abstract

Early diagnosis is crucial for the improvement of outcomes of patients with cardiac amyloidosis (CA). Emerging non-late gadolinium enhancement (LGE) based cardiac magnetic resonance (CMR) parameters may facilitate early identification of CA. We sought to investigate the diagnostic and prognostic value of T1, T2 mapping and extracellular volume (ECV) in CA. This single-center prospective analysis included 88 patients with CA, 33 patients with aortic stenosis (AS) and left ventricular hypertrophy (LVH), and 15 healthy controls who completed 3T cardiac MRI at the time of their diagnosis and were assessed with T1, T2 (modified Look-Locker inversion recovery), and ECV mapping of the heart and spleen. Echocardiographic, and biochemical parameters and clinical characteristics and outcomes were collected and analyzed. Of the patients with CA, 71 had light-chain (AL) and 17 had transthyretin (ATTR) amyloidosis. Native T1, native T2 and ECV were significantly higher in patients with CA compared to both patients with LVH-AS (P<0.001) and healthy controls (P<0.001). Good diagnostic accuracy was also demonstrated by measuring the area under the curve (AUC) of the receiver operating characteristic (ROC) curves for native T1 in the region of interest (ROI) (AUC=0.90), native T2 ROI (AUC=0.88), and ECV (AUC=0.90). Furthermore, native T1 ROI, native T2 ROI and ECV, correlated with both NT-proBNP levels and Mayo stage of patients (with AL). Spleen ECV was significantly increased in patients with AL versus ATTR amyloidosis (38.5 vs 30.5; P=0.004) and demonstrated good diagnostic accuracy in differentiating between the two types (AUC=0.79). Native T2 ROI was prognostic of mortality in AL CAwith a HR of 1.97 per 5 ms increase (P=0.001) and remained prognostic after adjustment for age, and Mayo stage. Non-LGE based CMR techniques correlated with established markers of disease and demonstrated good diagnostic accuracy, while native T2 ROI was also prognostic of mortality, thus reinforcing their use in the diagnosis and prognosis of CA.