Extracellular volume fraction by computed tomography predicts long-term prognosis among patients with cardiac amyloidosis. (3rd October 2022)

• Record Type: Journal Article
• Authors: Gama, F; Rosmini, S; Bandula, S; Patel, K P; Thornton, G D; Bennett, J B; Wechelakar, A; Gillmore, J D; Whelan, C; Lachmann, H; Taylor, S; Fontana, M; Moon, J; Hawkins, P N; Treibel, T
• Is Part Of: European heart journal. Volume 43(2022)Supplement 2
• Publication Date: 2022-10-03
• Subjects: Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/eurheartj/ehac544.184
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Objective: This study sought to investigate the association of extracellular volume fraction by computed tomography (ECVCT), myocardial remodeling and mortality in patients with systemic amyloidosis. Background: Light chain (AL) and transthyretin (ATTR) amyloid fibrils are deposited in the extracellular space of the myocardium, resulting in heart failure and premature mortality. Extracellular expansion can be quantified by CT, offering a rapid and cost-effective alternative to cardiovascular magnetic resonance (CMR), especially among patients with cardiac devices or on renal dialysis. Methods: Patients with confirmed systemic amyloidosis and varying degrees of cardiac involvement underwent ECG-gated cardiac CT. ECVCT was analysed in the inter-ventricular septum. All patients also underwent clinical assessment, ECG, echocardiography, serum amyloid protein component (SAP) and/or technetium-99m (99mTc) 3, 3-diphosphono-1, 2-propanodicarboxylic acid scintigraphy. ECVCT was compared across different extents of cardiac infiltration (ATTR Perugini Grade / AL Mayo Class) and evaluated for its association with myocardial remodelling and all-cause mortality. Results: 72 patients were studied (AL n=35, ATTR n=37; age 67 (59–76) years, 71% males). Mean septal ECVCT was 42.7±13.1% and 55.8±10.9% in AL and ATTR, respectively, and correlated with indexed left ventricular (LV) mass (r=0.426, p<0.001), LV ejection fraction [LVEF, (r=0.460, p<0.001)], NT-proBNP (r=0.563, p<0.001) and hsTnT (r=0.546, p=0.02). ECVCT increased with cardiac amyloid involvement in both AL and ATTR (Figure 1). Over a mean follow-up of 5.3±2.4 years, 40 deaths occurred (AL 14 [35%]; ATTR 26 [65%]). ECVCT was independently associated with all-cause mortality in ATTR (not AL) after adjustment for age and IV septal wall thickness (HR: 1.046, 95% CI: 1.003–1.090, p=0.037). Conclusion: Cardiac amyloid burden quantified by ECVCT is associated with adverse cardiac remodelling as well as all-cause mortality among ATTR amyloid patients. ECVCT may address the need for better identification and risk stratification of amyloid patients, using a widely-accessible imaging modality (Figure 2). Funding Acknowledgement: Type of funding sources: None.