Mechanisms of left ventricular systolic dysfunction in light chain amyloidosis: a multiparametric cardiac MRI study

• Published in: Frontiers in cardiovascular medicine 2024-05, Vol.11, p.1371810
• Main Authors: Katznelson, Ethan, Jerosch-Herold, Michael, Cuddy, Sarah A M, Clerc, Olivier F, Benz, Dominik C, Taylor, Alexandra, Rao, Shivani, Kijewski, Marie Foley, Liao, Ronglih, Landau, Heather, Yee, Andrew J, Ruberg, Frederick L, Di Carli, Marcelo F, Falk, Rodney H, Kwong, Raymond Y, Dorbala, Sharmila
• Format: Article
• Language: English
• Subjects: cardiac amyloidosis; cardiac MRI (CMR); Cardiovascular Medicine; extracellular volume; myocardial blood flow; myocardial work efficiency
• ISSN: 2297-055X; 2297-055X
• DOI: 10.3389/fcvm.2024.1371810

Cardiac systolic dysfunction is a poor prognostic marker in light-chain (AL) cardiomyopathy, a primary interstitial disorder; however, its pathogenesis is poorly understood. This study aims to analyze the effects of extracellular volume (ECV) expansion, a surrogate marker of amyloid burden on myocardial blood flow (MBF), myocardial work efficiency (MWE), and left ventricular (LV) systolic dysfunction in AL amyloidosis. Subjects with biopsy-proven AL amyloidosis were prospectively enrolled (April 2016-June 2021; Clinicaltrials.gov ID NCT02641145) and underwent cardiac magnetic resonance imaging (MRI) to quantify rest MBF by perfusion imaging, LV ejection fraction (LVEF) by cine MRI, and ECV by pre- and post-contrast T1 mapping. The MWE was estimated as external cardiac work from the stroke volume and mean arterial pressure normalized to the LV myocardial mass. Rest MBF in 92 subjects (62 ± 8 years, 52 men) with AL amyloidosis averaged 0.87 ± 0.21 ml/min/g and correlated with MWE (  = 0.42;