Right ventricular involvement evaluated by cardiac magnetic resonance imaging predicts mortality in patients with light chain amyloidosis

Few studies have focused on right ventricular (RV) involvement in cardiac amyloidosis (CA). We investigated the prognostic value of RV assessment by cardiovascular magnetic resonance (CMR) in CA. In 2011–2014, consecutive patients with suspected CA referred for CMR were retrospectively evaluated. Demographic and baseline clinical characteristics were collected. Healthy volunteers were matched for sex and age and served as controls. All subjects underwent a contrast-enhanced CMR examination. RV size, function, and late gadolinium enhancement (LGE) were analyzed. All deaths during follow-up were recorded. Sixty-one patients [37 males (60.7%), age 60 ± 11 years] were included; CA was diagnosed in 47 (77.0%) patients. CA patients displayed decreased biventricular ejection fraction, elevated left ventricular mass index, and increased biventricular end-systolic volume index (ESVi) compared with controls. A total of 27 deaths (57.4%) occurred in the CA group at 21-month median follow-up. Multivariable analysis demonstrated that RVESVi (HR 1.033, 95% CI 1.004–1.063, P = 0.026) and RV-LGE (HR 2.814, 95% CI 1.063–7.450, P = 0.037) were independent predictors of mortality in CA. For all amyloid patients, log NT-proBNP (HR 3.412; 95% CI 1.484–7.845; P = 0.004) and RV-LGE (HR 4.149; 95% CI 1.623–10.607; P = 0.003) were identified as independent predictors. RVESVi and RV-LGE are independent predictors of survival and evaluation of RV by CMR enables risk stratification in patients with CA.