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

Ke Wan,Dan Yang,Jiayu Sun,Yuchi Han,Hong Liu,Zhi Zeng,Yong Luo,Wei Cheng,Qing Zhang,Yucheng Chen

doi:10.1007/s00380-017-1043-y

Abstract

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.

Highlights

Amyloidosis is a group of rare diseases characterized by extracellular deposition of insoluble abnormal fibrillar proteins derived from various precursor proteins that leads to multi-organ structural alterations and functional impairment [1]
RVESVi (HR 1.033, 95% CI 1.004–1.063, P = 0.026 combined model) and right ventricular (RV)-late gadolinium enhancement (LGE) (HR 2.814, 95% CI 1.063–7.450, P = 0.037 combined model) remained significantly associated with the primary outcome when all significant variables of clinical, laboratory, and Cardiovascular magnetic resonance (CMR) model were added to the model
Our results indicate that higher RVESVi and positive RV-LGE portend a worse prognosis in patients with AL cardiac amyloidosis (CA)

Summary

Introduction

Amyloidosis is a group of rare diseases characterized by extracellular deposition of insoluble abnormal fibrillar proteins derived from various precursor proteins that leads to multi-organ structural alterations and functional impairment [1]. Most studies have emphasized the importance of left ventricular (LV) diastolic dysfunction in CA, but the value of the right ventricular (RV) function is usually ignored [3,4,5,6]. Some studies that evaluated RV systolic performance by Doppler myocardial imaging and speckle-tracking imaging have shown an association of RV systolic dysfunction with poor prognosis in CA [7,8,9]. Cardiovascular magnetic resonance (CMR) imaging is the reference modality for the quantification of RV volumes and systolic function [10]. CMR has emerged as a high specificity, noninvasive tool for the diagnosis of CA, with late gadolinium enhancement (LGE) reflecting the distribution of amyloid infiltration in the extracellular space

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