Liu Hui, Fu Hang, Guo Ying-Kun, Yang Zhi-Gang, Xu Hua-Yan, Shuai Xiao, Xu Rong, Li Zhen-Lin, Xia Chun-Chao, He Yong, Zhou Xiao-Yue
Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China.
Department of Radiology, National Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu 610041, China.
Cardiovasc Diagn Ther. 2020 Apr;10(2):161-172. doi: 10.21037/cdt.2020.01.03.
Early detection of right ventricular (RV) dysfunction is vital for determining the prognosis of light-chain amyloidosis (AL) patients. While few studies focused on RV deformation due to the limitation of research methods. The aim of this study was to determine the prognostic significance of RV myocardial strain in AL patients assessed by cardiac magnetic resonance (CMR) tissue tracking.
Sixty-four AL patients (28 females and 36 males, mean age 58±12.8 years old; range 25-81 years old) were enrolled from 1 October 2014 through 31 March 2017 and compared with 20 age- and sex-matched controls. Fifty-one AL patients met the criteria for cardiac amyloidosis (CA). Deformation parameters of both RV and left ventricle (LV) were measured by the CMR tissue tracking technique including myocardial global radial peak strain (GRPS), global circumferential peak strain (GCPS), and global longitudinal peak strain (GLPS). The follow-up time was 20 months or until the occurrence of death.
Thirty-two (50%) had preserved RV ejection fraction (RVEF ≥45%). AL patients had significantly lower RV-GRPS (20.3±2.12 31.31±7.61), GCPS (-2.12±0.88 -13.71±2.53), and GLPS (-5.33±0.64 -14.239±2.99) than controls even RVEF remain preserved (all P<0.001). Compared with controls and patients without CA, RV-GRPS (12.26±1.26 29.72±3.54, P<0.001) and RV-GLPS (-3.78±2.25 -5.66±2.08, P<0.05) were significantly lower in patients with CA. Cox multivariate analyses demonstrated that RV-GRPS [hazard ratio (HR) =0.93, 95% CI: 0.88-0.98, P=0.007] and Mayo stage were (HR =3.11, 95% CI: 1.30-7.41, P=0.01) predictors of mortality in AL patients.
CMR tissue tracking is a feasible and highly reproducible technique for the analysis of RV deformation and could aid in the early diagnosis of RV involvement in AL patients. RV-GRPS of RV strain and Mayo stage provides prognostic information about mortality in AL patients.
早期发现右心室（RV）功能障碍对于确定轻链淀粉样变性（AL）患者的预后至关重要。然而，由于研究方法的限制，很少有研究关注RV变形。本研究的目的是通过心脏磁共振（CMR）组织追踪来确定RV心肌应变在AL患者中的预后意义。
从2014年10月1日至2017年3月31日招募了64例AL患者（28例女性和36例男性，平均年龄58±12.8岁；范围25 - 81岁），并与20例年龄和性别匹配的对照进行比较。51例AL患者符合心脏淀粉样变性（CA）标准。通过CMR组织追踪技术测量RV和左心室（LV）的变形参数，包括心肌整体径向峰值应变（GRPS）、整体圆周峰值应变（GCPS）和整体纵向峰值应变（GLPS）。随访时间为20个月或直至死亡发生。
32例（50%）患者的右心室射血分数（RVEF）保留（RVEF≥45%）。即使RVEF保留，AL患者的RV - GRPS（20.3±2.12对31.31±7.61）、GCPS（-2.12±0.88对-13.71±2.53）和GLPS（-5.33±0.64对-14.239±2.99）仍显著低于对照组（所有P<0.001）。与对照组和无CA的患者相比，CA患者的RV - GRPS（12.26±1.26对29.72±3.54，P<0.001）和RV - GLPS（-3.78±2.25对-5.66±2.08，P<0.05）显著降低。Cox多因素分析表明，RV - GRPS[风险比（HR）=0.93，95%置信区间：0.88 - 0.98，P = 0.]和梅奥分期（HR =，95%置信区间：1.30 - 7.41，P = 0.01）是AL患者死亡率的预测因素。
CMR组织追踪是一种用于分析RV变形的可行且高度可重复的技术，有助于早期诊断AL患者的RV受累情况。RV应变的RV - GRPS和梅奥分期可提供AL患者死亡率的预后信息。
