National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom.
Department of Statistical Science, University College London, United Kingdom.
JACC Cardiovasc Imaging. 2019 May;12(5):823-833. doi: 10.1016/j.jcmg.2018.02.016. Epub 2018 Apr 18.
This cross-sectional study aimed to describe the functional and structural cardiac abnormalities that occur across a spectrum of cardiac amyloidosis burden and to identify the strongest cardiac functional and structural prognostic predictors in amyloidosis using cardiac magnetic resonance (CMR) and echocardiography.
Cardiac involvement in light chain and transthyretin amyloidosis is the main driver of prognosis and influences treatment strategies. Numerous measures of cardiac structure and function are assessed by multiple imaging modalities in amyloidosis.
A total f 322 subjects (311 systemic amyloidosis and 11 transthyretin gene mutation carriers) underwent comprehensive CMR and transthoracic echocardiography. The probabilities of 11 commonly measured structural and functional cardiac parameters being abnormal with increasing cardiac amyloidosis burden were evaluated. Cardiac amyloidosis burden was quantified using CMR-derived extracellular volume. The prognostic capacities of these parameters to predict death in amyloidosis were assessed using Cox proportional hazards models.
Left ventricular mass and mitral annular plane systolic excursion by CMR along with strain and E/e' by echocardiography have high probabilities of being abnormal at low cardiac amyloid burden. Reductions in biventricular ejection fractions and elevations in biatrial areas occur at high burdens of infiltration. The probabilities of indexed stroke volume, myocardial contraction fraction, and tricuspid annular plane systolic excursion (TAPSE) being abnormal occur more gradually with increasing extracellular volume. Ninety patients (28%) died during a median follow-up of 22 months (interquartile range: 10 to 38 months). Univariable analysis showed that all imaging markers studied significantly predicted outcome. Multivariable analysis showed that TAPSE (hazard ratio: 1.46; 95% confidence interval: 1.16 to 1.85; p < 0.01) and indexed stroke volume (hazard ratio: 1.24; 95% confidence interval: 1.04 to 1.48; p < 0.05) by CMR were the only independent predictors of mortality.
Specific functional and structural abnormalities characterize different burdens of cardiac amyloid deposition. In a multimodality imaging assessment of a large cohort of amyloidosis patients, CMR-derived TAPSE and indexed stroke volume are the strongest prognostic cardiac functional markers.
本横断面研究旨在描述各种心脏淀粉样变负荷下发生的功能和结构心脏异常，并使用心脏磁共振（CMR）和超声心动图确定淀粉样变中最强的心脏功能和结构预后预测因子。
轻链和转甲状腺素蛋白淀粉样变中的心脏受累是预后的主要驱动因素，并影响治疗策略。在淀粉样变性中，多种成像方式评估心脏结构和功能的多项指标。
共 322 例患者（311 例系统性淀粉样变性和 11 例转甲状腺素基因突变携带者）接受了全面的 CMR 和经胸超声心动图检查。评估了随着心脏淀粉样变负荷增加，11 种常见测量的结构和功能心脏参数异常的概率。使用 CMR 衍生的细胞外容积量化心脏淀粉样变负荷。使用 Cox 比例风险模型评估这些参数预测淀粉样变死亡的预后能力。
CMR 的左心室质量和二尖瓣环平面收缩期位移以及超声心动图的应变和 E/e'在低心脏淀粉样变负荷时异常的可能性较高。在高浸润负荷下，双心室射血分数降低和双心房面积升高。随着细胞外容积的增加，指数化的每搏量、心肌收缩分数和三尖瓣环平面收缩期位移（TAPSE）异常的概率逐渐增加。90 例患者（28%）在中位数为 22 个月（四分位距：10 至 38 个月）的随访期间死亡。单变量分析显示，所有研究的影像学标志物均显著预测预后。多变量分析显示，TAPSE（风险比：1.46；95%置信区间：1.16 至 1.85；p<0.01）和 CMR 索引的每搏量（风险比：1.24；95%置信区间：1.04 至 1.48；p<0.05）是死亡率的唯一独立预测因子。
特定的功能和结构异常特征是不同心脏淀粉样沉积负荷的特征。在对大量淀粉样变患者进行的多模态成像评估中，CMR 衍生的 TAPSE 和指数化的每搏量是最强的心脏功能预后标志物。
