University of Chicago, Department of Medicine, Section of Cardiology, Chicago, IL, USA.
University of Chicago, Department of Medicine, Section of Cardiology, Chicago, IL, USA.
Curr Probl Cardiol. 2024 Sep;49(9):102729. doi: 10.1016/j.cpcardiol.2024.102729. Epub 2024 Jun 29.
Current echocardiographic risk factors for prognosis in cardiac amyloidosis (CA) do not distinguish between the two main subtypes: transthyretin cardiomyopathy (TTR) and immunoglobulin light chain cardiomyopathy (AL), each of which require distinct diagnostic and therapeutic approaches. Additionally, only traditional parameters have been studied with little data on advanced techniques. Accordingly, we sought to determine whether differences exist in 2D transthoracic echocardiography (2DE) predictors of survival between the CA subtypes using a comprehensive approach.
220 patients (72±12 years) with confirmed CA (AL=89, TTR=131) who underwent 2DE at the time of CA diagnosis were enrolled. Left ventricular (LV) dimensions, indexed mass (LVMi), global longitudinal strain (LVGLS), apical-sparing ratio (LVASR), diastology, right ventricular (RV) size and function indices including tricuspid annular systolic excursion (TAPSE), RV free-wall (RVFWS) and global (RVGLS) strain, indexed left (LA) and right atrial volumes (LAVi and RAVi), LA strain (reservoir and booster) and RV systolic pressure (RVSP) were measured. A propensity-score weighted stepwise variable selection Cox proportional hazards model derived from NYHA class and renal impairment status at diagnosis was used to determine the associations between 2DE parameters and mortality specific to CA subtype over a median follow-up of 36-months.
After adjusting for age, atrial fibrillation and treatment, parameters associated with survival were RVFWS (p=0.003, HR 1.15, 95% CI[1.053,1.245]) and RVSP (p=0.03, HR 1.03, 95% CI[1.004,1.063]) in AL and LVASR (p=0.007, HR 6.68, 95% CI[1.75,25.492]) and RAVi (p=0.049, HR 1.03, 95% CI[1.000,1.052]) in TTR.
Echocardiographic prognosticators for survival are specific to cardiac amyloid subtype. These results potentially provide information critical for clinical decision-making and follow-up in these patients.
目前，用于预测心脏淀粉样变性（CA）预后的超声心动图危险因素不能区分两种主要亚型：转甲状腺素蛋白心肌病（TTR）和免疫球蛋白轻链心肌病（AL），这两种亚型需要不同的诊断和治疗方法。此外，仅研究了传统参数，而关于先进技术的数据很少。因此，我们试图确定使用综合方法在 CA 两种亚型之间 2 维经胸超声心动图（2DE）预测生存的差异是否存在。
纳入 220 名确诊 CA（AL=89，TTR=131）的患者，年龄（72±12）岁，在 CA 诊断时进行 2DE。测量左心室（LV）尺寸、指数化质量（LVMi）、整体纵向应变（LVGLS）、心尖保留比值（LVASR）、心脏舒张功能、右心室（RV）大小和功能指数，包括三尖瓣环收缩期位移（TAPSE）、RV 游离壁（RVFWS）和整体（RVGLS）应变、指数化左心房（LA）和右心房容积（LAVi 和 RAVi）、LA 应变（储备和增强）和 RV 收缩压（RVSP）。使用基于纽约心脏协会（NYHA）分级和诊断时肾功能损害状态的倾向评分加权逐步变量选择 Cox 比例风险模型，确定在中位随访 36 个月期间 2DE 参数与 CA 特定亚型死亡率之间的关联。
在调整年龄、心房颤动和治疗因素后，与生存相关的参数为 AL 中的 RVFWS（p=0.003，HR 1.15，95%CI[1.053,1.245]）和 RVSP（p=0.03，HR 1.03，95%CI[1.004,1.063]）以及 TTR 中的 LVASR（p=0.007，HR 6.68，95%CI[1.75,25.492]）和 RAVi（p=0.049，HR 1.03，95%CI[1.000,1.052]）。
用于预测生存的超声心动图预后因素与心脏淀粉样变亚型有关。这些结果可能为这些患者的临床决策和随访提供关键信息。
