背景:目前心脏淀粉样变性(CA)预后的超声心动图危险因素无法区分两种主要亚型:甲状腺素运载蛋白心肌病(TTR)和免疫球蛋白轻链心肌病(AL),每个都需要不同的诊断和治疗方法。此外,只研究了传统的参数,很少有关于先进技术的数据。因此,我们试图通过综合方法确定CA亚型之间的2D经胸超声心动图(2DE)生存预测因子是否存在差异.
方法:纳入220例(72±12年)确诊为CA的患者(AL=89,TTR=131),在诊断为CA时行2DE。左心室(LV)尺寸,指数质量(LVMi),全局纵向应变(LVGLS),根尖备用比(LVASR),非分解学,右心室(RV)大小和功能指标,包括三尖瓣环收缩偏移(TAPSE),RV自由壁(RVFWS)和全局(RVGLS)应变,左(LA)和右心房容积(LAVi和RAVi),测量LA应变(储层和助推器)和RV收缩压(RVSP)。在36个月的中位随访时间内,使用从NYHA分级和诊断时肾功能损害状态得出的倾向评分加权逐步变量选择Cox比例风险模型来确定2DE参数与CA亚型特有死亡率之间的关联。
结果:调整年龄后,心房颤动和治疗,与生存相关的参数是在AL和LVASR(p=0.007,HR6.68,95%CI[1.75,25.492])和RAVi(p=0.049,HR1.03,95%CI[1.053,1.245])中的RVSP(p=0.03,HR1.03,95%CI[1.0063])和在1.00052中的RAVi(p=HR1.03,TCI)。
结论:存活的超声心动图预测指标对心脏淀粉样蛋白亚型具有特异性。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。
BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.