Senapati Alpana, Sperry Brett W, Grodin Justin L, Kusunose Kenya, Thavendiranathan Paaladinesh, Jaber Wael, Collier Patrick, Hanna Mazen, Popovic Zoran B, Phelan Dermot
Houston Methodist DeBakey Heart and Vascular Center, Houston, Texas, USA.
Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Heart. 2016 May 15;102(10):748-54. doi: 10.1136/heartjnl-2015-308657. Epub 2016 Feb 1.
Cardiac amyloidosis (CA) is a rapidly progressive disease that portends poor prognosis. Our objective was to evaluate the prognostic impact of relative regional strain ratio (RRSR, a measure of the relative apical sparing of longitudinal strain (LS)) in CA.
This is a retrospective study evaluating 97 patients with CA from 2004 to 2013. Patients were included if they met criteria for CA based on endomyocardial biopsy or advanced imaging criteria coupled with either extracardiac biopsy or genetic analysis. Baseline clinical and imaging data were collected and compared between light-chain amyloidosis (AL) (n=59) and transthyretin amyloidosis (ATTR) (n=38) subtypes. RRSR was defined as the average apical LS divided by the sum of the average mid and basal LS values. A Cox proportional hazards model was used to assess the effects of clinical and echocardiographic characteristics, including RRSR, on the outcome of time to death or heart transplantation.
Despite younger age, the AL subtype had a statistically significant association with the composite outcome as compared with ATTR (p=0.022). Log-transformed RRSR was independently associated with the composite outcome at 5 years (HR 2.45 (1.36 to 4.40), p=0.003). Patients with low ejection fraction and high RRSR had the worst prognosis. In multivariable analysis, RRSR remained predictive of the primary outcome (p=0.018). Addition of covariates related to systolic function (global LS and ejection fraction) to the model attenuated this effect.
High RRSR is adversely prognostic in patients with cardiac amyloid. This novel tool is both diagnostic and prognostic and may have implications in management and suitability for treatment.
心脏淀粉样变性（CA）是一种进展迅速且预后不良的疾病。我们的目的是评估相对区域应变率（RRSR，一种纵向应变（LS）相对心尖保留情况的测量指标）在CA中的预后影响。
这是一项回顾性研究，评估了2004年至2013年期间的97例CA患者。如果患者根据心内膜心肌活检或先进的影像学标准，结合心外活检或基因分析符合CA标准，则纳入研究。收集基线临床和影像学数据，并在轻链淀粉样变性（AL）（n = 59）和转甲状腺素蛋白淀粉样变性（ATTR）（n = 38）亚型之间进行比较。RRSR定义为平均心尖LS除以平均中间段和基底段LS值之和。使用Cox比例风险模型评估临床和超声心动图特征（包括RRSR）对死亡或心脏移植时间结局的影响。
尽管年龄较轻，但与ATTR相比，AL亚型与复合结局有统计学显著关联（p = 0.022）。对数转换后的RRSR在5年时与复合结局独立相关（HR 2.45（1.36至4.40），p = 0.003）。射血分数低且RRSR高的患者预后最差。在多变量分析中，RRSR仍然是主要结局的预测指标（p = 0.018）。将与收缩功能相关的协变量（整体LS和射血分数）添加到模型中减弱了这种效应。
高RRSR对心脏淀粉样变性患者的预后不利。这种新工具兼具诊断和预后价值，可能对管理和治疗适用性有影响。
