Clinical Cardiovascular Research Laboratory for the Elderly, Columbia University Medical Center, Allen Hospital of New York Presbyterian Hospital , New York, NY , USA.
Amyloid. 2015 Mar;22(1):61-6. doi: 10.3109/13506129.2014.994202. Epub 2014 Dec 16.
Cardiac amyloidosis is a cause of diastolic heart failure in which ejection fraction (EF) remains "normal" despite progression of disease. The myocardial contraction fraction (MCF) is an index of myocardial function, defined as stroke volume (SV) over myocardial volume (MV). We hypothesized that MCF would be superior to EF, the conventional measure of left ventricular function, in predicting survival among patients with cardiac amyloidosis. Sixty-six subjects (mean age = 67 ± 12 years; 20% women) with cardiac amyloidosis (34 with light-chain amyloid and 32 with transthyretin amyloid) underwent two-dimensional echocardiography to determine left ventricular structure and function. Cox proportional hazard modeling was used to determine the association of MCF and EF with survival. Over a mean follow-up of 1.86 ± 1.78 years (range 0.03-7.36 years), 37 subjects (56.1%) died. Mean EF of the study population was 51 ± 13%. There was no significant difference in EF between patients who survived the study period and those who died (54 ± 11% versus 49 ± 14%; p = 0.1196) while there was a significant difference in MCF (35 ± 19% versus 23 ± 10%, p = 0.0065). Using Cox proportional hazards modeling, MCF was associated with death (HR = 0.953, 95% CI of 0.932-0.984, p = 0.0031) while EF was not (HR = 0.991, 95% CI of 0.968-1.014, p = 0.4320). In a multivariate model, amyloid light-chain (AL) amyloid type was an independent risk predictor of death with a HR of 2.841 (95% CI of 1.214-6.648, p = 0.0161) along with a MCF < 30 with a HR of 2.567 (95% CI of 1.197-5.508, p = 0.0155), which was driven by a higher risk in AL subjects with a MCF < 30, HR of 3.39 (95% CI of 1.20-9.55, p = 0.021) than TTR subjects with a MCF < 30, HR of 1.26 (95% CI of 0.36-3.28, p = 0.87). In conclusion, MCF, a novel measure of myocardial chamber function, is superior to EF in predicting overall survival among patients with AL cardiac amyloidosis.
心脏淀粉样变是舒张性心力衰竭的一个病因,尽管疾病进展,射血分数 (EF) 仍“正常”。心肌收缩分数 (MCF) 是心肌功能的指标,定义为心搏量 (SV) 除以心肌容积 (MV)。我们假设 MCF 优于 EF,即左心室功能的常规测量,可预测心脏淀粉样变患者的生存。66 名(平均年龄 67±12 岁;20%为女性)心脏淀粉样变患者(34 名轻链淀粉样变,32 名转甲状腺素蛋白淀粉样变)接受二维超声心动图检查以确定左心室结构和功能。使用 Cox 比例风险模型确定 MCF 和 EF 与生存的相关性。在平均 1.86±1.78 年(范围 0.03-7.36 年)的随访中,37 名(56.1%)患者死亡。研究人群的平均 EF 为 51±13%。存活研究期间的患者与死亡患者之间 EF 无显著差异(54±11%与 49±14%;p=0.1196),而 MCF 有显著差异(35±19%与 23±10%;p=0.0065)。使用 Cox 比例风险模型,MCF 与死亡相关(HR=0.953,95%CI 为 0.932-0.984,p=0.0031),而 EF 不相关(HR=0.991,95%CI 为 0.968-1.014,p=0.4320)。在多变量模型中,AL 淀粉样变类型是死亡的独立风险预测因子,HR 为 2.841(95%CI 为 1.214-6.648,p=0.0161),MCF<30 的 HR 为 2.567(95%CI 为 1.197-5.508,p=0.0155),这是由 AL 患者 MCF<30 的风险更高驱动的,HR 为 3.39(95%CI 为 1.20-9.55,p=0.021),而 TTR 患者 MCF<30 的 HR 为 1.26(95%CI 为 0.36-3.28,p=0.87)。总之,MCF 是一种新的心肌腔室功能指标,在预测 AL 心脏淀粉样变患者的总体生存方面优于 EF。