Sellers Zachary M, McGlocklin Lori, Brasch Andrea
College of Medicine, University of Illinois, Urbana-Champaign, Urbana, IL, USA.
Heart and Cardiovascular Center, Carle Foundation Hospital, Urbana, IL, USA.
J Cyst Fibros. 2015 Sep;14(5):654-60. doi: 10.1016/j.jcf.2015.03.010. Epub 2015 Apr 9.
CFTR is expressed in cardiac myocytes. In mice, lack of CFTR alters cardiomyocyte contraction and Ca2+ signaling, and decreases cardiac reserve. We undertook a pilot study evaluating left ventricular (LV) function in CF patients using strain and strain rate echocardiography.
Echocardiography with tissue Doppler and strain and strain rate imaging were performed in 8 CF adults following pulmonary function tests. Results were compared to literature values obtained in healthy subjects.
All CF individuals had normal LV ejection fractions. In contrast, 50% of men and 100% of women with CF had decreased LV systolic strain. Strain rates were significantly decreased in 100% of CF individuals. RV function was normal and LV function did not correlate with lung function.
Strain and strain rate echocardiography identified LV systolic abnormalities in CF individuals not detected by conventional echocardiography. We propose that this echocardiography modality may identify subclinical cardiac dysfunction in CF.
囊性纤维化跨膜传导调节因子（CFTR）在心肌细胞中表达。在小鼠中，CFTR的缺失会改变心肌细胞收缩和Ca2+信号传导，并降低心脏储备。我们进行了一项初步研究，使用应变和应变率超声心动图评估囊性纤维化（CF）患者的左心室（LV）功能。
在8名成年CF患者进行肺功能测试后，采用组织多普勒、应变和应变率成像进行超声心动图检查。将结果与健康受试者的文献值进行比较。
所有CF个体的左心室射血分数均正常。相比之下，50%的CF男性和100%的CF女性左心室收缩应变降低。100%的CF个体的应变率显著降低。右心室功能正常，左心室功能与肺功能无关。
应变和应变率超声心动图可识别常规超声心动图未检测到的CF个体的左心室收缩异常。我们认为这种超声心动图检查方式可能识别出CF患者的亚临床心脏功能障碍。
