Department of Paediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands.
Department of Paediatric Pulmonology, Wilhelmina Children's Hospital, University Medical Center Utrecht, The Netherlands.
J Cyst Fibros. 2018 Sep;17(5):643-649. doi: 10.1016/j.jcf.2017.12.009. Epub 2018 Feb 3.
As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children.
33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study. Measurements of lung function, arterial stiffness, and echocardiography (conventional measures and myocardial deformation imaging) were performed.
There were no differences in anthropometrics, lung function and blood pressure between the two groups. The cystic fibrosis children had a higher arterial stiffness compared to the healthy children (pulse wave velocity respectively 5.76±0.57m/s versus 5.43±0.61m/s, p-value 0.049). Using conventional echocardiographic parameters for right ventricular function, Tricuspid Annular Plane Systolic Excursion) and Tissue Doppler Imaging, cystic fibrosis children had a reduced right ventricular systolic function when compared to the healthy children. After adjustment for lung function, global strains of both right and left ventricles were significantly lower in the cystic fibrosis group than in healthy children (linear regression coefficient 1.45% left ventricle, p-value 0.022 and 4.42% right ventricle, p-value <0.01). Systolic strain rate of basal segment of the left ventricle, the mid segment of the right ventricle and the apical septum were significantly lower in the cystic fibrosis children than in healthy controls.
Our study suggests that already at a very young age, children with cystic fibrosis show an increased arterial stiffness and some signs of diminished both right and left ventricular function.
随着囊性纤维化患者的预期寿命延长，关注肺部以外的合并症变得尤为重要。一些研究表明，成年患者存在心肌功能障碍的迹象，但对于这些变化随时间推移的发生和发展知之甚少。在这项前瞻性研究中，我们将囊性纤维化患儿的心脏功能与健康儿童进行了比较。
从威廉敏娜儿童医院招募了 33 名年龄在 3-12 岁的囊性纤维化患儿，并从基于人群的 WHISTLER 研究中选择了 33 名年龄匹配的健康儿童。对肺功能、动脉僵硬度和超声心动图（常规测量和心肌应变成像）进行了测量。
两组儿童在人体测量学、肺功能和血压方面无差异。与健康儿童相比，囊性纤维化患儿的动脉僵硬度更高（脉搏波速度分别为 5.76±0.57m/s 和 5.43±0.61m/s，p 值为 0.049）。使用常规超声心动图参数评估右心室功能（三尖瓣环平面收缩期位移）和组织多普勒成像，囊性纤维化患儿的右心室收缩功能较健康儿童降低。在调整肺功能后，与健康儿童相比，囊性纤维化组左右心室的整体应变均显著降低（线性回归系数左心室 1.45%，p 值为 0.022，右心室 4.42%，p 值<0.01）。左心室基底段、右心室中段和心尖隔段的收缩应变率也显著低于健康对照组。
我们的研究表明，即使在非常年幼的时期，囊性纤维化患儿就已经表现出动脉僵硬度增加和左右心室功能下降的迹象。
