Hammer Peter E, McEnaney Kerry, Callahan Ryan, Baird Christopher W, Hoganson David M, Jenkins Kathy J
Department of Cardiac Surgery, Boston Children's Hospital, Boston, MA 02115, USA.
Department of Cardiology, Boston Children's Hospital, Boston, MA 02115, USA.
Children (Basel). 2021 Aug 25;8(9):729. doi: 10.3390/children8090729.
Pulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease progression include surgery and catheter-based balloon dilation and stent implantation. Its cause and mechanism of progression are unknown. In this pilot study, we propose and explore the hypothesis that elevated wall shear stress at discrete pulmonary venous sites triggers stenosis. To assess this theory, we retrospectively analyzed cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. Results are consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. The analysis also suggests the possibility of predicting the target lumen size necessary in a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression.
肺静脉狭窄是一种严重病症，其特征是由于婴儿或儿童肺静脉中出现的纤维化组织向内生长导致的狭窄或堵塞。它通常呈进行性发展，可导致严重的肺动脉高压和死亡。阻止或逆转疾病进展的措施包括手术、基于导管的球囊扩张和支架植入。其病因和进展机制尚不清楚。在这项初步研究中，我们提出并探讨了一个假设，即离散肺静脉部位的壁面剪切应力升高会引发狭窄。为了评估这一理论，我们回顾性分析了两名患者疾病进展过程中多个时间点的心脏导管检查、肺部扫描和X射线计算机断层扫描数据，以估计肺静脉中的壁面剪切应力。结果与壁面剪切应力存在一个升高水平相符，高于该水平疾病呈进行性发展，低于该水平进展则停止。分析还表明，有可能预测在给定静脉中使壁面剪切应力降至正常水平并消除狭窄进展触发因素所需的目标管腔大小。
