Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic.
Children's Heart Centre, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic.
Cardiovasc Pathol. 2019 May-Jun;40:59-64. doi: 10.1016/j.carpath.2019.02.004. Epub 2019 Feb 28.
The purpose of this study was to evaluate the microscopic structural abnormalities of the ascending aorta in infants with Tetralogy of Fallot (ToF) and compare them with aortic samples from control group of small children that died of other diseases. We aimed at identification of the specific histopathological changes associated with ToF and correlation of the severity of these changes with time to surgery and mean levels of saturation in the ToF group, and age at death in control group.
The full-thickness ascending aortic wall sample was taken from 23 children with ToF at the time of surgical reconstruction (age spread 2 to 19 months) and evaluated by light microscopy. Corresponding samples were taken from 16 cadaverous cases of children with other diseases (0-76 months). The assessed morphological variables included elastic fiber fragmentation/loss, thinning and disorganisation, presence of laminar medial necrosis, intralamellar and translamellar mucoid extracellular matrix accumulations, smooth muscle cell disorganisation, presence of fibrosis, calcifications and neovascularisation and finally grade of overall medial degeneration.
No difference was found between the two groups in the individual morphological variables. However, there was a difference in the distribution of the grades of the overall medial degeneration (P = .016). ToF group showed uniform mild degenerative changes, whereas control group harboured spectrum of changes ranging from normal to moderate. The presence of the given histopathological changes and their severity were associated neither with age at surgery or mean levels of saturation in ToF group, nor with the age at death in the control group.
This study emphasizes the histopathological assessment of the bioptic samples of the ascending aorta during the surgical repair of ToF, since the patients demonstrating moderate or severe degenerative changes already in the early childhood may be in increased risk of the subsequent late complications.
本研究旨在评估法洛四联症（ToF）患儿升主动脉的微观结构异常，并将其与因其他疾病死亡的小龄儿童的主动脉样本进行比较。我们旨在确定与 ToF 相关的特定组织病理学变化，并将这些变化的严重程度与 ToF 组的手术时间和饱和度平均值以及对照组的死亡年龄相关联。
在手术重建时（年龄 2 至 19 个月），从 23 例 ToF 患儿中采集全层升主动脉壁样本，并通过光镜进行评估。从 16 例因其他疾病死亡的儿童尸体中采集相应的样本（0-76 个月）。评估的形态学变量包括弹性纤维碎裂/缺失、变薄和紊乱、层状中膜坏死、层间和跨层粘多糖细胞外基质积聚、平滑肌细胞紊乱、纤维化、钙化和新生血管形成以及中膜整体退行性变的分级。
两组之间在个别形态学变量方面没有差异。然而，在整体中膜退行性变分级的分布上存在差异（P =.016）。ToF 组表现为均匀的轻度退行性改变，而对照组则表现为从正常到中度的变化范围。组织病理学变化的存在及其严重程度既与 ToF 组的手术年龄或饱和度平均值无关，也与对照组的死亡年龄无关。
本研究强调了在 ToF 手术修复期间对升主动脉活检样本进行组织病理学评估，因为在幼儿期已经表现出中度或重度退行性改变的患者可能面临随后发生晚期并发症的风险增加。
