Department of Pediatric Cardiology, University Children's Hospital, University of Tübingen, Tübingen, Germany.
Department of Pediatric Cardiology, University Hospital Tübingen, Hoppe-Seyler-Str. 1, 72076, Tübingen, Germany.
Sci Rep. 2024 Nov 29;14(1):29752. doi: 10.1038/s41598-024-81299-w.
Recent research in patients with functionally univentricular hearts (UVH) is focusing on pathologies of the lymphatic vessels. Morphology of the abdominal lymphatic vessels was analyzed by MRI in patients with UVH following total cavopulmonary connection (TCPC) and it was examined, if clinical and laboratory parameters correlate with changes after TCPC. We prospectively examined 33 patients at the age of 19.8 (14.6;30.2) years [median (Q1;Q3)] after TCPC (follow-up 14.3 years (9.7;24.9) with a heavily T2-weighted MRI sequence on a 3.0 T scanner. Examinations in coronal orientation were performed with respiratory gating, slice thickness 0.6 mm, TR 2400 ms, TE 692 ms, FoV 460 mm (covering thoracic and abdominal regions), scan time 14:41 min (13:18;16:30) after a solid meal and a cup of pineapple juice. The findings were classified according to delineation of abdominal lymphatic vessels. Type 1: <3 abdominal vessels (av) definable; type 2: 4-6 av definable; type 3: >6 av and/or oedematous changes or ascites. The results were correlated with parameters obtained at the annual routine check-up. Statistical analysis was performed using U-test and Chi-square test. Fifteen patients (group 1) showed type 3 lymphatic morphologies, two of which had ascites. Eighteen patients (group 2) showed lower grade morphologies (type 1-2). Image quality was rated considering the delineation of the common hepatic duct and did not differ between groups (p = 0.134). "Lymphatic burden" was automatically examined and was indexed to the number of delineated abdominal vessels and showed quantification according to the chosen categories type 1-3. Patients in group 1 were younger at MRI examination (17.4;14.3/18.9 vs. 26.2;18.2/32.3 years, p = 0.03). Superior cavopulmonary connection (SCPC) had been performed earlier in group 1 (9.9;7.9/25.5 vs. 29.2;13.7/66.6 months, p = 0.018). Laboratory examinations in group 1 showed lower levels for Immunoglobulin G (IgG), Lipase, α-Antitrypsin, Cystatin C and TSH. There were no significant differences for total protein, NTproBNP, lymphocytes or platelets. A history of chylothorax was present in 7/15 versus 2/18 p = 0.022. Protein-losing enteropathy (PLE) occurred in 4/15 versus 1/18 (p = 0.092). T2 weighted MRI is feasible for noninvasive delineation of abdominal lymphatic vessel in patients following TCPC. In the long-term follow-up, patients with more pronounced changes of the abdominal lymphatic vessels were younger at SCPC and were more likely to show a history of chylothorax and lower IgG values.
最近，对功能性单心室心脏（UVH）患者的研究重点是淋巴管的病理学。通过 MRI 分析了 TCPC 后 UVH 患者的腹部淋巴管形态，并检查了临床和实验室参数是否与 TCPC 后变化相关。我们前瞻性地检查了 33 名年龄为 19.8（14.6；30.2）岁[中位数（Q1；Q3）]的患者在 TCPC 后 14.3 年（9.7；24.9）[中位数（Q1；Q3）]，使用 3.0 T 扫描仪上的重 T2 加权 MRI 序列进行检查。在冠状方向上进行检查，采用呼吸门控，层厚 0.6 毫米，TR 2400 毫秒，TE 692 毫秒，FOV 460 毫米（覆盖胸部和腹部区域），扫描时间为固体餐后 14:41 分钟（13:18；16:30）和一杯菠萝汁。根据腹部淋巴管的描绘将发现分类。1 型：可定义<3 个腹部血管（av）；2 型：可定义 4-6 个 av；3 型：>6 个 av 和/或水肿改变或腹水。将结果与年度常规检查中获得的参数相关联。使用 U 检验和卡方检验进行统计分析。15 名患者（组 1）表现出 3 型淋巴管形态，其中 2 名患者有腹水。18 名患者（组 2）表现出较低等级的形态（1-2 型）。考虑到共同肝管的描绘，图像质量被评为不分组（p=0.134）。“淋巴负荷”自动检查，并根据所选类别 1-3 进行指数化至描绘的腹部血管数量。组 1 的 MRI 检查年龄较小（17.4；14.3/18.9 岁 vs. 26.2；18.2/32.3 岁，p=0.03）。组 1 更早地进行了 superior cavopulmonary connection（SCPC）（9.9；7.9/25.5 岁 vs. 29.2；13.7/66.6 个月，p=0.018）。组 1 的实验室检查显示 IgG、脂肪酶、α-抗胰蛋白酶、胱抑素 C 和 TSH 水平较低。总蛋白、NTproBNP、淋巴细胞或血小板无显著差异。7/15 例有乳糜胸病史，18 例中有 2/18 例（p=0.022）。15 例中有 4 例发生蛋白丢失性肠病（PLE），18 例中有 1 例（p=0.092）。T2 加权 MRI 是 TCPC 后患者非侵入性描绘腹部淋巴管的可行方法。在长期随访中，腹部淋巴管变化更明显的患者在 SCPC 时年龄更小，更有可能有乳糜胸病史和更低的 IgG 值。
