From the Department of Radiology (D.M.B., D.S.) and Division of Cardiology (A.G.D., E.M.P., R.E.M., J.A.J., H.G., M.L.O., M.A.F., M.A.H., S.L.P., K.K.W., D.J.G., J.R., A.C.G., M.J.G., J.J.R., Y.D.), Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104; and University of Pennsylvania School of Medicine, Philadelphia, PA (D.M.B., A.G.D., H.G., M.L.O., M.A.F., M.A.H., S.L.P., K.K.W., D.S., D.J.G., J.R., A.C.G., M.J.G., J.J.R., Y.D.).
Radiology. 2019 Jun;291(3):774-780. doi: 10.1148/radiol.2019180877. Epub 2019 Apr 2.
Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. Purpose To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. Materials and Methods Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. Results A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; < .01). Conclusion Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019
背景 法乐四联症手术是治疗单心室生理的姑息性手术。该手术通常先进行上腔静脉-肺动脉连接（SCPC）；SCPC 后 MRI 可显示淋巴异常，且可在法乐四联症手术前发现。目的 确定在接受上腔静脉-肺动脉连接（SCPC）姑息性治疗的单心室疾病患者中，T2 加权 MRI 上的颈部和胸部淋巴异常程度与 Fontan 手术的手术结果是否相关。材料与方法 对 2012 年 7 月至 2015 年 5 月在一家医疗机构接受 SCPC 姑息性治疗的单心室疾病患者进行了回顾性研究。T2 加权图像评分分为淋巴 1 型（纵隔和锁骨上区域信号很少或没有 T2 信号）至 4 型（T2 信号进入纵隔和肺实质）。记录 Fontan 术失败、Fontan 术后住院时间和胸腔积液、术后塑型性支气管炎、需要移植和死亡率。采用方差分析（ANOVA）、Kruskal-Wallis H 检验和 Fisher 确切检验评估淋巴类型与临床结局的关系。结果 共评估了 83 例患者（平均年龄，7.9 岁±2.6 岁）。在这 83 例患者中，53 例（64%）存在 1 型或 2 型淋巴异常，17 例（20%）存在 3 型，12 例（16%）存在 4 型。4 型患者 Fontan 术失败率高于 1 型或 2 型（分别为 54%和 2%； =.004）。心脏移植需求（13 例中的 1 例[8%]）和死亡（13 例中的 3 例[23%]）仅发生在 4 型患者中。4 型患者术后住院时间中位数长于 1 型或 2 型（分别为 29 天和 9 天； <.01）。结论 在计划进行 Fontan 手术前，患者的 MRI 显示淋巴异常严重程度越高，Fontan 术失败和术后住院时间延长的可能性越大。
