From the Department of Pediatrics (F.Z.), Heart Institute (A.M.L., D.A.K., A.R.O., A.L., C.C., A.W.P., C.R.V., T.A.), Department of Radiology (A.T.T., J.R.D.), Division of Gastroenterology, Hepatology, and Nutrition (J.J.P.), and Division of Cardiothoracic Surgery (D.L.M.), Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229; Department of Radiology (A.T.T., J.R.D.) and Division of Gastroenterology (N.A.), University of Cincinnati Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio (A.T.T.); and Heart Institute, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pa (T.A.).
Radiology. 2022 Jun;303(3):557-565. doi: 10.1148/radiol.211037. Epub 2022 Mar 15.
Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9-55 years; 32 children) were evaluated (median age, 23 years; IQR, 17-30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12-23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); median follow-up duration was 0.4 year (IQR, 0.1-3.1 years). Compared with patients who had Fontan circulation without deterioration, patients with Fontan deterioration were more likely to have moderate or severe ventricular systolic dysfunction ( < .01), moderate or severe atrioventricular valve regurgitation ( < .01), higher Fontan pressure ( = .01), radiologic evidence of portal hypertension ( < .01), and VAST score of 2 or greater ( < .01). Conclusion Radiologic evidence of portal hypertension at abdominal imaging in children and adults with Fontan circulation was associated with higher venous pressures and an increased risk for Fontan deterioration. These characteristics may be used to identify patients who warrant comprehensive hemodynamic evaluation. © RSNA, 2022.
背景 在 Fontan 循环中，门静脉高压是系统性静脉压升高和肝纤维化的结果。目的 定量评估 Fontan 循环中儿童和成人的放射学门静脉高压证据的流行率（静脉曲张、腹水、脾肿大和血小板减少各 1 分，VAST 评分≥2 分），并确定其与血液动力学和不良结局的关系。材料与方法 这是一项回顾性研究，纳入了 2012 年 1 月至 2019 年 12 月期间因局灶性肝脏病变监测而行腹部 MRI 或 CT 检查的 Fontan 循环患者。门静脉高压定义为存在以下至少两种情况：静脉曲张、腹水或脾肿大。Fontan 恶化定义为心力衰竭的综合体征或症状，需要利尿剂升级、心室辅助装置植入、心脏移植或死亡。使用单变量和多变量逻辑回归评估变量与复合终点之间的关系。结果 共评估了 123 例患者（年龄 9-55 岁；32 例为儿童）（中位年龄 23 岁；IQR，17-30 岁；63 例为男性）。自诊断为 Fontan 循环以来的中位时间为 16 年（IQR，12-23 年）。123 例患者中有 25 例（20%）存在放射学门静脉高压证据，34 例（28%）VAST 评分≥2 分。123 例患者中有 25 例（20%）发生了 Fontan 恶化；中位随访时间为 0.4 年（IQR，0.1-3.1 年）。与 Fontan 循环无恶化的患者相比，Fontan 恶化的患者更可能出现中度或重度心室收缩功能障碍（<.01）、中度或重度房室瓣反流（<.01）、较高的 Fontan 压力（=.01）、放射学门静脉高压证据（<.01）和 VAST 评分≥2 分（<.01）。结论 在儿童和成人的 Fontan 循环腹部影像学中发现门静脉高压的放射学证据与较高的静脉压和更高的 Fontan 恶化风险相关。这些特征可用于识别需要全面血液动力学评估的患者。
