Lewis Matthew J, Hecht Elizabeth, Ginns Jonathan, Benton Joshua, Prince Martin, Rosenbaum Marlon S
Division of Cardiology, Department of Medicine, Schneeweiss Adult Congenital Heart Center, Columbia University Medical Center, New York, New York, USA.
Division of Abdominal Imaging, Department of Radiology, Columbia University Medical Center, New York, New York, USA.
Congenit Heart Dis. 2017 Mar;12(2):153-158. doi: 10.1111/chd.12422. Epub 2016 Nov 28.
The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.
A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004).
Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.
成人Fontan患者肝病的进展情况尚未完全明确。他们回顾了成人Fontan患者系列心脏磁共振成像（CMR）的经验，以确定是否存在长期Fontan循环暴露的肝脏解剖学标志物，以及是否能够识别进行性肝充血的临床预测因素。
对所有接受过至少两次CMR检查的成人Fontan患者进行了一项回顾性队列研究。根据临床指导CMR检查时获取的图像，确定肝脏尺寸、下腔静脉（IVC）大小、右肝静脉（RHV）大小和脾脏直径。两名具有肝脏成像专业知识的放射科医生使用钆剂增强后的序列独立对充血情况和肝脏大小进行分级。27例患者符合纳入标准。在两次CMR检查之间平均5.1年的时间里，肝脏左右径平均值（P = 0.005）、RHV直径平均值（P = 0.004）和脾脏直径平均值（P = 0.001）均有显著增加。27例患者中有25例（93%）可获得系列钆剂增强后的图像，其中15例（55%）在系列研究中显示出进行性肝充血的证据。进行性肝充血与单心室射血分数（SVEF）低于50%（P = 0.008）以及较大的体表面积校正舒张末期容积（EDVI）和收缩末期容积（ESVI）相关。RHV直径是唯一与Fontan手术完成后的时间显著相关的解剖学变量（P = 0.004）。
系列CMR检查在我们的成人Fontan患者队列中，平均5.2年的时间里检测到肝脏和肝静脉的进行性增大。大量成人Fontan患者发生进行性肝充血，可能与CMR显示的心室增大和心室功能降低有关。
