Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel; DZHK (German Center for Cardiovascular Research), Partner Site Hamburg/Kiel/Lübeck, Germany.
Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel.
Semin Thorac Cardiovasc Surg. 2022 Summer;34(2):642-650. doi: 10.1053/j.semtcvs.2021.04.005. Epub 2021 May 9.
Fontan associated liver disease (FALD) has been recognized as a potentially serious sequela of the Fontan circulation. Prevalence of FALD among different age groups and risk factors for advanced changes were assessed. FALD screening included abdominal ultrasound and laboratory tests. A "liver disease score (LDS)" incorporating items from ultrasound and blood testing was calculated to grade FALD severity (5 items each, maximum score 10 points). 240 patients (male: n = 139, female: n = 101, systemic right ventricle: n = 160) underwent FALD screening 10 (IQR 7-15) years after Fontan surgery. Ultrasound was abnormal in 184 (76.6%) patients (surface nodularity / blunted liver edge: n = 133, 55.4%; heterogeneous parenchyma: n = 93, 38.8%; splenomegaly: n = 68, 28.3%; ascites: n = 23, 9.6%). At least one abnormal laboratory test was detected in 218 (90.8%) patients. Gamma-glutamyl-transpeptidase was elevated in the majority of patients (n = 206, 85.8%). Median LDS was 3 (2-4). Scores ≥5 were observed in 32 (13.3%) patients. Longer follow-up (15 (11-20) vs 9 (6-14) years, P <0.001), higher central venous (13 (11-15) vs 10 (9-12) mmHg, P <0.001) and end-diastolic pressure (8 (5-10) vs 6 (5-7) mmHg, P = 0.001), impaired ventricular function and absence of sinus rhythm were associated with LDS ≥5. Longer follow-up (OR 1.2 (1.1-1.3), P <0.001) and higher central venous pressure (OR 1.6 (1.3-2.1), p < 0.001) were the only independent predictors of advanced FALD. Abdominal ultrasound and laboratory abnormalities suggestive of FALD are common during routine follow-up already in childhood and adolescence irrespective of ventricular morphology. More advanced findings are associated with longer follow-up and higher central venous pressure.
Fontan 相关肝疾病（FALD）已被认为是 Fontan 循环的一种潜在严重后遗症。本研究评估了不同年龄组 FALD 的患病率和进展性改变的危险因素。FALD 筛查包括腹部超声和实验室检查。计算了包含超声和血液检测项目的“肝疾病评分（LDS）”，以分级 FALD 严重程度（每项 5 分，最高 10 分）。240 名患者（男性：n=139，女性：n=101，右心系统：n=160）在 Fontan 手术后 10（IQR 7-15）年接受 FALD 筛查。184 名患者（76.6%）的超声检查异常（表面结节/肝脏边缘变钝：n=133，55.4%；不均匀实质：n=93，38.8%；脾肿大：n=68，28.3%；腹水：n=23，9.6%）。218 名患者（90.8%）至少有一项异常实验室检查。大多数患者的γ-谷氨酰转肽酶升高（n=206，85.8%）。中位 LDS 为 3（2-4）。32 名患者（13.3%）的评分≥5。较长的随访时间（15（11-20）比 9（6-14）年，P<0.001）、更高的中心静脉压（13（11-15）比 10（9-12）mmHg，P<0.001）和舒张末期压力（8（5-10）比 6（5-7）mmHg，P=0.001）、心室功能受损和窦性节律缺失与 LDS≥5 相关。较长的随访时间（OR 1.2（1.1-1.3），P<0.001）和更高的中心静脉压（OR 1.6（1.3-2.1），P<0.001）是 FALD 进展的唯一独立预测因素。腹部超声和实验室异常提示 FALD 在儿童和青少年时期的常规随访中已经很常见，与心室形态无关。更严重的发现与较长的随访时间和更高的中心静脉压有关。
