重新审视先天性心脏病中的肝纤维化风险：来自无创标志物和超声心动图的见解
Revisiting Hepatic Fibrosis Risk in Congenital Heart Disease: Insights from Non-Invasive Markers and Echocardiography.
作者信息
Yamazaki Fusako, Kamimura Hiroteru, Endo Saori, Miida Suguru, Maruyama Hiroki, Yoshida Tomoaki, Kumagai Masaru, Kimura Naruhiro, Abe Hiroyuki, Sakamaki Akira, Yokoo Takeshi, Tsukada Masanori, Numano Fujito, Saitoh Akihiko, Watanabe Maya, Shiraishi Shuichi, Tsuchida Masanori, Fujiki Shinya, Kashimura Takeshi, Inomata Takayuki, Nonaka Hirofumi, Kamimura Kenya, Tsuchiya Atsunori, Terai Shuji
机构信息
Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata 951-8510, Japan.
Department of Pediatrics, Graduate School of Medical and Dental Sciences, Niigata University, Niigata 950-3198, Japan.
出版信息
Children (Basel). 2025 Aug 27;12(9):1131. doi: 10.3390/children12091131.
BACKGROUND/OBJECTIVES: This study aimed to investigate the prevalence of liver damage and its associated non-invasive markers and echocardiographic risk factors in patients who underwent surgery for congenital heart disease.
METHODS
This retrospective observational study was conducted at a single tertiary-care university hospital in Niigata, Japan. Of 142 patients (ventricular septal defect [VSD] n = 47, tetralogy of Fallot [TOF] n = 67, Fontan n = 28), 52.8% were male [median age: 22.7 years; VSD (24.3 years), TOF (24.0 years), and Fontan (12.5 years)]. Pediatric patients with liver diseases unrelated to congestive liver disease, such as viral hepatitis and alcoholic liver disease, were excluded. We compared non-invasive liver fibrosis age-invariant biomarkers, such as the aspartate aminotransferase-to-platelet ratio index (APRI), and various serum markers and echocardiographic parameters to assess the prevalence and predictors of hepatic fibrosis.
RESULTS
The Fontan circulation group had the highest APRI, followed by the TOF group, while the VSD group had a low risk of APRI elevation. Postoperative TOF patients required monitoring for cirrhosis progression. Inferior vena cava mobility was associated with echocardiographic parameters and fibrosis severity, along with a loss of respiratory variability. The limitations of other cardiac assessments were highlighted by poor anatomical measurements. Gamma-glutamyl transpeptidase (γ-GTP) demonstrated strong discriminatory ability. The optimal cutoff value was 53.0 U/L, suggesting its use as a clinical marker.
CONCLUSIONS
Assessing fibrosis is crucial in CHD patients, especially those with late post-TOF repair findings. Non-invasive markers (APRI, γ-GTP, and B-type natriuretic peptide), along with echocardiographic findings, may help detect fibrosis early, enabling timely intervention and improving long-term outcomes.
CLINICAL TRIAL REGISTRATION
2020-0199.
背景/目的：本研究旨在调查先天性心脏病手术患者肝损伤的患病率及其相关的非侵入性标志物和超声心动图危险因素。
方法
本回顾性观察性研究在日本新潟的一家三级大学医院进行。142例患者（室间隔缺损[VSD]47例，法洛四联症[TOF]67例，Fontan手术28例）中，52.8%为男性[中位年龄：22.7岁；VSD（24.3岁），TOF（24.0岁），Fontan（12.5岁）]。排除患有与充血性肝病无关的肝病（如病毒性肝炎和酒精性肝病）的儿科患者。我们比较了非侵入性肝纤维化年龄不变生物标志物，如天冬氨酸氨基转移酶与血小板比值指数（APRI），以及各种血清标志物和超声心动图参数，以评估肝纤维化的患病率和预测因素。
结果
Fontan循环组的APRI最高，其次是TOF组，而VSD组APRI升高的风险较低。TOF术后患者需要监测肝硬化进展情况。下腔静脉活动度与超声心动图参数和纤维化严重程度相关，同时伴有呼吸变异性丧失。解剖测量不佳突出了其他心脏评估的局限性。γ-谷氨酰转肽酶（γ-GTP）显示出很强的鉴别能力。最佳临界值为53.0 U/L，表明其可作为临床标志物使用。
结论
评估纤维化对先天性心脏病患者至关重要，尤其是那些TOF修复术后晚期的患者。非侵入性标志物（APRI、γ-GTP和B型利钠肽）以及超声心动图结果可能有助于早期发现纤维化，从而实现及时干预并改善长期预后。
临床试验注册
2020-0199。
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