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Expert consensus on clinical practice for the diagnosis and treatment of hepatic vascular diseases （2026 edition）
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摘要: 肝脏血管性疾病（如门静脉血栓、门静脉肝窦血管病、遗传性出血性毛细血管扩张症、动脉门静脉瘘、肝窦阻塞综合征、布-加综合征等）临床表现多样、诊断困难、治疗方案多样，给临床诊疗带来巨大挑战，目前国内尚无肝脏血管性疾病诊治指南或共识。为规范并提高我国肝脏血管性疾病的诊疗水平，中国研究型医院学会肝病（中西医结合）专业委员会、中国研究型医院学会介入医学专业委员会、北京医师协会门静脉高压症多学科诊治医师分会组织相关领域专家，基于最新循证医学证据，结合我国临床实践，围绕肝脏血管性疾病的临床诊断与治疗，制定本共识。Abstract: Hepatic vascular diseases （such as portal vein thrombosis， porto-sinusoidal vascular disease， hereditary hemorrhagic telangiectasia， arterioportal fistula， and sinusoidal obstruction syndrome， Budd-Chiari syndrome） are characterized by diverse clinical manifestations， difficulties in diagnosis， and various treatment regimens， which brings huge challenges to clinical diagnosis and treatment， and at present， there are still no guidelines or consensus statements on the diagnosis and treatment of hepatic vascular diseases in China. To standardize and improve the diagnosis and treatment of hepatic vascular diseases in China， Committee on Liver Diseases （Integrated Traditional Chinese and Western Medicine） of China Research Hospital Association， Committee on Interventional Medicine of China Research Hospital Association， and Branch for Multidisciplinary Diagnosis and Treatment of Portal Hypertension of Beijing Medical Doctor Association organized the experts in related fields to develop expert consensus on clinical practice for the diagnosis and treatment of hepatic vascular diseases （2025 edition） based on the latest evidence-based medical research and the clinical practice in China， with a focus on the diagnosis and treatment of hepatic vascular diseases.
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Key words:
- Hepatic Vascular Diseases /
- Diagnosis /
- Therapeutics /
- Hypertension， Portal /
- Practice Guideline
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注： a，增强CT横轴位显示肝静脉层面图；b、c，门静脉及左、右分支层面图。CT，计算机体层成像；IVC，下腔静脉；LHV，肝左静脉；MHV，肝中静脉；RHV，肝右静脉；MPV，门静脉主干；LPV，门静脉左支；RAPV，门静脉右前支；RPPV，门静脉右后支。
图 1 肝静脉与门静脉增强CT示意图
Figure 1. Diagram of hepatic vein and portal vein on contrast-enhanced CT
注： a、b， CTA最大密度投影（MIP）显示肝动脉系统；c， CTA容积再现技术（VR）显示肝动脉系统；d、e， MIP、VR技术显示门静脉系统。CTA，计算机体层血管造影；CA，腹腔干；CHA，肝总动脉；SpA，脾动脉；PHA，肝固有动脉；GDA，胃十二指肠动脉；SMA，肠系膜上动脉；RHA，肝右动脉；LHA，肝左动脉；LGA，胃左动脉。
图 2 肝血管CTA示意图
Figure 2. Diagram of hepatic vessels on CTA
注： FHVP，肝静脉游离压力；RAP，右心房压力；WHVP，肝静脉楔压；IVCP，下腔静脉压力；PVP，门静脉压力；ISP，脾内压力；HAP，肝动脉压力。
图 3 肝脏血管测压位点及压力值
Figure 3. Pressure measuring sites of hepatic vessels and pressure values
注： PSVD（a～c）：门静脉管壁增厚，管腔狭小（a，苏木精-伊红染色，×200）；结节性再生性增生，萎缩的肝板围绕增生的肝板形成结节状（b，网织染色，×100）；不全间隔肝纤维化/肝硬化，纤细的不完全的纤维隔不规则分隔肝实质（c，网织染色，×100）。HSOS（d、e）：中央区肝窦扩张、肝板萎缩、肝细胞坏死消失（d，苏木精-伊红染色，×200）；中央静脉内膜增生、管腔闭塞（e，箭头，Masson 三色染色，×400）。BCS（f）：中央静脉伸出纤维化带分隔肝实质，周围肝实质内肝窦扩张淤血（f，苏木精-伊红染色，×100）。PSVD，门脉肝窦血管病；PV，门静脉；HE，苏木精-伊红；NRH，结节性再生性增生；HSOS，肝窦阻塞综合征；BCS，布加综合征。
图 4 PSVD、HSOS及BCS病理图
Figure 4. Pathological diagram of PSVD， HSOS and BCS
注： NCPVT，非硬化性门静脉血栓；PVT，门静脉血栓；GOV，食管胃静脉曲张；TIPS，经颈静脉肝内门体分流术；NSBB，非选择性β 受体阻滞剂；PLT，血小板；SMVT，肠系膜上静脉血栓。
图 5 PVT的治疗流程
Figure 5. Treatment process of PVT
表 1 牛津大学循证医学中心证据分级（2011版）
Table 1. Oxford Centre for Evidence-based Medicine - levels of evidence （2011 edition）
证据级别 描述 1 基于RCT的系统评价，全或无研究（即干预前所
有患者结局一致，干预后几乎全部转变）、效应量
大的观察性研究2 单个RCT研究、效应量大的观察性研究 3 非RCT 研究，随访研究 4 病例系列，病例对照研究、回顾性对照研究 5 专家意见（基于机制推理） 注：RCT，随机对照试验。
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表 2 GRADE推荐标准
Table 2. GRADE recommendation system
推荐强度 描述 A 强烈推荐：明确显示干预措施利大于弊或
弊大于利B 较弱推荐：利弊不明确或无论证据质量高
低均显示利弊相当注：GRADE，推荐分级的评估、制定与评价。
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表 3 肝脏血管性疾病分类及其致病因素
Table 3. Classification and etiology of hepatic vascular diseases
疾病分类 相关疾病 主要致病因素 肝动脉系统及其延伸 肝动脉狭窄（血栓或外科手术后）
动脉瘤
缺血性胆管病
动脉门静脉瘘
遗传性出血性毛细血管扩张症
脾动脉盗血综合征后天性因素（包括机械性损伤、医源性损伤
等）、先天发育异常、遗传因素、感染等门静脉系统及其延伸 门静脉阻塞性疾病（包括PVT）
脾静脉阻塞性疾病（包括脾静脉血栓）
肠系膜静脉阻塞性疾病（包括肠系膜上静脉血栓、
肠系膜下静脉血栓）
门静脉肝窦血管病
先天性门体分流（包括Abernethy综合征等）
门静脉瘤血栓形成、门静脉纤维化、机械性及药物性肝损
伤、先天发育异常、遗传因素、肿瘤、胰腺疾病、
感染等肝窦系统 肝窦阻塞综合征
肝窦阻塞相关性疾病（肝淀粉样变、镰状细胞病、
骨髓纤维化、戈谢病、恶性肿瘤等引起肝窦阻塞）
肝紫斑病遗传因素、细胞浸润或物质沉积性疾病、血液
系统疾病、药源性损伤等肝静脉系统及其延伸 布-加综合征
肉芽肿性静脉炎
淤血性肝病
肝静脉瘤发育异常、血栓性疾病、机械性及药物性肝损
伤、心源性疾病、感染等
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表 4 不同类型PH血管压力特征
Table 4. Features of vascular pressure in different types of PH
类型 FHVP WHVP HVPG RAP IVCP PVP ISP PPG 肝前性 → → → → → ↑ ↑ ↑ 窦前性 → ↑ →↑ → → ↑ ↑ ↑ 窦性 → ↑ →↑ → → ↑ ↑ ↑ 窦后性 → ↑ →↑ → →↑ ↑ ↑ →↑ 肝后性 ↑ ↑ →↑ →↑ ↑ ↑ ↑ →↑ 注：↑表示压力升高，→表示压力正常，→↑表示压力正常或升高。FHVP，肝静脉游离压力；WHVP，肝静脉楔压；HVPG，肝静脉压力梯度；RAP，右心房压力；IVCP，下腔静脉压；PVP，门静脉压力；ISP，脾内压力；PPG，门静脉压力梯度。
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表 5 HSCT-HSOS与PA-HSOS的诊断标准
Table 5. Diagnostic criteria for HSCT-HSOS and PA-HSOS
诊断 EBMT-HSCT-HSOS精细分类（成人） PA-HSOS 疑似 HSCT后（经典型21天内，迟发型21天后）出现以下其中两项：
胆红素≥34.2 μmol/L、体重增加5%、腹水、肝区胀痛肝肿大、超
声和/或弹性成像提示HSOS腹胀和/或肝区疼痛、肝大和腹水；血清总胆红素升高或
其他肝功能异常；典型的增强CT或MRI表现临床 HSCT后出现总胆红素≥34.2 μmol/L且包括以下其中两项：体重
增加5%、腹水、肝区胀痛肝肿大上述症状加上有明确服用含PA植物史或者血清吡咯蛋
白加合物阳性，同时排除其他已知病因所致肝损伤确诊 组织学证据 组织学证据 注：HSCT，造血干细胞移植；HSOS，肝窦阻塞综合征；PA，吡咯生物碱；EBMT， 欧洲血液与骨髓移植学会；CT，计算机体层成像；MRI，磁共振成像。
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