Department of Radiology, Section of Interventional Radiology, University of Washington Medical Center, Seattle, WA, USA.
Department of Radiology, Section of Interventional Radiology, Seattle Children's Hospital, 4800 Sand Point Way NE, M/S R-5417, Seattle, WA, 98105, USA.
Pediatr Radiol. 2020 Oct;50(11):1579-1586. doi: 10.1007/s00247-020-04751-9. Epub 2020 Jun 24.
Accurate and reproducible means of measuring the portosystemic gradient are essential for risk stratification and treatment of portal hypertension.
To report the reliability of hepatic venous pressure gradients in children with intrahepatic veno-venous collateralization.
Between January 2012 and December 2019 (96 months), 39 patients with native livers underwent wedge hepatic venography and hepatic venous pressure gradient measurements at a tertiary pediatric center. All archived images were reviewed for balloon isolation of the hepatic vein and hepatic vein-to-hepatic vein (HV-HV) collaterals. HV-HV collaterals were categorized as present on the basis of non-catheterized segmental venous opacification despite appropriate balloon isolation. Hepatic venous pressure gradient was defined as the difference of wedge and free hepatic venous pressures. Wedge portosystemic gradient was defined as the difference between wedge hepatic venous pressure and right atrial (RA) pressures. For patients subsequently undergoing portal venous catheterization, portosystemic gradient was defined as the difference between main portal vein and RA pressures.
Thirteen of 39 (33.3%) patients demonstrated HV-HV collaterals on wedge hepatic venography. The mean hepatic venous pressure gradient was 5.2±3.8 mmHg (range: 0-15 mmHg). The mean hepatic venous pressure gradient was 3.6±2.6 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 5.9±4.2 mmHg (range: 1-15 mmHg) in the absence of HV-HV collaterals (P=0.043). Twelve (30.8%) patients were found to have varices: 10 gastroesophageal, 1 rectal and 1 stomal. The mean hepatic venous pressure gradient in patients with varices was 5.4±47 mmHg (range: 0-15 mmHg). For patients with varices, mean hepatic venous pressure gradient was 3.0±2.7 mmHg (range: 0-9 mmHg) in the presence of HV-HV collaterals and 10.3±4.1 mmHg (range: 5-15 mmHg) in the absence of HV-HV collaterals (P=0.004). Four (10.3%) patients had extrahepatic portal vein occlusion: 3 with cavernous transformation and 1 with type Ib Abernethy malformation. All patients with extrahepatic portal vein occlusion demonstrated HV-HV collaterals compared with 8 of 35 (22.9%) patients without extrahepatic portal vein occlusion (P=0.002). Four of 39 (10.3%) patients underwent direct portal pressure measurements: 3 via transhepatic and 1 via trans-splenic portal access. All had demonstrated HV-HV collaterals on wedged imaging. One had extrahepatic portal vein occlusion. The mean time between wedge portosystemic gradient and portosystemic gradient measurement was 3.75 days (range: 0-8 days). The mean wedge portosystemic gradient was 4.5±3.1 mmHg (range: 2-9 mmHg) and the mean portosystemic gradient was 14.5±3.7 mmHg (range: 12-20 mmHg) (P=0.006).
HV-HV collateralization is frequently observed in children undergoing wedged portal venography and leads to misrepresentative hepatic venous pressure gradients. All patients undergoing hepatic venous pressure gradient measurement should have wedged venography to identify HV-HV collaterals and to qualify measured pressures. Additional techniques to obtain representative pressures in the presence of HV-HV collaterals warrant further investigation.
准确且可重现的测量门脉系统梯度的方法对于门静脉高压的风险分层和治疗至关重要。
报告在有肝内静脉-静脉侧支形成的儿童中测量肝静脉压力梯度的可靠性。
在 2012 年 1 月至 2019 年 12 月（96 个月）期间，39 例原发性肝病患者在一家三级儿科中心接受了楔形肝静脉造影和肝静脉压力梯度测量。所有存档的图像均进行了回顾，以评估球囊隔离肝静脉和肝静脉-肝静脉（HV-HV）侧支的情况。HV-HV 侧支的存在是基于尽管适当隔离了导管化的节段性静脉显影，但仍存在非导管化的静脉显影。肝静脉压力梯度定义为楔形和游离肝静脉压力之间的差异。楔形门脉系统梯度定义为楔形肝静脉压力与右心房（RA）压力之间的差异。对于随后接受门静脉导管插入术的患者，门脉系统梯度定义为主门静脉和 RA 压力之间的差异。
在 39 例患者中，有 13 例（33.3%）在楔形肝静脉造影中显示 HV-HV 侧支。平均肝静脉压力梯度为 5.2±3.8mmHg（范围：0-15mmHg）。在存在 HV-HV 侧支的情况下，平均肝静脉压力梯度为 3.6±2.6mmHg（范围：0-9mmHg），而在不存在 HV-HV 侧支的情况下，平均肝静脉压力梯度为 5.9±4.2mmHg（范围：1-15mmHg）（P=0.043）。12 例（30.8%）患者发现有静脉曲张：10 例胃食管静脉曲张，1 例直肠静脉曲张，1 例门脉静脉曲张。静脉曲张患者的平均肝静脉压力梯度为 5.4±47mmHg（范围：0-15mmHg）。对于有静脉曲张的患者，在存在 HV-HV 侧支的情况下，平均肝静脉压力梯度为 3.0±2.7mmHg（范围：0-9mmHg），而在不存在 HV-HV 侧支的情况下，平均肝静脉压力梯度为 10.3±4.1mmHg（范围：5-15mmHg）（P=0.004）。4 例（10.3%）患者有肝外门静脉闭塞：3 例为海绵状转化，1 例为 Ib 型 Abernethy 畸形。所有有肝外门静脉闭塞的患者均显示 HV-HV 侧支，而 35 例无肝外门静脉闭塞的患者中，有 8 例（22.9%）显示 HV-HV 侧支（P=0.002）。39 例患者中有 4 例（10.3%）进行了直接门脉压力测量：3 例经肝穿刺，1 例经脾穿刺门脉入路。所有患者在楔形成像上均显示 HV-HV 侧支。1 例有肝外门静脉闭塞。楔形门脉系统梯度和门脉系统梯度测量之间的平均时间为 3.75 天（范围：0-8 天）。平均楔形门脉系统梯度为 4.5±3.1mmHg（范围：2-9mmHg），平均门脉系统梯度为 14.5±3.7mmHg（范围：12-20mmHg）（P=0.006）。
在接受楔形门静脉造影的儿童中，HV-HV 侧支形成经常被观察到，导致肝静脉压力梯度的不代表性。所有接受肝静脉压力梯度测量的患者均应进行楔形静脉造影，以识别 HV-HV 侧支，并确定测量的压力。需要进一步研究在存在 HV-HV 侧支的情况下获得有代表性的压力的其他技术。
