Srinivasan Abhay, Guzman Anthony K, Rand Elizabeth B, Rychik Jack, Goldberg David J, Russo Pierre A, Cahill Anne Marie
Department of Radiology, Children's Hospital of Philadelphia, 3401 Civic Center Blvd., Radiology 3NW47, Philadelphia, PA, 19104, USA.
Children Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA, USA.
Pediatr Radiol. 2019 Mar;49(3):342-350. doi: 10.1007/s00247-018-4311-9. Epub 2018 Nov 30.
Patients who have undergone the Fontan operation for palliation of congenital heart disease with single-ventricle pathophysiology are at high risk for developing progressive liver fibrosis. Pathological assessment from percutaneous liver biopsy is central to the management of Fontan-associated liver disease, but liver biopsy in this vulnerable population poses unique challenges and potential risks.
This retrospective study describes our experience with percutaneous liver biopsy performed to assess changes of Fontan-associated liver disease, with particular regard to procedural outcomes.
Data from liver biopsy procedure reports, pathology reports, cardiac angiography pressure measurements and laboratory values of patients with single ventricle heart disease after the Fontan operation who underwent ultrasound-guided percutaneous liver biopsy performed in interventional radiology at a pediatric tertiary care center during a 3-year period were retrospectively analyzed.
Sixty-eight liver biopsies were performed in 67 patients (mean age: 20.2 years, range: 7.2-39 years). The technical success rate was 100%, and tissue was adequate for assessing liver disease in 100% of the procedures, including biopsies performed with a single pass. Anticoagulation was routinely suspended before biopsy, and no cardiac complications were encountered due to this suspension. A coaxial biopsy system using an 18-gauge (G) full-core instrument through a 17-G introducer trocar was most commonly used, in 41/68 cases (60%). The most common trough length was 2.3 cm, used in 37 cases (54%). One pass was made in 27 procedures (40%) and two passes in 30 (44%); tract embolization with gelatin sponge was performed in 52 (76%). The only complication was hemorrhage, which occurred in 5/68 (7.4%) of the biopsies, minor in four (5.9%) and major in one (1.5%) -- similar to rates reported for liver biopsy in non-Fontan patients. Hemorrhage had a delayed presentation in three of these five cases. Immediate post-biopsy hemoglobin decrease of ≥2 mg/dL showed a low sensitivity for hemorrhage. The mean Fontan pressure measured during cardiac angiography was 13.8 mmHg, and shunt pressures were not associated with an increased risk of hemorrhage.
Percutaneous liver biopsy in Fontan patients can be performed safely with high technical success rates and without increased complication rates. Meticulous technique and close observation are recommended to reduce post-biopsy complications. The degree of right heart pressure elevation was not associated with hemorrhage.
接受Fontan手术以缓解单心室病理生理的先天性心脏病患者发生进行性肝纤维化的风险很高。经皮肝活检的病理评估是Fontan相关性肝病管理的核心，但在这一脆弱人群中进行肝活检带来了独特的挑战和潜在风险。
这项回顾性研究描述了我们进行经皮肝活检以评估Fontan相关性肝病变化的经验，尤其关注手术结果。
回顾性分析了一家儿科三级护理中心在3年期间对接受Fontan手术后的单心室心脏病患者进行超声引导下经皮肝活检的肝活检手术报告、病理报告、心脏血管造影压力测量和实验室值的数据。
67例患者共进行了68次肝活检（平均年龄：20.2岁，范围：7.2 - 39岁）。技术成功率为100%，100%的手术中获取的组织足以评估肝脏疾病，包括单次穿刺活检。活检前常规暂停抗凝，未因这种暂停而出现心脏并发症。最常用的是通过17G穿刺套管针使用18G全芯器械的同轴活检系统，共41/68例（60%）。最常见的针道长度为2.3cm，共37例（54%）。27例手术（40%）进行了单次穿刺，30例（44%）进行了两次穿刺；52例（76%）使用明胶海绵进行了针道栓塞。唯一的并发症是出血，68例活检中有5例（7.4%）发生，4例（5.9%）为轻微出血，1例（1.5%）为严重出血——与非Fontan患者肝活检报告的发生率相似。这5例中有3例出血表现延迟。活检后即刻血红蛋白下降≥2mg/dL对出血的敏感性较低。心脏血管造影期间测得的平均Fontan压力为13.8mmHg，分流压力与出血风险增加无关。
Fontan患者的经皮肝活检可以安全进行，技术成功率高且并发症发生率不增加。建议采用精细技术并密切观察以减少活检后并发症。右心压力升高程度与出血无关。
