Sastry Usha Mandikal Kodandarama, Doddaiah Madhu Swandenahalli, Arunakumar Priyadarshini, Marimuthu Varun, Kasturi Sowmya, Srinivas Budnur C, Jayranganath Mahimarangaiah, Manjunath Cholenahally Nanjappa
Department of Pediatric Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bengaluru, Karnataka, India.
Department of Radiodiagnosis, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India.
Ann Pediatr Cardiol. 2023 May-Jun;16(3):168-174. doi: 10.4103/apc.apc_160_22. Epub 2023 Sep 8.
Budd - Chiari syndrome (BCS) due to hepatic venous outflow obstruction is a rare cause of liver disease with dismal outcome, often amenable to catheter intervention.
This retrospective single-center study analyzed the clinical profile and medium-term outcome of interventional treatment with balloon angioplasty ± stenting in all pediatric BCS over a 10-year period. Clinical, laboratory, imaging, and interventional data were retrieved. Transhepatic (TH) access was utilized in the recent 3 years.
We included a total of 27 patients. Acute and subacute BCS comprised 93% of subjects. Ascites was the most common symptom. COVID-19 infection and Takayasu arteritis were two novel etiologies in our study. There was isolated hepatic vein (HV) narrowing in 11 (41%), isolated inferior vena cava obstruction in 4, and combined occlusion in 12 (44%). Intervention was successful in 22 (82%) patients. Stenting was required in 14 (64%) patients and the rest underwent balloon angioplasty. The immediate outcome was better with stenting than balloon (91% vs. 64%). Transhepatic access in 6 patients allowed HV cannulation in all and achieved patency in five patients. Two patients from the balloon group (25%) and 9 from the stent group (64%) are alive with patent veins at a median follow-up of 60 months, indicating a high attrition rate.
Catheter interventions restored physiological blood flow in pediatric BCS. TH route improved cannulation of occluded HV compared to other accesses. Immediate and medium-term outcomes were better after stenting with lower rates of reinterventions than balloon angioplasty. Life-long surveillance is required as mortality is high on follow-up.
肝静脉流出道梗阻所致布加综合征（BCS）是一种罕见的肝脏疾病病因，预后不佳，常需导管介入治疗。
这项回顾性单中心研究分析了10年间所有儿童BCS患者行球囊血管成形术±支架置入术的临床特征及中期预后。收集临床、实验室、影像学及介入治疗数据。最近3年采用经肝（TH）入路。
共纳入27例患者。急性和亚急性BCS患者占93%。腹水是最常见的症状。新型冠状病毒肺炎感染和高安动脉炎是本研究中的两种新病因。单纯肝静脉（HV）狭窄11例（41%），单纯下腔静脉梗阻4例，联合闭塞12例（44%）。22例（82%）患者介入治疗成功。14例（64%）患者需要置入支架，其余患者接受球囊血管成形术。支架置入术的即刻效果优于球囊血管成形术（91%对64%）。6例患者采用经肝入路，均成功实现HV插管，5例实现血管通畅。球囊组2例（25%）和支架组9例（64%）患者在中位随访60个月时静脉通畅存活，提示损耗率较高。
导管介入治疗恢复了儿童BCS的生理性血流。与其他入路相比，经肝入路改善了闭塞HV的插管成功率。支架置入术后的即刻和中期预后较好，再次干预率低于球囊血管成形术。由于随访期间死亡率较高，需要终身监测。
