Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center

Background : 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. Materials and Methods : This retrospective single-center study analyzed the clinical profile and medium-term outcome of i...

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Main Authors: Usha Mandikal Kodandarama Sastry, Madhu Swandenahalli Doddaiah, Priyadarshini Arunakumar, Varun Marimuthu, Sowmya Kasturi, Budnur C Srinivas, Mahimarangaiah Jayranganath, Cholenahally Nanjappa Manjunath
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-01-01
Series:Annals of Pediatric Cardiology
Subjects:
Online Access:http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=168;epage=174;aulast=Kodandarama
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author Usha Mandikal Kodandarama Sastry
Madhu Swandenahalli Doddaiah
Priyadarshini Arunakumar
Varun Marimuthu
Sowmya Kasturi
Budnur C Srinivas
Mahimarangaiah Jayranganath
Cholenahally Nanjappa Manjunath
author_facet Usha Mandikal Kodandarama Sastry
Madhu Swandenahalli Doddaiah
Priyadarshini Arunakumar
Varun Marimuthu
Sowmya Kasturi
Budnur C Srinivas
Mahimarangaiah Jayranganath
Cholenahally Nanjappa Manjunath
author_sort Usha Mandikal Kodandarama Sastry
collection DOAJ
description Background : 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. Materials and Methods : 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. Results : 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. Conclusion : 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.
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spelling doaj.art-74d00e3c8eb54a87ab031b56ba715eb22023-10-26T05:47:19ZengWolters Kluwer Medknow PublicationsAnnals of Pediatric Cardiology0974-20692023-01-0116316817410.4103/apc.apc_160_22Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary centerUsha Mandikal Kodandarama SastryMadhu Swandenahalli DoddaiahPriyadarshini ArunakumarVarun MarimuthuSowmya KasturiBudnur C SrinivasMahimarangaiah JayranganathCholenahally Nanjappa ManjunathBackground : 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. Materials and Methods : 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. Results : 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. Conclusion : 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.http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=168;epage=174;aulast=Kodandaramabudd–chiari syndromeportal hypertensiontranshepatic access
spellingShingle Usha Mandikal Kodandarama Sastry
Madhu Swandenahalli Doddaiah
Priyadarshini Arunakumar
Varun Marimuthu
Sowmya Kasturi
Budnur C Srinivas
Mahimarangaiah Jayranganath
Cholenahally Nanjappa Manjunath
Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
Annals of Pediatric Cardiology
budd–chiari syndrome
portal hypertension
transhepatic access
title Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
title_full Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
title_fullStr Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
title_full_unstemmed Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
title_short Percutaneous hepatic vein recanalization in pediatric Budd–Chiari syndrome – 10 years' experience from a tertiary center
title_sort percutaneous hepatic vein recanalization in pediatric budd chiari syndrome 10 years experience from a tertiary center
topic budd–chiari syndrome
portal hypertension
transhepatic access
url http://www.annalspc.com/article.asp?issn=0974-2069;year=2023;volume=16;issue=3;spage=168;epage=174;aulast=Kodandarama
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