Transcatheter closure of congenital portosystemic shunts – A multicenter experience

Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their ou...

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Main Authors: Nageswara Rao Koneti, Shweta Bakhru, Mahimarangaiah Jayranganath, Mahesh Kappanayil, Prashant Bobhate, Lakshmivenkateshiah Srinivas, Snehal Kulkarni, Usha Mandikal Kodandarama Sastry, Raman Krishna Kumar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2022-01-01
Series:Annals of Pediatric Cardiology
Subjects:
Online Access:http://www.annalspc.com/article.asp?issn=0974-2069;year=2022;volume=15;issue=2;spage=114;epage=120;aulast=Koneti
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author Nageswara Rao Koneti
Shweta Bakhru
Mahimarangaiah Jayranganath
Mahesh Kappanayil
Prashant Bobhate
Lakshmivenkateshiah Srinivas
Snehal Kulkarni
Usha Mandikal Kodandarama Sastry
Raman Krishna Kumar
author_facet Nageswara Rao Koneti
Shweta Bakhru
Mahimarangaiah Jayranganath
Mahesh Kappanayil
Prashant Bobhate
Lakshmivenkateshiah Srinivas
Snehal Kulkarni
Usha Mandikal Kodandarama Sastry
Raman Krishna Kumar
author_sort Nageswara Rao Koneti
collection DOAJ
description Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.
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spelling doaj.art-565c7ef33ffb4cfc8f5944c572862dde2022-12-22T04:02:23ZengWolters Kluwer Medknow PublicationsAnnals of Pediatric Cardiology0974-20692022-01-0115211412010.4103/apc.apc_5_22Transcatheter closure of congenital portosystemic shunts – A multicenter experienceNageswara Rao KonetiShweta BakhruMahimarangaiah JayranganathMahesh KappanayilPrashant BobhateLakshmivenkateshiah SrinivasSnehal KulkarniUsha Mandikal Kodandarama SastryRaman Krishna KumarBackground: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.http://www.annalspc.com/article.asp?issn=0974-2069;year=2022;volume=15;issue=2;spage=114;epage=120;aulast=Konetidevice closureportal vein anomalyportosystemic shuntsabernethy malformationpulmonary arteriovenous malformationpulmonary hypertensiontranscatheter closure
spellingShingle Nageswara Rao Koneti
Shweta Bakhru
Mahimarangaiah Jayranganath
Mahesh Kappanayil
Prashant Bobhate
Lakshmivenkateshiah Srinivas
Snehal Kulkarni
Usha Mandikal Kodandarama Sastry
Raman Krishna Kumar
Transcatheter closure of congenital portosystemic shunts – A multicenter experience
Annals of Pediatric Cardiology
device closure
portal vein anomaly
portosystemic shunts
abernethy malformation
pulmonary arteriovenous malformation
pulmonary hypertension
transcatheter closure
title Transcatheter closure of congenital portosystemic shunts – A multicenter experience
title_full Transcatheter closure of congenital portosystemic shunts – A multicenter experience
title_fullStr Transcatheter closure of congenital portosystemic shunts – A multicenter experience
title_full_unstemmed Transcatheter closure of congenital portosystemic shunts – A multicenter experience
title_short Transcatheter closure of congenital portosystemic shunts – A multicenter experience
title_sort transcatheter closure of congenital portosystemic shunts a multicenter experience
topic device closure
portal vein anomaly
portosystemic shunts
abernethy malformation
pulmonary arteriovenous malformation
pulmonary hypertension
transcatheter closure
url http://www.annalspc.com/article.asp?issn=0974-2069;year=2022;volume=15;issue=2;spage=114;epage=120;aulast=Koneti
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