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Title: Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

Abstract

Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8more » {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.« less

Authors:
;  [1]; ;  [2];  [3]; ;  [1];  [4];  [1]
  1. Schneider Children's Medical Center Israel, Section of Pediatric Cardiology (Israel)
  2. Schneider Children's Medical Center Israel, Section of Radiology (Israel)
  3. Schneider Children's Medical Center Israel, Section of Anesthesiology (Israel)
  4. Schneider Children's Medical Center Israel, Section of Gastroenterology (Israel)
Publication Date:
OSTI Identifier:
22207977
Resource Type:
Journal Article
Journal Name:
Cardiovascular and Interventional Radiology
Additional Journal Information:
Journal Volume: 36; Journal Issue: 6; Other Information: Copyright (c) 2013 Springer Science+Business Media New York and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE); http://www.springer-ny.com; Country of input: International Atomic Energy Agency (IAEA); Journal ID: ISSN 0174-1551
Country of Publication:
United States
Language:
English
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; AMMONIA; ARTERIES; BIOMEDICAL RADIOGRAPHY; BYPASSES; CHILDREN; PATIENTS; SURGERY; VASCULAR DISEASES

Citation Formats

Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net, Dagan, Tamir, Atar, Eli, Schwartz, Michael, Kachko, Ludmila, Superina, Riccardo, Amir, Gabriel, Shapiro, Rivka, and Birk, Einat. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children. United States: N. p., 2013. Web. doi:10.1007/S00270-013-0581-7.
Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net, Dagan, Tamir, Atar, Eli, Schwartz, Michael, Kachko, Ludmila, Superina, Riccardo, Amir, Gabriel, Shapiro, Rivka, & Birk, Einat. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children. United States. doi:10.1007/S00270-013-0581-7.
Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net, Dagan, Tamir, Atar, Eli, Schwartz, Michael, Kachko, Ludmila, Superina, Riccardo, Amir, Gabriel, Shapiro, Rivka, and Birk, Einat. Sun . "Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children". United States. doi:10.1007/S00270-013-0581-7.
@article{osti_22207977,
title = {Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children},
author = {Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net and Dagan, Tamir and Atar, Eli and Schwartz, Michael and Kachko, Ludmila and Superina, Riccardo and Amir, Gabriel and Shapiro, Rivka and Birk, Einat},
abstractNote = {Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.},
doi = {10.1007/S00270-013-0581-7},
journal = {Cardiovascular and Interventional Radiology},
issn = {0174-1551},
number = 6,
volume = 36,
place = {United States},
year = {2013},
month = {12}
}