Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children

Elchanan Bruckheimer*, Tamir Dagan, Eli Atar, Michael Schwartz, Ludmila Kachko, Riccardo Superina, Gabriel Amir, Rivka Shapiro, Einat Birk

*Corresponding author for this work

Research output: Contribution to journalArticle

9 Citations (Scopus)

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 ~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 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

Original languageEnglish (US)
Pages (from-to)1580-1585
Number of pages6
JournalCardioVascular and Interventional Radiology
Volume36
Issue number6
DOIs
StatePublished - Dec 1 2013

Fingerprint

Surgical Portasystemic Shunt
Hyperammonemia
Portal Pressure
Stents
Growth
Therapeutics
Pressure
Superior Mesenteric Artery
Portal Hypertension
Ammonia
Angiography
Equipment and Supplies

Keywords

  • Abernethy
  • Portal hypoplasia
  • Portosystemic shunt
  • Reducing stent

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Bruckheimer, Elchanan ; 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. In: CardioVascular and Interventional Radiology. 2013 ; Vol. 36, No. 6. pp. 1580-1585.
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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 ~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 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.",
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Bruckheimer, E, Dagan, T, Atar, E, Schwartz, M, Kachko, L, Superina, R, Amir, G, Shapiro, R & Birk, E 2013, 'Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children', CardioVascular and Interventional Radiology, vol. 36, no. 6, pp. 1580-1585. https://doi.org/10.1007/s00270-013-0581-7

Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children. / Bruckheimer, Elchanan; Dagan, Tamir; Atar, Eli; Schwartz, Michael; Kachko, Ludmila; Superina, Riccardo; Amir, Gabriel; Shapiro, Rivka; Birk, Einat.

In: CardioVascular and Interventional Radiology, Vol. 36, No. 6, 01.12.2013, p. 1580-1585.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children

AU - Bruckheimer, Elchanan

AU - Dagan, Tamir

AU - Atar, Eli

AU - Schwartz, Michael

AU - Kachko, Ludmila

AU - Superina, Riccardo

AU - Amir, Gabriel

AU - Shapiro, Rivka

AU - Birk, Einat

PY - 2013/12/1

Y1 - 2013/12/1

N2 - 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 ~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 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

AB - 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 ~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 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

KW - Abernethy

KW - Portal hypoplasia

KW - Portosystemic shunt

KW - Reducing stent

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U2 - 10.1007/s00270-013-0581-7

DO - 10.1007/s00270-013-0581-7

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JO - CardioVascular and Interventional Radiology

JF - CardioVascular and Interventional Radiology

SN - 7415-5101

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