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

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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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Conflict of interest

Bruckheimer is a paid consultant for Atrium Medical Corp., Hudson, NH. The other authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Section of Pediatric Cardiology, Schneider Children’s Medical Center Israel, Kaplan 14, Petach Tikva, Israel

    Elchanan Bruckheimer, Tamir Dagan, Riccardo Superina, Gabriel Amir & Einat Birk

  2. Section of Radiology, Schneider Children’s Medical Center Israel, Kaplan 14, Petach Tikva, Israel

    Eli Atar & Michael Schwartz

  3. Section of Anesthesiology, Schneider Children’s Medical Center Israel, Kaplan 14, Petach Tikva, Israel

    Ludmila Kachko

  4. Section of Gastroenterology, Schneider Children’s Medical Center Israel, Kaplan 14, Petach Tikva, Israel

    Rivka Shapiro

Authors
  1. Elchanan Bruckheimer
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  2. Tamir Dagan
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  3. Eli Atar
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  4. Michael Schwartz
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  5. Ludmila Kachko
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  6. Riccardo Superina
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  7. Gabriel Amir
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  8. Rivka Shapiro
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  9. Einat Birk
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Corresponding author

Correspondence to Elchanan Bruckheimer.

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Bruckheimer, E., Dagan, T., Atar, E. et al. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children. Cardiovasc Intervent Radiol 36, 1580–1585 (2013). https://doi.org/10.1007/s00270-013-0581-7

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  • DOI: https://doi.org/10.1007/s00270-013-0581-7

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