The congenital porto-caval fistula: A unique presentation and novel intervention

Wynton Hoover, Veda Ackerman, Marcus Schamberger, Manjusha Kumar, Francis Marshalleck, Mark Hoyer

Research output: Contribution to journalArticle

7 Scopus citations

Abstract

Congenital porto-caval tistulas are uncommon vascular malformations with a varied clinical presentation beginning in intancy and spanning late adulthood. We report a 14-year-old male who presented with dyspnea and cough. His past medical history was signiticant tor a chronic non-immune hemolytic anemia, thrombocytopenia, coagulopathy and a learning disability. He was found to have severe pulmonary hypertension and hyperammonemia associated with a large congenital porto-caval tistula. The abnormal vessel was occluded via endovascular covered stent placement in the vena cava. His pulmonary hypertension has improved remarkably while his chronic anemia, thrombocytopenia and ammonia have normalized to allow improved cognitive performance.

Original languageEnglish (US)
Pages (from-to)196-199
Number of pages4
JournalPediatric pulmonology
Volume43
Issue number2
DOIs
StatePublished - Feb 1 2008

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Keywords

  • Abernethy malformation
  • Congenital porto-caval fistula
  • Hemolytic anemia
  • Hyperammonemia
  • Portosystemic shunt
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

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