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

Wynton Hoover1, Veda Ackerman, Marcus Schamberger

  • 1Division of Pediatric Pulmonology, Riley Hospital for Children, Indiana University, Indianapolis, Indiana, USA. whoover@Peds.UAB.edu

Pediatric Pulmonology
|December 19, 2007
PubMed

Insights

Congenital porto-caval fistula caused severe pulmonary hypertension and hyperammonemia in a teen. Endovascular stent placement successfully treated the fistula, resolving symptoms and improving cognitive function.

Area of Science:

  • Vascular Surgery
  • Pediatric Cardiology
  • Hepatology

Background:

  • Congenital porto-caval fistulas are rare vascular anomalies.
  • These malformations can present with diverse symptoms across all age groups.

Observation:

  • A 14-year-old male presented with dyspnea and cough.
  • He had a history of chronic hemolytic anemia, thrombocytopenia, coagulopathy, and learning disability.

Findings:

  • The patient was diagnosed with severe pulmonary hypertension and hyperammonemia due to a large congenital porto-caval fistula.
  • Endovascular covered stent placement in the vena cava successfully occluded the abnormal vessel.

Implications:

  • Successful treatment led to remarkable improvement in pulmonary hypertension.
  • Normalization of anemia, thrombocytopenia, and ammonia levels allowed for enhanced cognitive performance.

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