Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: A cause of sudden death in an infant

Daisuke Kobayashi, Henry D. Edwards, Jasmeet Singh, Milan D. Nadkarni, Patrick E. Lantz, Amanda L. Cook

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations

Abstract

Congenital extrahepatic portosystemic shunt (CEPS) is associated with polysplenia and heterotaxy and can cause portopulmonary hypertension. We report a 12-month-old girl who acutely died likely due to portopulmonary hypertension secondary to CEPS associated with heterotaxy and polysplenia. A retrospective radiographic review following her autopsy identified an anatomical explanation for the acute death in an infant.

Original languageEnglish
Pages (from-to)1041-1044
Number of pages4
JournalPediatric Pulmonology
Volume46
Issue number10
DOIs
StatePublished - Oct 2011
Externally publishedYes

Keywords

  • heterotaxy
  • interrupted inferior vena cava
  • polysplenia
  • portopulmonary hypertension
  • portosystemic shunt
  • sudden death

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