title: Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: a cause of sudden death in an infant.
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.
authors.affiliation: Department of Pediatrics, Wake Forest University School of Medicine, Winston-Salem, North Carolina. dkobayas@dmc.org
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kobayashi
authors.forename: Henry D
authors.initials: HD
authors.lastname: Edwards
authors.forename: Jasmeet
authors.initials: J
authors.lastname: Singh
authors.forename: Milan D
authors.initials: MD
authors.lastname: Nadkarni
authors.forename: Patrick E
authors.initials: PE
authors.lastname: Lantz
authors.forename: Amanda L
authors.initials: AL
authors.lastname: Cook
mesh_terms: D003645:Death, Sudden / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D059446:Heterotaxy Syndrome / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000209:etiology; D007223:Infant; D011168:Portal System / Q000002:abnormalities*; D011169:Portal Vein