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

✍ Scribed by Daisuke Kobayashi; Henry D. Edwards; Jasmeet Singh; Milan D. Nadkarni; Patrick E. Lantz; Amanda L. Cook


Publisher
John Wiley and Sons
Year
2011
Tongue
English
Weight
518 KB
Volume
46
Category
Article
ISSN
8755-6863

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✦ Synopsis


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. Pediatr. Pulmonol. 2011; 46:1041–1044. © 2011 Wiley‐Liss, Inc.