✦ LIBER ✦
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.