Premium
Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: A cause of sudden death in an infant
Author(s) -
Kobayashi Daisuke,
Edwards Henry D.,
Singh Jasmeet,
Nadkarni Milan D.,
Lantz Patrick E.,
Cook Amanda L.
Publication year - 2011
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.21463
Subject(s) - medicine , heterotaxy , polysplenia , portosystemic shunt , portopulmonary hypertension , portal hypertension , autopsy , cardiology , surgery , cirrhosis , heart disease , situs inversus
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.