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

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

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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.

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