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

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

doi:10.1002/ppul.21463

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

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

Research output: Contribution to journal › Review article › peer-review

9 Scopus citations

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.

Original language	English
Pages (from-to)	1041-1044
Number of pages	4
Journal	Pediatric Pulmonology
Volume	46
Issue number	10
DOIs	https://doi.org/10.1002/ppul.21463
State	Published - Oct 2011
Externally published	Yes

Keywords

heterotaxy
interrupted inferior vena cava
polysplenia
portopulmonary hypertension
portosystemic shunt
sudden death

Access to Document

10.1002/ppul.21463

Cite this

@article{0df11b6367ca45e28ed90c7c931a330d,

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.",

keywords = "heterotaxy, interrupted inferior vena cava, polysplenia, portopulmonary hypertension, portosystemic shunt, sudden death",

author = "Daisuke Kobayashi and Edwards, {Henry D.} and Jasmeet Singh and Nadkarni, {Milan D.} and Lantz, {Patrick E.} and Cook, {Amanda L.}",

year = "2011",

month = oct,

doi = "10.1002/ppul.21463",

language = "English",

volume = "46",

pages = "1041--1044",

journal = "Pediatric Pulmonology",

issn = "8755-6863",

number = "10",

}

TY - JOUR

T1 - Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia

T2 - A cause of sudden death in an infant

AU - Kobayashi, Daisuke

AU - Edwards, Henry D.

AU - Singh, Jasmeet

AU - Nadkarni, Milan D.

AU - Lantz, Patrick E.

AU - Cook, Amanda L.

PY - 2011/10

Y1 - 2011/10

N2 - 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.

AB - 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.

KW - heterotaxy

KW - interrupted inferior vena cava

KW - polysplenia

KW - portopulmonary hypertension

KW - portosystemic shunt

KW - sudden death

UR - http://www.scopus.com/inward/record.url?scp=80052968923&partnerID=8YFLogxK

U2 - 10.1002/ppul.21463

DO - 10.1002/ppul.21463

M3 - Review article

C2 - 21520436

AN - SCOPUS:80052968923

VL - 46

SP - 1041

EP - 1044

JO - Pediatric Pulmonology

JF - Pediatric Pulmonology

SN - 8755-6863

IS - 10

ER -

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

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this