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

Division of Cardiology

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

12 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

Keywords

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

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10.1002/ppul.21463

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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",

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pages = "1041--1044",

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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 - https://www.scopus.com/pages/publications/80052968923

U2 - 10.1002/ppul.21463

DO - 10.1002/ppul.21463

M3 - Review article

C2 - 21520436

AN - SCOPUS:80052968923

SN - 8755-6863

VL - 46

SP - 1041

EP - 1044

JO - Pediatric Pulmonology

JF - Pediatric Pulmonology

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

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