TY - JOUR
T1 - Pulmonary Arteriovenous Malformations in Heterotaxy Syndrome
T2 - The Case for Early, Direct Hepatic Vein–to–Azygos Vein Connection
AU - Burstein, Danielle S.
AU - Mavroudis, Constantine
AU - Puchalski, Michael D.
AU - Stewart, Robert D.
AU - Blanco, Carlos J.
AU - Jacobs, Marshall L.
N1 - Copyright:
Copyright 2016 Elsevier B.V., All rights reserved.
PY - 2011/1
Y1 - 2011/1
N2 - We report a surgical approach using hepatic vein–to–azygos vein connection without cardiopulmonary bypass or deep hypothermic circulatory arrest in a patient with heterotaxy syndrome and interrupted inferior vena cava with persistence of pulmonary arteriovenous malformations (PAVMs) after previous Fontan completion. We advocate early performance of hepatic vein–to–azygos vein connection following the Kawashima operation for heterotaxy with functionally univentricular heart and interrupted inferior vena cava. We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population.
AB - We report a surgical approach using hepatic vein–to–azygos vein connection without cardiopulmonary bypass or deep hypothermic circulatory arrest in a patient with heterotaxy syndrome and interrupted inferior vena cava with persistence of pulmonary arteriovenous malformations (PAVMs) after previous Fontan completion. We advocate early performance of hepatic vein–to–azygos vein connection following the Kawashima operation for heterotaxy with functionally univentricular heart and interrupted inferior vena cava. We review the physiology of heterotaxy syndrome with congenital heart disease and justify our approach in the context of a review of previous surgical strategies used in this patient population.
KW - CHD univentricular heart
KW - Fontan
KW - heterotaxy
KW - pulmonary arteriovenous malformations
UR - http://www.scopus.com/inward/record.url?scp=84990339303&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84990339303&partnerID=8YFLogxK
U2 - 10.1177/2150135110387310
DO - 10.1177/2150135110387310
M3 - Article
C2 - 23804943
AN - SCOPUS:84990339303
SN - 2150-1351
VL - 2
SP - 119
EP - 128
JO - World Journal for Pediatric and Congenital Hearth Surgery
JF - World Journal for Pediatric and Congenital Hearth Surgery
IS - 1
ER -