2016
DOI: 10.5935/1678-9741.20160020
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Abstract: Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's system… Show more

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Cited by 3 publications
(3 citation statements)
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“…Reports of intrahepatic VVC presenting with profound cyanosis following the Fontan operation are rare and usually associated with visceral heterotaxy and a nest of intrahepatic venous plexus. The few reported cases that involved occlusion of these intrahepatic VVC were associated with a favorable outcome [5][6][7][8][9][10]. Veno-venous collaterals closure is still debatable since high-risk patients (patients with elevated central venous pressure or heterotaxies) might benefit from the natural fenestrations created by the veno-venous collaterals.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Reports of intrahepatic VVC presenting with profound cyanosis following the Fontan operation are rare and usually associated with visceral heterotaxy and a nest of intrahepatic venous plexus. The few reported cases that involved occlusion of these intrahepatic VVC were associated with a favorable outcome [5][6][7][8][9][10]. Veno-venous collaterals closure is still debatable since high-risk patients (patients with elevated central venous pressure or heterotaxies) might benefit from the natural fenestrations created by the veno-venous collaterals.…”
Section: Discussionmentioning
confidence: 99%
“…Reports of intrahepatic VVC presenting with profound cyanosis following the Fontan operation are rare and usually associated with visceral heterotaxy and a nest of intrahepatic venous plexus. The few reported cases that involved occlusion of these intrahepatic VVC were associated with a favorable outcome [5][6][7][8][9][10].…”
Section: Introductionmentioning
confidence: 99%
“…In patients with advanced fibrosis, portal hypertension must be due to an added sinusoidal component, since the frequent development of decompressive porto-systemic shunts. In fact, the numerous presence of fistulas between the hepatic and portal veins could be another source of underestimation of sinusoidal pressure [63,64]. Figure 13.7 presents the distinctive features of portal hypertension in FALD.…”
Section: Hepatic Hemodynamics In Faldmentioning
confidence: 99%