Article (Scientific journals)
Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.
Joye, Raphael; Lador, Frédéric; Aggoun, Yacine et al.
2020In Journal of Hepatology, 74 (3), p. 742-747
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Keywords :
Liver transplantation; Medical treatment; Paediatrics; Portal hypertension; Portosystemic shunts; Pulmonary arterial hypertension
Abstract :
[en] Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.
Disciplines :
Cardiovascular & respiratory systems
Pediatrics
Author, co-author :
Joye, Raphael
Lador, Frédéric
Aggoun, Yacine
FARHAT, Nesrine  ;  Centre Hospitalier Universitaire de Liège - CHU > Département de Pédiatrie > Service de pédiatrie
Wacker, Julie
Wildhaber, Barbara Elisabeth
Vallée, Jean-Paul
Hachulla, Anne-Lise
McLin, Valérie Anne
Beghetti, Maurice
Language :
English
Title :
Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.
Publication date :
01 December 2020
Journal title :
Journal of Hepatology
ISSN :
0168-8278
eISSN :
1600-0641
Publisher :
Elsevier, Netherlands
Volume :
74
Issue :
3
Pages :
742-747
Peer reviewed :
Peer Reviewed verified by ORBi
Commentary :
Copyright © 2020 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
Available on ORBi :
since 15 February 2021

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