Perioperative management of severe portopulmonary hypertension in a paediatric living donor liver transplantation using pulmonary vasodilators

Portopulmonary hypertension (PoPH) is a rare but serious disorder carrying a very poor prognosis. PoPH is defined as the presence of pulmonary arterial hypertension (PAH) associated with portal hypertension, with or without underlying liver disease. We are reporting our institutional experience of a...

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Bibliographic Details
Published in:Journal of liver transplantation 2023-08, Vol.11, p.100158, Article 100158
Main Authors: Jolly, Annu Sarin, Metri, Vidyadhar, Goja, Sanjay K., Dave, Nandini, Thorat, Ashok, Vaibhav, Singh, Manoj
Format: Article
Language:English
Subjects:
iNO
Paediatric LDLT
Paediatric PoPH
Sildenafil
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Summary:Portopulmonary hypertension (PoPH) is a rare but serious disorder carrying a very poor prognosis. PoPH is defined as the presence of pulmonary arterial hypertension (PAH) associated with portal hypertension, with or without underlying liver disease. We are reporting our institutional experience of anaesthetic management of an 8-year old female child weighing 20 kg with severe PoPH who underwent liver transplantation (LT). The use of pulmonary vasodilators in the perioperative period along with a carefully planned anaesthetic strategy was critical in the successful outcome of this potentially lethal disorder.
ISSN:2666-9676
2666-9676
DOI:10.1016/j.liver.2023.100158