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 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.