Respiratory and physical therapy in a girl with very severe hepatopulmonary syndrome after liver transplantation: a case report

Abstract

Introduction: Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary vascular dilation and hypoxemia in patients with chronic liver disease. Bubble contrast echocardiography is the gold standard for its diagnosis. Liver transplantation (LT) is the only definitive treatment, and refractory hypoxemia (RH) is its most common complication. The objective of this case report was to describe the respiratory and physical therapy and the clinical course of a patient with very severe HPS secondary to a congenital portosystemic shunt with post-transplant HR.

Case presentation: An 11-year-old female patient with HPS secondary to a congenital extrahepatic portosystemic shunt underwent an LT after 64 days on the waiting list. She developed RH post-transplantation and required extracorporeal membrane oxygenation for 28 days. She remained on mechanical ventilation for 85 days and in the intensive care unit for 87 days. Due to acquired weakness and diaphragmatic dysfunction, a tracheostomy was performed to facilitate respiratory and motor rehabilitation.

Conclusion: The treatment of a patient with severe HPS who developed RH after an LT was described. The treatment focused on managing RH and, in the long term, on weaning from mechanical ventilation and motor reconditioning.