Rehabilitation of the Patient with Hepatopulmonary Syndrome Who had Living-related Orthotopic Liver Transplantation

Hepatopulmonary syndrome (HPS) is a rare but severe complication for patients with liver disease. So far, there are no reports as to rehabilitation approaches for patients with HPS. We report a case of HPS patient and introduce the approaches of rehabilitation to them. The patient is a 17-year-old woman with HPS who had portal hypertension after Kasai's operation for congenital biliary atresia and had living related orthotopic liver transplantation (LT). After LT, her allograft functioned well but she continued to have hypoxemia and orthodeoxia. She was referred to us for rehabilitation to disuse atrophy, contracture of hip and shoulder joints, left common peroneal nerve palsy and rehabilitation to respiratory dysfunction. By the time of 103 days after LT, her orthodeoxia was improved by supine exercise and ADL training and her disuse atrophy was markedly improved by exercise and ADL training. Special consideration as to rehabilitation was needed for this patient. Patients with orthodeoxia secondary to HPS respond poorly to oxygen therapy, and their recovery from hypoxemia is slow after LT. We recommend supine exercise for rehabilitation intervention early after LT surgery because hypoxemia probably caused by unevenness of ventilation-perfusion in equality becomes minimized by supine posture.