Abstract
Pulmonary arterial hypertension (PAH) is a rare and severe disease characterised by the progressive narrowing of the small pulmonary arteries resulting in right-sided heart failure. Despite advances in treatment, some PAH patients require lung transplantation, which carries risks of long-term complications. We report the case of a male patient with no significant comorbidities, who developed progressive dyspnoea on exertion at the age of 38 years. After echocardiography and right heart catheterisation, he was confirmed with idiopathic PAH and was started treatment with bosentan, sildenafil and beraprost. After 3 years of stability, the PAH worsened. Treatment with continuous intravenous epoprostenol was introduced in a national premiere, unfortunately with little effect. The patient underwent lung transplantation at the age of 43 years. Afterwards, he developed several complications, including chronic lung allograft dysfunction, post-transplant lymphoproliferative disorder and pulmonary embolism. He died at the age of 52 years of invasive pulmonary aspergillosis. The case is noteworthy due to the above-average post-transplant survival despite multiple complications.