2-Year Follow-Up of Lung Transplantation as a Treatment of Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)

Hemorrhagic telangiectasia (HHT) is a disease of initially mild course - manifesting with recurrent nosebleeds and increased fatigue. Nevertheless, its progression can deteriorate patient’s health. Solid organ transplantation becomes the only therapeutic option to save a life. The case report describes a 19-year-old female patient who was diagnosed with HHT and qualified for lung transplantation. She met the Curacao criteria for HHT (¾). Her health deteriorated significantly to the point of the referral to Department of Cardiac, Vascular and Endovascular Surgery and Transplantology in Silesian Center for Heart Diseases. Due to her condition, she was qualified for lung transplantation as one diagnosed with pulmonary arteriovenous malformations and then transplanted at the age of 17. A direct postoperative period was complicated by HSV2 infection of the wound. 18 months after the procedure, the patient underwent acute cholangitis. The presence of portal and systemic fistulas was noted and the final diagnosis of HHT was made. Despite the fact that proper diagnosis was made posttransplant, it was a good treatment. The patient is currently 2 years after the lung transplantation and feels good. Lung transplantation is a viable therapeutic option for patients with HHT as there are reports of other patients who have benefited from lung transplantation after other therapeutic options were exhausted.