2-year follow-up of Lung transplantation as a treatment of Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) - a case report
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. Case series describes 19 years old female patient, who was diagnosed with HHT and qualified for lung transplantation. She meets Curacao criteria for HHT (¾). Her health deteriorated significantly to the point of the referral to Covered for Blind Review. 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. Direct postoperative period was complicated by HSV2 infection of the wound. 18 months after the procedure, patient underwent acute cholangitis. 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 transplant 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.