Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant vascular disorder characterized by mucocutaneous telangiectasias and visceral arteriovenous malformations (AVMs), leading to recurrent bleeding and multisystem complications. Pulmonary arteriovenous malformations (PAVMs) are a major cause of morbidity due to hemoptysis, hypoxemia, and paradoxical embolic events. We report the case of a 63-year-old man with a known diagnosis of HHT and a history of endovascular embolization of a pulmonary AVM who presented with acute epistaxis, oral bleeding, and massive hemoptysis. Laboratory evaluation revealed anemia with normal platelet count and coagulation profile. Computed tomography pulmonary angiography demonstrated previously embolized AVM in the left upper lobe with surrounding ground-glass opacities suggestive of pulmonary hemorrhage. The patient was managed conservatively with antifibrinolytic therapy, blood transfusion, antimicrobial treatment, blood pressure control, and supportive care, resulting in clinical stabilization without recurrence of massive bleeding during hospitalization. This case highlights the persistent risk of hemorrhagic complications in patients with HHT despite prior definitive treatment and emphasizes the need for lifelong surveillance and multidisciplinary management to reduce morbidity and prevent life-threatening events.
WILSON et al. (Sun,) studied this question.