A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient / 대한내과학회지

ABSTRACT

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.