Rupture of pulmonary arteriovenous fistula in the setting of hereditary hemorrhagic teleangiectasia

A 49-year-old man presented with a 2-hour history of right-sided chest pain and mental status changes. He reported having spontaneous recurrent nosebleeds, which also occurred in his father. Mucocutaneous telangiectasia was found in his right thigh. His-temperature was 36.0 °C. The blood pressure was 68/43 mm Hg on arrival. Laboratory evaluation revealed the following: white blood cell count, 18.2 × 10⁹/L; red blood cell count, 3.84 × 10¹²/L; blood platelet, 180 × 10⁹/L; N%, 95.4%; hemoglobin 116 g/L. C-reactive protein was normal (0.64 mg/L). Tumor markers were normal, including carcinoembryonic antigen and neuron-specific enolase. Chest computed tomography (CT) revealed a large amount of fluid in the right thorax and lung nodules (black arrow) in the right lower lobe (Fig. 1A-B). Chest computed tomography angiography (CTA) showed pulmonary arteriovenous fistula (PAVF) in the right lower lobe (white arrows), with one feeding artery and one draining vein (Figure 1C-D). He was diagnosed with right hemothorax due to PAVF rupture, and emergency surgery was performed. Approximately, 2500 ml of blood and blood clots were found in the right thoracic cavity. After removal of the blood and blood clots, a PAVF lesion with active bleeding was found in the lateral basal segment, approximately 2.0 cm in diameter. Video-assisted thoracoscopic surgery was performed for pulmonary wedge resection of the right lower lobe. He was discharged 6 days after surgery.