Department of Radiology, the First Hospital of Jilin University, No. 71 of Xinmin Street, Changchun, Jilin, 130021, China.
J Cardiothorac Surg. 2024 Jun 21;19(1):342. doi: 10.1186/s13019-024-02867-9.
Pulmonary arteriovenous malformation (PAVM), also known as pulmonary arteriovenous fistula, is a rare vascular developmental anomaly. Most cases of PAVM are associated with hereditary hemorrhagic telangiectasia (HHT). Hemothorax associated with PAVM is even rarer, and management concerning this complication still challenges.
A 55-year-old man with sudden onset of dyspnea and chest pain was admitted to our hospital. He had a medical history of epistaxis, intraperitoneal germ cell tumor and PAVM. Chest unenhanced CT revealed the left-sided pleural effusion together with partial passive atelectasis and gradual increase at the interval of six days. Diagnostic thoracocentesis further revealed hemorrhagic effusion. CT angiography (CTA) showed tortuously dilated lumen of the left lower pulmonary artery and PAVM with the formation of aneurysm. Due to his family's refusal of surgery, the patient underwent transcatheter embolization therapy. However, the left pleural effusion did not significantly reduce and there was a slow drop in hemoglobin value even after interventional treatment, indicating the possibility of ongoing active bleeding. Eventually, the patient received lobectomy of the left lower lobe with a satisfactory outcome.
Massive hemothorax resulting from PAVM rupture into the pleural space can lead to fatal outcomes. CTA can accurately diagnose this pathologic condition. Transcatheter embolization is frequently used in the treatment of PAVM, but it may be challenging to achieve the desirable effect in patients with hemothorax. Combined with our case and literature review, direct radical surgery can lead to a successful outcome when PAVM complicated with hemothorax and a large diameter of the draining vein.
肺动静脉畸形（PAVM），又称肺动静脉瘘，是一种罕见的血管发育异常。大多数 PAVM 病例与遗传性出血性毛细血管扩张症（HHT）相关。与 PAVM 相关的血胸更为罕见，对这种并发症的处理仍然具有挑战性。
一名 55 岁男性，突发呼吸困难和胸痛，收入我院。他有鼻出血、腹腔内生殖细胞肿瘤和 PAVM 的病史。胸部未增强 CT 显示左侧胸腔积液伴部分被动性肺不张，六天间隔逐渐增加。诊断性胸腔穿刺进一步显示血性渗出液。CT 血管造影（CTA）显示左下肺动脉迂曲扩张的管腔和 PAVM，形成动脉瘤。由于患者家属拒绝手术，患者接受了经导管栓塞治疗。然而，左侧胸腔积液并未明显减少，即使在介入治疗后血红蛋白值也缓慢下降，表明可能存在持续活动性出血。最终，患者接受了左下肺叶切除术，结果满意。
PAVM 破裂至胸膜腔引起的大量血胸可导致致命后果。CTA 可准确诊断这种病理状况。经导管栓塞术常用于治疗 PAVM，但在血胸患者中可能难以达到理想效果。结合我们的病例和文献复习，当 PAVM 合并血胸和引流静脉直径较大时，直接根治性手术可获得成功。
