Department of General Surgery, Georgetown University School of Medicine, MedStar Washington Hospital Center, Washington, DC, USA.
Department of Pulmonology, MedStar Washington Hospital Center, Washington, DC, USA.
J Cardiothorac Surg. 2023 Jun 29;18(1):199. doi: 10.1186/s13019-023-02255-9.
Asymptomatic, isolated cases of unilateral pulmonary artery atresia may present in adulthood with symptoms such as recurrent respiratory infections, dyspnea, hemoptysis, and pulmonary hypertension. Unlike previously reported patients that underwent surgical management for this pathology, the patient in this report had no chronic history of recurrent respiratory infections, dyspnea, or pulmonary hypertension, making a diagnosis prior to extensive imaging difficult.
A 55-year-old male presented to our emergency department (ED) with a 3-day history of recurrent cough with 2-3 tablespoons of hemoptysis per episode, chills, and occasional wheezing. A computed tomography angiography (CTA) was performed, which identified a congenital absence of the left pulmonary artery and a right-sided aortic arch. Hypertrophied left intercostal and bronchial arteries were noted to be perfusing the left lung. V/Q scan confirmed a heterogeneous distribution of gas throughout both lung fields with 97% perfusion to the right lung, but no visualization of the left lung on the perfusion images. Given extensive collateral blood supply to the left lung, interventional radiology performed a GELFOAM® embolization of the hypertrophied left bronchial artery and two parasitized arteries from the left subclavian artery to minimize intra-operative blood loss. This was immediately followed by a left thoracotomy, pneumonectomy, intercostal muscle flap placement, and bronchoscopy. The procedure was 360 min long with a total of 1500 cc blood loss that was salvaged and re-infused. No additional blood products were administered. The patient remained intubated post-operatively and was transferred to the surgical intensive care unit. His postoperative course was complicated by troponin leak, rhabdomyolysis, delirium, and ileus, all of which resolved over time. He was discharged home on postoperative day seven and continues to do well one-year later.
The patient in this report presented with several episodes of isolated hemoptysis but unlike previously reported cases of unilateral pulmonary artery atresia, he had no history of recurrent respiratory infections, dyspnea, or pulmonary hypertension. Although unilateral pulmonary artery atresia is a rare diagnosis, in patients with unexplained, isolated hemoptysis, further examination of the vasculature may be warranted, and surgical management may be beneficial in appropriate, symptomatic patients.
孤立性无症状肺动脉瓣闭锁在成人中可能表现为复发性呼吸道感染、呼吸困难、咯血和肺动脉高压等症状。与之前报道的接受该病理手术治疗的患者不同，本报告中的患者没有复发性呼吸道感染、呼吸困难或肺动脉高压的慢性病史，因此在进行广泛的影像学检查之前难以做出诊断。
一名 55 岁男性因反复咳嗽 3 天就诊，每次咳嗽咳出 2-3 勺咯血，伴有寒战和偶尔喘息。进行了计算机断层血管造影（CTA）检查，发现左肺动脉先天性缺失和右位主动脉弓。观察到左肋间和支气管动脉增粗，为左肺供血。通气灌注扫描证实两肺均存在气体分布不均，右肺灌注 97%，但灌注图像上未见左肺。鉴于左肺有广泛的侧支循环供血，介入放射科对增粗的左支气管动脉和来自左锁骨下动脉的两条寄生动脉进行了 GELFOAM®栓塞，以尽量减少术中失血。紧接着进行了左开胸、肺切除术、肋间肌瓣放置和支气管镜检查。手术持续 360 分钟，总失血量为 1500cc，全部回收并重新输入。未使用其他血液制品。患者术后仍需插管，并转入外科重症监护病房。他的术后过程中出现肌钙蛋白漏、横纹肌溶解、意识障碍和肠梗阻，随着时间的推移这些问题都得到了解决。他在术后第 7 天出院，一年后情况良好。
本报告中的患者出现了几次孤立性咯血，但与之前报道的单侧肺动脉闭锁病例不同，他没有复发性呼吸道感染、呼吸困难或肺动脉高压的病史。虽然单侧肺动脉闭锁是一种罕见的诊断，但对于不明原因孤立性咯血的患者，可能需要进一步检查血管情况，对于有症状的合适患者，手术治疗可能是有益的。
