Brown Morgan L, Cedeño Alex R, Edell Eric S, Hagler Donald J, Schaff Hartzell V
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Ann Thorac Surg. 2009 Jul;88(1):233-7. doi: 10.1016/j.athoracsur.2009.04.012.
Fibrosing mediastinitis is a rare disease characterized by an excessive fibrotic reaction in the mediastinum, which may entrap mediastinal structures including the pulmonary arteries. Our objectives were to assess the surgical strategies and outcomes of repair of pulmonary artery occlusion attributable to mediastinal fibrosis.
With approval from the Mayo Clinic Institutional Review Board, we identified all patients with fibrosing mediastinitis who underwent an operation for relief of pulmonary artery obstruction between 1980 and 2008. Perioperative data were collected using medical records and late follow-up surveys.
Operative procedures to bypass or reconstruct an obstructed pulmonary artery were performed in 5 patients. Patients' median age was 40 years (range, 27 to 51 years), and all patients were symptomatic and had right ventricular hypertension. In 3 patients, a double-outlet right ventricle was constructed using a valved conduit (porcine valved conduit, n = 1; aortic homograft, n = 2) from the right ventricle to the right pulmonary artery. Two patients required complete reconstruction of the pulmonary artery confluence using a pulmonary homograft in 1 patient and a hybrid technique of autologous pericardial reconstruction and intraoperative stenting in another patient. All patients had a reduction in right ventricular pressures after operation. One patient died perioperatively owing to respiratory failure; the remaining 4 patients were alive at a median follow-up of 7.4 years (range, 0.5 to 14.7 years). One patient required late balloon dilatation of the conduit and distal pulmonary arteries 10 years after initial operation, but the remaining conduits were widely patent at late follow-up. Late functional improvement was limited owing to other complications from mediastinal fibrosis or other comorbidities.
Treatment of pulmonary artery occlusion attributable to mediastinal fibrosis can be challenging. Successful operative strategies include both creation of a double-outlet right ventricle and complete reconstruction of the pulmonary artery confluence. Hybrid techniques of both conduit placement and stenting should also be considered for patients with occluded pulmonary arteries.
纤维性纵隔炎是一种罕见疾病，其特征为纵隔内出现过度纤维化反应，这可能会压迫包括肺动脉在内的纵隔结构。我们的目标是评估因纵隔纤维化导致的肺动脉闭塞的手术策略及修复结果。
经梅奥诊所机构审查委员会批准，我们确定了1980年至2008年间所有因纤维性纵隔炎接受手术以缓解肺动脉梗阻的患者。通过病历和后期随访调查收集围手术期数据。
5例患者接受了绕过或重建阻塞肺动脉的手术。患者的中位年龄为40岁（范围为27至51岁），所有患者均有症状且存在右心室高压。3例患者使用带瓣管道（猪带瓣管道，1例；主动脉同种异体移植物，2例）构建了右心室双出口，从右心室连接至右肺动脉。2例患者需要完全重建肺动脉汇合处，其中1例使用肺同种异体移植物，另1例采用自体心包重建与术中支架置入的联合技术。所有患者术后右心室压力均降低。1例患者围手术期因呼吸衰竭死亡；其余4例患者在中位随访7.4年（范围为0.5至14.7年）时存活。1例患者在初次手术后10年需要对管道和远端肺动脉进行后期球囊扩张，但其余管道在后期随访时仍保持通畅。由于纵隔纤维化或其他合并症的其他并发症，后期功能改善有限。
因纵隔纤维化导致的肺动脉闭塞的治疗具有挑战性。成功的手术策略包括构建右心室双出口和完全重建肺动脉汇合处。对于肺动脉闭塞的患者，还应考虑管道置入和支架置入的联合技术。
