AimsTo determine the impact of anesthesia encountered and to optimize the treatment of perioperative pulmonary arterial hypertension (PAH) in an effort to improve perioperative management and reduce complications.MethodsWe conducted a retrospective analysis of scoliosis patients with PAH who underwent scoliosis surgery.ResultsDuring this period, we identified a total of 22 patients. Their mean age was 22.18 ± 2.11 years. 16 PAH patients (72.72%) received PAH-specific treatment. Only Propofol-based TIVA was used intraoperatively. During the procedure, pulmonary artery catheters and PICCO catheters were placed in all patients to monitor intraoperative and postoperative mPAP, MAP, PRVI and SRVI. During tracheal intubation and intraoperative awake testing, mPAP generally tended to increase in all patients. 6 patients (27.27%) received intraoperative PAH-Specific therapy. All patients received oral sildenafil (75-100 mg/d orally), and 9 patients received postoperative oral sildenafil combined with nebulized iloprost (20 μg/d); intravenous treprostinil (2 ng/kg/min started and titrated to 10-17.5 ng/kg/min); or bosentan (250 mg/d) postoperatively. 7 patients (31.82%) reported postoperative complications, including 2 cases of respiratory failure requiring reintubation, 1 case of right heart failure, 2 cases of superficial surgical site infection, 1 case of fluid and electrolyte and acid-base imbalances, 2 cases of pneumonia and 1 case of pulmonary oedema with fluid overload. Two patients developed more than 1 postoperative complication. No in-hospital death occurred.ConclusionsThe anesthetic management of scoliosis patients with PAH is important task that, like its own surgery, relies on the input of the multidisciplinary team for its success. Close monitoring, optimization of systemic blood pressure, pain control, oxygenation and ventilation, avoidance of exacerbating factors, and the use of vasopressors and pulmonary vasodilators when necessary are essential elements of management.

中文翻译：

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严重脊柱侧凸合并肺动脉高压的管理：单中心回顾性病例系列研究

目的确定麻醉对围手术期肺动脉高压（PAH）的影响，优化围手术期肺动脉高压（PAH）的治疗，以改善围手术期管理，减少并发症。方法我们对接受脊柱侧弯手术的PAH脊柱侧凸患者进行回顾性分析。我们共确定了 22 名患者。他们的平均年龄为 22.18 ± 2.11 岁。16名PAH患者（72.72%）接受了PAH特异性治疗。术中仅使用基于丙泊酚的 TIVA。手术过程中，所有患者均置入肺动脉导管和PICCO导管，监测术中和术后mPAP、MAP、PRVI和SRVI。在气管插管和术中清醒测试期间，所有患者的 mPAP 通常都有增加的趋势。6名患者（27. 27%) 接受了术中 PAH 特异性治疗。所有患者均口服西地那非（75～100 mg/d口服），9例患者术后口服西地那非联合雾化伊洛前列素（20 μg/d）；静脉注射曲前列环素（2 ng/kg/min 开始，滴定至 10-17.5 ng/kg/min）；或波生坦（250 mg/d）术后。术后并发症7例（31.82%），其中呼吸衰竭需重新插管2例，右心衰竭1例，手术部位浅表感染2例，水电解质及酸碱失衡1例，肺炎2例1例肺水肿伴液体超负荷。两名患者出现超过 1 种术后并发症。未发生院内死亡。结论脊柱侧弯合并肺动脉高压患者的麻醉管理是一项重要任务，就像它自己的手术一样，它的成功依赖于多学科团队的投入。密切监测、优化全身血压、控制疼痛、氧合和通气、避免恶化因素以及在必要时使用血管加压药和肺血管扩张剂是管理的基本要素。