BACKGROUND Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressures managed by vasodilator therapies. Current guidelines encourage PAH management in specialty care centers (SCCs), but evidence is sparse regarding improvement in clinical outcomes and correlation to vasodilator use with referral. We hypothesize that PAH management at SCCs is associated with improved clinical outcomes. METHODS A single-center, retrospective study was performed at UPMC (overseeing 40 hospitals). PAH patients were identified between 2008-2018 and classified into a SCC or non-SCC cohort. Cox proportional hazard model was compared for all-cause mortality and negative binomial regression modeling for hospitalizations. Vasodilator therapy was included to adjust outcomes. RESULT Of 580 PAH patients at UPMC, 455 (78%) were managed at the SCC, comprising a younger (58.8 vs. 64.8 years, p<0.001) and female (68.4% vs. 51.2%, p<0.001) population with more comorbidities without differences in race or income. SCC demonstrated improved survival (HR=0.68, p = 0.012), fewer hospitalizations (IR=0.54, p<0.001), and provided more frequent disease monitoring. Early patient referral to SCC (<6 months of diagnosis) was associated with improved outcomes compared to non-SCC. SCC patients were more frequently prescribed vasodilators (p<0.001) and carried more diagnostic PAH coding (p<0.001). Vasodilators were associated with improved outcomes irrespective of location but without statistical significance when comparing between locations (p>0.05). CONCLUSION The UPMC SCC demonstrated improved outcomes in mortality and hospitalizations. SCC Benefit was multifactorial, with more frequent vasodilator therapy and disease monitoring. These findings provide robust evidence for early and regular referral of PAH patients to SCCs.

中文翻译：

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肺动脉高压的结果在专科护理中心得到改善

背景肺动脉高压(PAH)的特征在于通过血管扩张剂疗法控制的肺动脉压升高。目前的指南鼓励在专业护理中心 (SCC) 进行 PAH 管理，但关于临床结果的改善以及与血管扩张剂使用与转诊的相关性的证据很少。我们假设 SCC 的 PAH 管理与改善的临床结果相关。方法 在 UPMC（监督 40 家医院）进行了一项单中心、回顾性研究。PAH 患者在 2008 年至 2018 年间被确定，并分为 SCC 或非 SCC 队列。比较 Cox 比例风险模型的全因死亡率和住院的负二项式回归模型。包括血管扩张剂治疗以调整结果。结果 UPMC 的 580 名 PAH 患者，455 (78%) 名患者在 SCC 接受治疗，包括年轻（58.8 岁与 64.8 岁，p<0.001）和女性（68.4% 与 51.2%，p<0.001），合并症更多，种族或收入没有差异。SCC 证明生存率提高（HR=0.68，p=0.012），住院率降低（IR=0.54，p<0.001），并提供更频繁的疾病监测。与非 SCC 相比，早期患者转诊至 SCC（<6 个月的诊断）与改善的结果相关。SCC 患者更常使用血管扩张剂 (p<0.001) 并携带更多诊断性 PAH 编码 (p<0.001)。无论位置如何，血管扩张剂都与改善的结果相关，但在位置之间进行比较时没有统计学意义（p>0.05）。结论 UPMC SCC 在死亡率和住院治疗方面表现出改善的结果。SCC 的好处是多因素的，血管扩张剂治疗和疾病监测更频繁。这些发现为 PAH 患者早期和定期转诊至 SCC 提供了强有力的证据。