Department of Cardiovascular and Respiratory Science, I School of Medicine, University of Rome Sapienza, Policlinico Umberto I.
J Heart Lung Transplant. 2012 Apr;31(4):364-72. doi: 10.1016/j.healun.2011.12.011. Epub 2012 Jan 29.
Oral drugs have made the treatment of pulmonary hypertension (PH) feasible in non-expert centers, which could delay patient access to prostanoid therapy.
Fifty-seven consecutive patients with precapillary PH received a prostanoid in our center. Data at prostanoid initiation included modality of center referral, medical history, New York Heart Association [NYHA] class, exercise capacity, echocardiographic parameters, and hemodynamics.
Overall survival at 1, 2, and 3 years was 85%, 69%, 55%, respectively. Non-survivors had worse NYHA class III/IV (17/12) than survivors (27/1; p < 0.01) and exercise capacity on 6-minute-walk distance (254 ± 114 vs 354 ± 91 meters; p < 0.01). Non-survivors were more frequently referred on oral therapy (83% vs 36%; p < 0.01) and had a higher rate of urgent prostanoid treatment (69% vs 17%; p < 0.0001). Multivariate analysis (hazard ratio [95% confidence interval]) found the independent prognostic factors were urgent prostanoid therapy (2.0 [1.1-3.9]) and NYHA class (3.5 [1.5-8.2]). Survivors had a significant response to prostanoid, improving NYHA class from 2.8 ± 0.4 to 2.3 ± 0.5 (p = 0.002), 6-minute walk distance from 354 ± 91 to 426 ± 82 meters (p = 0.0001), and pulmonary hemodynamics (pulmonary artery pressure from 56 ± 13 to 44 ± 18 mm Hg [p < 0.05]; cardiac index from 2.0 ± 1.2 to 3.1 ± 1.2 liters/min/m(2) [p = 0.002], and pulmonary vascular resistance from 17 ± 10 to 8 ± 6 WU [p = 0.001]).
Referral of patients on oral treatment to a tertiary PH center is delayed and significantly affects prognosis.
口服药物使肺动脉高压（PH）的治疗在非专家中心成为可能，这可能会延迟患者接受前列环素治疗的时间。
我们中心为 57 例毛细血管前 PH 患者使用前列环素治疗。前列环素起始时的数据包括中心转诊方式、病史、纽约心脏协会（NYHA）心功能分级、运动能力、超声心动图参数和血液动力学。
1、2、3 年总生存率分别为 85%、69%、55%。非幸存者 NYHA 心功能分级为 III/IV 级（17/12）的比例明显高于幸存者（27/1；p < 0.01），6 分钟步行距离的运动能力也更低（254 ± 114 比 354 ± 91 米；p < 0.01）。非幸存者更常接受口服治疗（83%比 36%；p < 0.01），更频繁地需要紧急前列环素治疗（69%比 17%；p < 0.0001）。多变量分析（危险比[95%置信区间]）发现独立的预后因素是紧急前列环素治疗（2.0[1.1-3.9]）和 NYHA 心功能分级（3.5[1.5-8.2]）。幸存者对前列环素有显著反应，NYHA 心功能分级从 2.8 ± 0.4 改善至 2.3 ± 0.5（p = 0.002），6 分钟步行距离从 354 ± 91 改善至 426 ± 82 米（p = 0.0001），肺血流动力学（肺动脉压从 56 ± 13 改善至 44 ± 18 mm Hg[p < 0.05]；心指数从 2.0 ± 1.2 改善至 3.1 ± 1.2 升/分钟/米 2[p = 0.002]，肺血管阻力从 17 ± 10 改善至 8 ± 6 伍德单位[p = 0.001]）。
口服治疗患者转诊至三级 PH 中心延迟，显著影响预后。
