Respiration. 2018;96(2):127-137. doi: 10.1159/000488000. Epub 2018 Apr 11.
The latest pulmonary hypertension (PH) guidelines define therapeutic goals in terms of symptoms, exercise capacity, and haemodynamics for patients with pulmonary arterial hypertension (PAH) and recommend advanced combined medical therapy. For inoperable or post-surgical residual distal chronic thromboembolic PH (CTEPH) medical treatment is similarly advised.
We analysed whether risk factor goals are achieved and combination therapy is used in prevalent patients with PAH or distal CTEPH.
PAH or distal CTEPH patients who were seen at the University Hospital Zurich during the last year were analysed in terms of demography, clinical data, medication, and therapeutic goals. Achievement of therapeutic goals was defined as New York Heart Association (NYHA) class ≤II, N-terminal pro-brain natriuretic peptide (NTpro-BNP) < 300 ng/L, and 6-min walking distance (6MWD) > 440 m.
A total of 108 PAH patients (age 59 ± 18 years, 62% female, 64% idiopathic, 36% associated) and 38 distal CTEPH patients (age 69 ± 14 years, 55% female) were included. They had been diagnosed on average 66 ± 48 months (±SD) previously. The percentage of PAH/CTEPH patients with NYHA ≤II was 52/53, respectfully, the percentage of those with NTproBNP < 300 ng/L was 61/52, and with 6MWD > 440 m 63/50. Overall, 33/31% fulfilled 3 and 29/35% fulfilled 2 of these goals. Regarding therapy, 43% of PAH patients were on double and 10% on triple combination therapy, whereas 16% of distal CTEPH patients were on double and 3% on triple combination therapy.
In this real-life cohort of prevalent patients with PAH or distal CTEPH, targeted drug therapy resulted in an achievement of ≥2/3 predefined therapeutic goals in 2/3 of patients. Patients with PAH were more likely to receive combination therapy compared to CTEPH patients.
最新的肺动脉高压（PH）指南根据症状、运动能力和血液动力学为肺动脉高压（PAH）患者定义了治疗目标，并建议采用先进的联合药物治疗。对于无法手术或手术后残留的慢性血栓栓塞性肺动脉高压（CTEPH），也建议采用药物治疗。
我们分析了在现患 PAH 或远端 CTEPH 患者中，是否达到了危险因素目标并采用了联合治疗。
对过去一年在苏黎世大学医院就诊的 PAH 或远端 CTEPH 患者进行了人口统计学、临床数据、药物和治疗目标的分析。治疗目标的实现定义为纽约心脏协会（NYHA）心功能分级≤Ⅱ级、N 末端脑利钠肽前体（NTpro-BNP）<300ng/L 和 6 分钟步行距离（6MWD）>440m。
共纳入 108 例 PAH 患者（年龄 59±18 岁，62%为女性，64%为特发性，36%与其他疾病相关）和 38 例远端 CTEPH 患者（年龄 69±14 岁，55%为女性）。他们的平均诊断时间为 66±48 个月（±SD）。NYHA≤Ⅱ级的 PAH/CTEPH 患者比例分别为 52/53，NTpro-BNP<300ng/L 的患者比例分别为 61/52，6MWD>440m 的患者比例分别为 63/50。总体而言，33/31%的患者达到了这三个目标中的三个，29/35%的患者达到了其中两个。在治疗方面，43%的 PAH 患者接受了双联药物治疗，10%的患者接受了三联药物治疗，而 16%的远端 CTEPH 患者接受了双联药物治疗，3%的患者接受了三联药物治疗。
在现患 PAH 或远端 CTEPH 患者的真实队列中，有 2/3 的患者通过靶向药物治疗达到了≥2/3 的既定治疗目标。与 CTEPH 患者相比，PAH 患者更有可能接受联合治疗。
