Calcium channel blockers (CCB) are the first effective therapy for vasoreactive patients with idiopathic pulmonary arterial hypertension (IPAH). However, the advent of modern PAH-specific drugs may undermine the role of vasoreactivity tests and CCB treatment. We aimed to clarify the effect of acute vasoreactivity testing and CCB on patients with IPAH receiving PAH-specific treatment. We retrospectively investigated consecutive patients with IPAH ( = 136) diagnosed between 2000 and 2020 and collected data from patients who underwent acute vasoreactivity testing using inhaled nitric oxide (NO). The effects of vasoreactivity testing and CCB therapy were reviewed. Long-term survival was analysed using the Kaplan–Meier method. Acute vasoreactivity testing was performed in 49% of patients with IPAH ( = 67), including 23 patients (34%) receiving PAH-specific therapy without vasoreactivity testing. Eight patients (12%), including three patients (4.4%) receiving PAH-specific therapy, presented acute responses at vasoreactivity testing. They received high-dose CCB therapy (CCB monotherapy for five patients [7.5%] and CCB therapy and PAH-specific therapy for three patients [4.4%]). They presented a significant improvement in clinical parameters and near-normalisation of haemodynamics (mean pulmonary arterial pressure decreased from 46 [interquartile range: 40–49] to 19.5 [interquartile range: 18–23] mmHg [ < .001] at 1-year follow-up). All eight vasoreactive responders receiving CCB therapy showed better long-term survival than non-responders treated with PAH-specific therapy ( < .001). CCB therapy benefited patients with IPAH who showed acute response to vasoreactivity testing using inhaled NO, even when receiving modern PAH-specific therapy. Acute vasoreactive responders may benefit more from CCB than from PAH-specific therapy.

中文翻译：

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接受 PAH 特异性治疗的肺动脉高压患者中的钙通道阻滞剂

钙通道阻滞剂（CCB）是治疗特发性肺动脉高压（IPAH）血管反应性患者的第一个有效疗法。然而，现代 PAH 特异性药物的出现可能会削弱血管反应性测试和 CCB 治疗的作用。我们的目的是阐明急性血管反应性测试和 CCB 对接受 PAH 特异性治疗的 IPAH 患者的影响。我们回顾性调查了 2000 年至 2020 年间诊断的连续 IPAH 患者 (= 136)，并收集了接受吸入一氧化氮 (NO) 急性血管反应性测试的患者的数据。回顾了血管反应性测试和 CCB 治疗的效果。使用 Kaplan-Meier 方法分析长期生存。 49% 的 IPAH 患者 (= 67) 进行了急性血管反应性测试，其中 23 名患者 (34%) 接受了 PAH 特异性治疗但未进行血管反应性测试。 8 名患者 (12%)，包括 3 名接受 PAH 特异性治疗的患者 (4.4%)，在血管反应性测试中出现急性反应。他们接受了大剂量 CCB 治疗（5 名患者 [7.5%] 接受 CCB 单一治疗，3 名患者 [4.4%] 接受 CCB 治疗和 PAH 特异性治疗）。他们的临床参数显着改善，血流动力学接近正常化（平均肺动脉压在 1 年时从 46 [四分位距：40–49] 降至 19.5 [四分位距：18–23] mmHg [ < .001]跟进）。接受 CCB 治疗的所有 8 名血管反应性反应者均表现出比接受 PAH 特异性治疗的无反应者更好的长期生存率 (< .001)。 CCB 治疗使 IPAH 患者受益，即使在接受现代 PAH 特异性治疗时，他们对吸入 NO 的血管反应性测试也表现出急性反应。急性血管反应性反应者从 CCB 中获益可能比从 PAH 特异性治疗中获益更多。