Predictors of the response to phosphodiesterase-5 inhibitors in pulmonary arterial hypertension: an analysis of the Spanish registry.
Predictors of the response to phosphodiesterase-5 inhibitors in pulmonary arterial hypertension: an analysis of the Spanish registry.
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DOI：
10.1186/s12931-023-02531-1
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发表时间：
2023-09-15
影响因子：
5.8
通讯作者：
中科院分区：
文献类型：
--
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实现并维持低风险特征与肺动脉高压（PAH）的良好结局相关。治疗对风险特征的影响因患者而异。确定可能预测PAH患者对磷酸二酯酶-5抑制剂（PDE-5i）治疗反应的变量。我们对西班牙PAH登记研究中830例确诊为PAH并开始PDE 5i治疗且随访> 1年的患者进行了队列分析。644例患者开始PDE-5i单药治疗或添加治疗，186例患者开始PDE-5i和内皮素受体拮抗剂（ERA）联合治疗。在1年时，当应答者：（1）存活;（2）未出现临床恶化;（3）欧洲心脏病学会/欧洲呼吸学会（ESC/ERS）风险评分改善或保持低风险时，则视为应答者。使用单变量和多变量logistic回归模型分析与有利反应相关的变量。210例（33%）开始PDE-5i单药治疗的患者被归类为应答者，无论是单药治疗还是添加治疗。除了PAH结局的已知预测因素（基线时低风险，年龄较小）外，男性和门脉高压（PoPH）或HIV-PAH的诊断是PDE-5i良好应答的独立预测因素。一氧化碳弥散量（DLco）≤预测值的40%与不良反应相关。当PDE-5i用于前期组合时，58%的患者是应答者。在该组中，特发性PAH（IPAH）的诊断是有利缓解的独立预测因素，而结缔组织病-PAH与不利缓解相关。男性和PoPH或HIV-PAH的诊断是PDE-5i作为单药治疗或添加治疗对风险特征有利的预测因素。IPAH患者在前期联合使用PDE-5i时反应更好。这些结果确定了可能对PDE-5i单药治疗产生有利反应的患者特征，以及可能从替代治疗策略中获益的患者特征。在线版本包含补充材料，可通过10.1186/s12931-023-02531-1获得。
Achieving and maintaining a low-risk profile is associated with favorable outcome in pulmonary arterial hypertension (PAH). The effects of treatment on risk profile are variable among patients. To Identify variables that might predict the response to treatment with phosphodiesterase-5 inhibitors (PDE-5i) in PAH. We carried out a cohort analysis of the Spanish PAH registry in 830 patients diagnosed with PAH that started PDE5i treatment and had > 1 year follow-up. 644 patients started PDE-5i either in mono- or add-on therapy and 186 started combined treatment with PDE-5i and endothelin receptor antagonist (ERA). Responders were considered when at 1 year they: (1) were alive; (2) did not present clinical worsening; and (3) improved European Society of Cardiology/European Respiratory Society (ESC/ERS) risk score or remained in low-risk. Univariate and multivariate logistic regression models were used to analyze variables associated with a favorable response. Two hundred and ten patients (33%) starting PDE-5i alone were classified as responders, irrespective of whether it was mono- or add-on therapy. In addition to known predictors of PAH outcome (low-risk at baseline, younger age), male sex and diagnosis of portopulmonary hypertension (PoPH) or HIV-PAH were independent predictors of favorable response to PDE-5i. Diffusing capacity for carbon monoxide (DLco) ≤ 40% of predicted was associated with an unfavorable response. When PDE-5i were used in upfront combination, 58% of patients were responders. In this group, diagnosis of idiopathic PAH (IPAH) was an independent predictor of favorable response, whereas connective tissue disease-PAH was associated with an unfavorable response. Male sex and diagnosis of PoPH or HIV-PAH are predictors of favorable effect of PDE-5i on risk profile when used as mono- or add-on therapy. Patients with IPAH respond more favorably to PDE-5i when used in upfront combination. These results identify patient profiles that may respond favorably to PDE-5i in monotherapy and those who might benefit from alternative treatment strategies. The online version contains supplementary material available at 10.1186/s12931-023-02531-1.
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影响因子：
8.9
作者：
Hoeper, Marius M.;Pausch, Christine;Held, Matthias
通讯作者：
Held, Matthias
影响因子：
4.6
作者：
Gough, Michael S.;White, R. James
通讯作者：
White, R. James
影响因子：
24.3
作者：
Boucly, Athenais;Weatherald, Jason;Sitbon, Olivier
通讯作者：
Sitbon, Olivier
DOI：
10.1183/13993003.02024-2021
发表时间：
2022-06
期刊：
The European respiratory journal
影响因子：
--
作者：
通讯作者：
--
DOI：
10.1164/rccm.202004-1006oc
发表时间：
2021-02-15
影响因子：
24.7
作者：
Badagliacca, Roberto;D'Alto, Michele;Vizza, Carmine Dario
通讯作者：
Vizza, Carmine Dario