Clinical outcomes of upfront combination therapy for portopulmonary hypertension

IF 1.9 Q3 PERIPHERAL VASCULAR DISEASE
Takatoyo Kiko , Ryotaro Asano , Hiroyuki Endo , Naruhiro Nishi , Hiroya Hayashi , Jin Ueda , Tatsuo Aoki , Akihiro Tsuji , Takeshi Ogo
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引用次数: 0

Abstract

Background

Limited data exists on upfront combination therapy for portopulmonary hypertension. We evaluated the clinical efficacy, long-term outcomes, and safety of upfront combination therapy in patients with portopulmonary hypertension.

Methods

We performed a retrospective, single-center cohort study involving a final analysis of 33 consecutive patients diagnosed with portopulmonary hypertension who were taking pulmonary arterial hypertension-specific medication. We compared hemodynamic parameters, risk profiles, composite clinical worsening events, and safety between monotherapy (n = 23) and upfront combination therapy (n = 10).

Results

Twenty-seven patients (82 %) were classified into the Child–Pugh A stage. The change ratios of pulmonary vascular resistance (−32 % vs. −57 %, P = 0.006) were significantly better with upfront combination therapy. Upfront combination therapy also showed significant improvement in risk profiles. Kaplan–Meier analysis showed that the composite event-free rate was significantly lower in patients who received upfront combination therapy than in those who received monotherapy (P = 0.016), although no statistical differences were observed in all-cause death. In the univariate Cox proportional hazards analysis, upfront combination therapy was a factor for decreasing composite clinical worsening outcomes (hazard ratio 0.190, 95 % confidence interval 0.042–0.854; P = 0.030). No significant hepatic impairments were observed over 2 years of follow-up in the upfront combination group.

Conclusions

In patients with portopulmonary hypertension, upfront combination therapy significantly improved symptoms and short-term hemodynamics, and reduced long-term clinical worsening events without serious adverse effects. This study's findings suggest that patients with portopulmonary hypertension presenting with mild hepatic impairment benefit from upfront combination therapy.

门肺动脉高压前期综合疗法的临床疗效
背景有关门肺动脉高压前期联合治疗的数据有限。我们评估了门静脉高压患者前期联合治疗的临床疗效、长期预后和安全性。方法 我们进行了一项回顾性单中心队列研究,最终分析了 33 名连续确诊患有门静脉高压且正在服用肺动脉高压特异性药物的患者。我们比较了单药治疗(23 例)和前期联合治疗(10 例)的血流动力学参数、风险概况、综合临床恶化事件和安全性。肺血管阻力的变化比率(-32 % 对 -57 %,P = 0.006)明显优于前期联合疗法。前期联合疗法也明显改善了风险状况。卡普兰-梅耶尔分析显示,接受前期联合疗法的患者的复合无事件发生率明显低于接受单一疗法的患者(P = 0.016),但在全因死亡方面未观察到统计学差异。在单变量考克斯比例危险分析中,前期联合疗法是降低综合临床恶化结果的一个因素(危险比为0.190,95%置信区间为0.042-0.854;P = 0.030)。结论 在门静脉高压症患者中,前期联合治疗可显著改善症状和短期血流动力学,并减少长期临床恶化事件,且无严重不良反应。本研究结果表明,伴有轻度肝功能损害的门静脉高压症患者可从前期联合治疗中获益。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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