Heart:右心室重构在肺动脉高压中的重要性

2022-05-25 网络 网络

最近的研究表明 PAH 进展的特征是 RV 尺寸和功能的变化(体积增加和射血分数降低),即使在明显稳定的患者中也是如此,这突出了RV在确定预后中的重要性。

肺动脉高压 (PAH) 患者症状和结果的主要决定因素是右心室 (RV) 功能及其与肺循环的耦合。为了保持足够的心输出量,RV 通过增加收缩力(恒速适应)来适应增加的后负荷,并且当这种机制耗尽时,通过增加容量(异速适应)来适应增加的后负荷。

最近的研究表明 PAH 进展的特征是 RV 大小和功能的变化(体积增加和射血分数降低),即使在明显稳定的患者中也是如此,这突出了 RV 在确定预后中的重要性。Goh 及其同事的研究强调了 RV 重塑在 PAH 中的相关性。作者分析了来自 ASPIRE(评估在转诊中心确定的肺动脉高压谱)登记处的 505 名患者的大型队列。心脏磁共振允许根据其体积和质量识别四种不同的 RV 适应集群。具有良好适应性重塑(低体积和低质量)的患者预后最好。有趣的是,这些患者的心脏指数、混合静脉血氧饱和度、右室射血分数和右室-肺动脉耦合最高,平均肺动脉压和肺血管阻力 (PVR) 最低,右心房面积最小。所有这些预后指标都与更好的 RV 功能相关。相反,具有适应不良重塑(高容量-低质量)的患者预后最差。该研究具有重要的临床意义。

RV 反向重塑与良好的长期生存率和生活质量相关,但受不同治疗策略的影响。例如,仅少数接受单一疗法或逐步添加初始单一疗法的患者可实现 RV 反向重塑。来自意大利网络 IPHNET的最新数据表明,PVR 的显着降低 (>50%) 能够确定 RV 的反向重塑和每搏输出量指数的正常化。

尽管所有治疗都可以改善血流动力学(心脏指数增加和 PVR 降低),但只有更积极的策略,例如双口服或三联药物(包括肠外前列腺素)才能实现关键的 PVR 降低,增加反向 RV 重塑的可能性。

越来越多的证据强调了包括肠外前列腺素在内的三重前期联合治疗对患有晚期疾病的特发性 PAH 患者的安全性和有效性。这些研究表明,PVR 比基线空前降低了约 2/3,风险显着降低,并且 RV 反向重塑(体积减少和射血分数增加)。

综合所有这些信息,我们可以得出结论,基线时有利的 RV 重塑或后续期间的 RV 反向重塑预计将预测 PAH 的更好结果。因此,不应推迟对更多晚期 PAH 患者采取更积极的策略,包括肠外前列腺素。

图 1显示了一种基于风险状况和 RV 重塑的不同表型整合的可能治疗方法。虚线箭头表示治疗处于中等风险和右心室至肺动脉的患者的一种可能选择,其中包括肠外前列腺素在内的三重前期治疗。

如今,有两个问题需要回答。首先,尽管当前的风险评分模型间接受到 RV 适应增加后负荷的能力的影响,但它们都没有包括 RV 大小和功能的非侵入性测量。为什么?从理论上讲,磁共振和超声心动图是理想的工具:它们是非侵入性的,可以提供详细的信息并且可以很容易地重复。一方面,磁共振的成本和可用性,另一方面,超声心动图的可重复性削弱了它们在风险分层中的应用。稳健的多中心研究应填补这一空白,使影像学在不久的将来在风险分层中发挥更重要的作用。

其次,患者对类似策略(即前期双口服或前期三联疗法,包括肠外前列腺素)的治疗反应已被证明是异质的。是否取决于不同的化合物或时间?种族重要吗?是否存在影响个体反应的遗传背景?对于这样一种毁灭性疾病,精准医学和量身定制的疗法将是可取的。出于这个原因,进一步调查 PAH 领域的共同努力是强制性的。

 

文献参考:

D'Alto M, Badagliacca R. The importance of right ventricular remodelling in pulmonary arterial hypertension. Heart. 2022 May 23:heartjnl-2022-321143. doi: 10.1136/heartjnl-2022-321143. Epub ahead of print. PMID: 35606110.

 

 

 

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    2022-08-01 mgqwxj
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    2022-06-02 SophylioJerry

    欢迎关注肺动脉高压研究进展

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    2022-05-26 zhaojie88
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    2022-05-26 slcumt

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