Background

Multiparametric risk assessment is used in pulmonary arterial hypertension (PAH) to target therapy. However, this strategy is imperfect because most patients remain at intermediate or high risk after initial treatment, with low risk being the goal. Metrics of right ventricular (RV) adaptation are promising tools that may help refine our therapeutic strategy.

Research Question

Does RV adaptation predict therapeutic response over time?

Study Design and Methods

We evaluated 52 incident treatment-naive patients with advanced PAH by catheterization and cardiac imaging longitudinally at baseline, follow-up 1 (∼3 months), and follow-up 2 (∼18 months). All patients received goal-directed therapy with parenteral treprostinil and/or combination therapy with treatment escalation if functional class I or II was not achieved. On the basis of their therapeutic response, patients were evaluated at follow-up 1 as nonresponders (died) or as responders, and again at follow-up 2 as super-responders (low risk) or partial responders (high/intermediate risk). Multiparametric risk was based on a simplified European Respiratory Society/European Society of Cardiology guideline score. RV adaptation was evaluated with the single-beat coupling ratio (Ees/Ea) and diastolic function with diastolic elastance (Eed). Data are expressed as mean ± SD or as OR (95% CI).

Results

Nine patients (17%) were nonresponders. PAH-directed therapy improved the European Respiratory Society low-risk score from 1 (2%) at baseline to 23 (55%) at follow-up 2. Ees/Ea at presentation was nonsignificantly higher in responders (0.9 ± 0.4) vs nonresponders (0.6 ± 0.4; P = .09) but could not be used to predict super-responder status at follow-up 2 (OR, 1.40 [95% CI, 0.28-7.0]; P = .84). Baseline RV ejection fraction and change in Eed were successfully used to predict super-responder status at follow-up 2 (OR, 1.15 [95% CI, 1.0-1.27]; P = .009 and OR, 0.29 [95% CI, 0.86-0.96]; P = .04, respectively).

Interpretation

In patients with advanced PAH, RV-pulmonary arterial coupling could not discriminate irreversible RV failure (nonresponders) at presentation but showed a late trend to improvement by follow-up 2. Early change in Eed and baseline RV ejection fraction were the best predictors of therapeutic response.

中文翻译：

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右心室-肺动脉耦合和舒张功能对肺动脉高压治疗的反应

背景

多参数风险评估用于肺动脉高压 (PAH) 以进行靶向治疗。然而，这种策略并不完美，因为大多数患者在初始治疗后仍处于中等或高风险，目标是低风险。右心室 (RV) 适应指标是很有前途的工具，可以帮助改进我们的治疗策略。

研究问题

RV 适应是否可以预测随时间推移的治疗反应？

研究设计和方法

我们在基线、随访 1（~3 个月）和随访 2（~18 个月）时通过导管插入术和纵向心脏成像评估了 52 例未接受过治疗的晚期 PAH 患者。如果未达到 I 级或 II 级功能，所有患者都接受了曲前列尼肠外注射的目标导向治疗和/或治疗升级的联合治疗。根据他们的治疗反应，患者在随访 1 中被评估为无反应者（死亡）或反应者，并在随访 2 中再次被评估为超级反应者（低风险）或部分反应者（高/中风险）。多参数风险基于简化的欧洲呼吸学会/欧洲心脏病学会指南评分。RV 适应性通过单搏耦合比 (Ees/Ea) 和舒张功能与舒张弹性 (Eed) 进行评估。

结果

9 名患者 (17%) 无反应。针对 PAH 的治疗将欧洲呼吸学会的低风险评分从基线时的 1 (2%) 提高到随访时的 23 (55%) 2。就诊时的 Ees/Ea 在反应者 (0.9 ± 0.4) 与无反应者中没有显着升高(0.6 ± 0.4; P  = .09) 但不能用于预测随访 2 中的超级反应者状态（OR，1.40 [95% CI，0.28-7.0]；P  = .84）。基线 RV 射血分数和 Eed 的变化被成功地用于预测随访 2 的超级反应状态（OR，1.15 [95% CI，1.0-1.27]；P = .009 和 OR，0.29 [95% CI ，  0.86 -0.96]；P  = .04）。

解释

在患有晚期 PAH 的患者中，RV-肺动脉耦合在就诊时无法区分不可逆的 RV 衰竭（无反应者），但在随访 2 中显示出改善的晚期趋势。Eed 和基线 RV 射血分数的早期变化是治疗效果的最佳预测因子回复。