Comprehensive Pulmonary Hypertension Center, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA.
Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Anschutz Medical Campus, Denver, Colorado, USA.
Heart. 2023 Jun 26;109(14):1098-1105. doi: 10.1136/heartjnl-2022-321760.
Pulmonary artery compliance (PAC), estimated as stroke volume (SV) divided by pulmonary artery pulse pressure (PP), may be a predictor of survival in pulmonary arterial hypertension (PAH). Resistance-compliance (RC) time, the product of PAC and pulmonary vascular resistance, is reported to be a physiological constant. We investigated if differences in PAC and RC time exist between pulmonary hypertension (PH) subgroups and examined whether PAC is an independent predictor of transplant-free survival in PAH.
This was a retrospective analysis of adult PAH (n=532) and chronic thromboembolic PH (CTEPH, n=84) patients enrolled in the US Pulmonary Hypertension Association Registry from 2015 to 2019. PAC and RC time were compared between PH subgroups (connective tissue disease-PAH (CTD-PAH), idiopathic/heritable-PAH (i/h-PAH), drug/toxin-PAH (d/t-PAH)). Cox proportional hazards models were constructed for transplant-free survival, adjusting for REVEAL 2.0 risk score.
There were no differences in estimated PAC between PAH subgroups, nor between PAH and CTEPH. RC time was shorter in CTEPH compared with PAH (median 0.55 (IQR 0.45-0.64) vs 0.62 (0.52-0.73) s, p<0.0001). RC time was shortest in CTD-PAH when compared with i/h-PAH and d/t-PAH ((0.59±0.18) vs (0.65±0.20) vs (0.73±0.25) s, p=0.0001). PAC was associated with transplant-free survival (HR 0.72, 95% CI 0.55 to 0.94, p=0.02) but was not an independent predictor of outcome after adjustment for REVEAL 2.0 score.
PAC was similar between PH groups and was not an independent predictor of transplant-free survival in PAH. RC time was different between PH subgroups, challenging RC time constancy.
NCT04071327.
肺动脉顺应性（PAC）可通过每搏量（SV）除以肺动脉脉搏压（PP）来估计，可能是肺动脉高压（PAH）患者生存的预测指标。阻力顺应性（RC）时间是 PAC 与肺血管阻力的乘积，据报道为生理常数。我们研究了 PH 亚组之间是否存在 PAC 和 RC 时间的差异，并检查了 PAC 是否是 PAH 无移植生存的独立预测因素。
这是对 2015 年至 2019 年期间在美国肺动脉高压协会登记处登记的成年 PAH（n=532）和慢性血栓栓塞性 PH（CTEPH，n=84）患者进行的回顾性分析。比较了 PH 亚组（结缔组织疾病相关-PAH（CTD-PAH）、特发性/遗传性-PAH（i/h-PAH）、药物/毒素相关-PAH（d/t-PAH）之间的 PAC 和 RC 时间。构建了无移植生存的 Cox 比例风险模型，调整了 REVEAL 2.0 风险评分。
PAH 亚组之间以及 PAH 与 CTEPH 之间的估计 PAC 无差异。与 PAH 相比，RC 时间在 CTEPH 中较短（中位数 0.55（IQR 0.45-0.64）与 0.62（0.52-0.73）s，p<0.0001）。与 i/h-PAH 和 d/t-PAH 相比，CTD-PAH 时 RC 时间最短（（0.59±0.18）与（0.65±0.20）与（0.73±0.25）s，p=0.0001）。PAC 与无移植生存相关（HR 0.72，95%CI 0.55 至 0.94，p=0.02），但在调整 REVEAL 2.0 评分后，PAC 不是无移植生存的独立预测因素。
PAH 组之间的 PAC 相似，并且不是 PAH 无移植生存的独立预测因素。RC 时间在 PH 亚组之间不同，这对 RC 时间的恒定性提出了挑战。
NCT04071327。
