Department of Clinical Sciences Lund, Clinical Physiology, Lund University, Lund, Sweden.
Department of Clinical Physiology and Nuclear Imaging, Skåne University Hospital, Getingevägen 5, SE-22185, Lund, Sweden.
ESC Heart Fail. 2018 Oct;5(5):864-875. doi: 10.1002/ehf2.12304. Epub 2018 Jun 19.
Pre-capillary pulmonary hypertension (PH ) has a poor prognosis, especially when caused by pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). Whether cardiac magnetic resonance (CMR)-based quantification of atrial volumes in PH is beneficial in risk assessment is unknown. The aims were to investigate if (i) atrial volumes using CMR are associated with death or lung transplantation in PH , (ii) atrial volumes differ among four unmatched major PH subgroups, and (iii) atrial volumes differ between SSc-PAH and idiopathic/familial PAH (IPAH/FPAH) when matched for pulmonary vascular resistance (PVR).
Seventy-five PH patients (57 ± 19 years, 53 female, 43 de novo) with CMR and right heart catheterization were retrospectively included. Short-axis stacks of cine images were analysed, and right and left atrial maximum (RAV and LAV ) and minimum volume (RAV and LAV ) were indexed for body surface area. Increased (mean + 2 SD) and reduced (mean - 2 SD) volumes were predefined from CMR normal values. Transplantation-free survival was lower in patients with increased RAV than in those with normal [hazard ratio (HR) = 2.1, 95% confidence interval (CI) 1.1-4.0] but did not differ between those with reduced LAV and normal (HR 2.0, 95% CI 0.8-5.1). RAV and RAV showed no differences among unmatched or matched groups (P = ns). When matched for PVR, LAV , LAV , and pulmonary artery wedge pressure were reduced in SSc-PAH compared with IPAH/FPAH (95% CI 0.3-21.4, 95% CI 0.8-19.6, and 95% CI 2-7, respectively).
Patients with PH and increased right atrial volume measured with CMR had worse clinical outcome. When matched for PVR, left atrial volume was lower in SSc-PAH than in IPAH/FPAH, consistent with left-sided underfilling, indicating a potential differentiator between the groups.
毛细血管前肺动脉高压（PH ）预后较差，尤其是由与系统性硬化症（SSc-PAH）相关的肺动脉高压（PAH）引起时。尚不清楚基于心脏磁共振（CMR）的心房容积定量评估在 PH 中的风险评估是否有益。本研究旨在探讨：（i）CMR 测量的心房容积与 PH 患者的死亡或肺移植是否相关；（ii）在四个不匹配的主要 PH 亚组中，心房容积是否存在差异；（iii）当匹配肺血管阻力（PVR）时，SSc-PAH 与特发性/家族性 PAH（IPAH/FPAH）之间的心房容积是否存在差异。
回顾性纳入 75 例接受 CMR 和右心导管检查的 PH 患者（57±19 岁，53 例女性，43 例初诊）。分析电影图像的短轴堆栈，用体表面积对右心房最大容积（RAV 和 LAV ）和最小容积（RAV 和 LAV ）进行指数化。从 CMR 正常值中预先设定增大（平均值+2 个标准差）和减小（平均值-2 个标准差）的容积。与正常组相比，RAV 增大患者的无移植生存率较低（危险比 [HR] 2.1，95%置信区间 [CI] 1.1-4.0），而 RAV 减小患者与正常组之间无差异（HR 2.0，95% CI 0.8-5.1）。不匹配或匹配组之间 RAV 和 RAV 无差异（P=ns）。当匹配 PVR 时，SSc-PAH 患者的 LAV 、LAV 和肺动脉楔压较 IPAH/FPAH 患者降低（95% CI 0.3-21.4、95% CI 0.8-19.6 和 95% CI 2-7）。
CMR 测量的 PH 患者右心房容积增大与临床结局较差相关。当匹配 PVR 时，SSc-PAH 患者的 LAV 小于 IPAH/FPAH，这与左心房充盈不足一致，表明两组之间存在潜在的差异。
