Cardiology Department, School of Medicine, Ondokuz Mayıs University, 25270, Atakum, Samsun, Turkey.
Cardiology Department, School of Medicine, Cumhuriyet University, Sivas, Turkey.
Int J Cardiovasc Imaging. 2022 Oct;38(10):2191-2197. doi: 10.1007/s10554-022-02625-9. Epub 2022 May 28.
Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple clinical conditions and complicate most systemic diseases. Systemic sclerosis (SSc), represents the leading cause of connective tissue disease (CTD) associated with PAH. Although SSc is a rare disease, it is associated with higher morbidity and early mortality than other rheumatological diseases due to developing SSc-associated interstitial pulmonary disease (ILD) and/or pulmonary arterial hypertension (PAH). The impact of the early diagnosis on the prognosis is evident. In this context, in our study, we aimed to investigate the early changes in pulmonary vascular bed by measuring pulmonary arterial stiffness (PAS) in SSc patients without overt PAH. Sixty-two SSc patients and fifty-eight gender and age-matched, healthy subjects enrolled in this cross-sectional observational study. SSc patients were evaluated in terms of disease duration and severity. Modified rodnan skin score (mRSS) was calculated as disease severity index. Echocardiographic parameters were assessed and compared to the control group. Right ventricular (RV) diameters, systolic pulmonary artery pressure (sPAP), and right ventricle myocardial performance index (RV-MPI) were significantly higher in the SSc group compared to the control group (p < 0.05). Tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) were significantly lower in the SSc group compared to the control group (p < 0.05). PAS value (25.5 ± 9.2 kHz/ms vs. 18.1 ± 7.4 kHz/ms, p < 0.001) was significantly higher in the SSc group than in the control group. A statistically significant positive correlation relationship was detected between the PAS value and CRP, ESR, disease duration, mRSS. According to these results, in SSc patients, PAS as an inexpensive and easily applicable echocardiographic method might serve as a marker of early detection of PAH.
肺动脉高压（PH）是一种病理生理紊乱，可能涉及多种临床情况，并使大多数系统性疾病复杂化。系统性硬化症（SSc）是与肺动脉高压（PAH）相关的结缔组织疾病（CTD）的主要原因。尽管 SSc 是一种罕见疾病，但由于发生 SSc 相关的间质性肺疾病（ILD）和/或肺动脉高压（PAH），其发病率和早期死亡率高于其他风湿病。早期诊断对预后的影响是显而易见的。在这种情况下，我们在本研究中旨在通过测量无明显 PAH 的 SSc 患者的肺血管床早期变化来研究这一问题。我们招募了 62 名 SSc 患者和 58 名性别和年龄匹配的健康对照者进行这项横断面观察性研究。我们评估了 SSc 患者的疾病持续时间和严重程度。改良罗德纳皮肤评分（mRSS）被计算为疾病严重程度指数。评估并比较了超声心动图参数与对照组。与对照组相比，SSc 组的右心室（RV）直径、收缩期肺动脉压（sPAP）和右心室心肌做功指数（RV-MPI）显著升高（p<0.05）。与对照组相比，SSc 组的三尖瓣环平面收缩期位移（TAPSE）和右心室射血分数（RVFAC）显著降低（p<0.05）。与对照组相比，SSc 组的 PAS 值（25.5±9.2 kHz/ms 与 18.1±7.4 kHz/ms，p<0.001）显著升高。PAS 值与 CRP、ESR、疾病持续时间、mRSS 呈显著正相关。根据这些结果，在 SSc 患者中，PAS 作为一种廉价且易于应用的超声心动图方法，可能作为早期检测 PAH 的标志物。
