D'Andrea Antonello, Stanziola Anna, Di Palma Enza, Martino Maria, D'Alto Michele, Dellegrottaglie Santo, Cocchia Rosangela, Riegler Lucia, Betancourt Cordido Meredyth Vanessa, Lanza Maurizia, Maglione Marco, Diana Veronica, Calabrò Raffaele, Russo Maria Giovanna, Vannan Mani, Bossone Eduardo
Department of Cardiology, Second University of Naples, Monaldi Hospital, "dei Colli - Monaldi" Hospitals, Naples, Italy.
Division of Pneumology, Federico II University, Monaldi Hospital, "dei Colli - Monaldi" Hospitals, Naples, Italy.
Echocardiography. 2016 Jan;33(1):57-65. doi: 10.1111/echo.12992. Epub 2015 Jun 11.
To elucidate right ventricular (RV) function in patients with idiopathic pulmonary fibrosis (IPF) with and without pulmonary hypertension (PH) and its relation to other features of the disease.
Clinical evaluation, standard Doppler echo, Doppler myocardial imaging (DMI), and 2D strain echocardiography (STE) of RV septal and lateral walls were performed in 52 IPF patients (66.5 ± 8.5 years; 27 males) and in 45 age- and sex-comparable controls using a commercial US system (MyLab Alpha, Esaote). Pulmonary artery mean pressure (mPAP) was estimated by standard echo Doppler. RV global longitudinal strain (RV GLS) was calculated by averaging RV local strains. The IPF patients were divided into 2 groups by noninvasive assessment of PH: no PH (mPAP<25 mmHg; 36 pts) and PH (mPAP ≥25 mmHg; 16 pts). Left ventricular diameters and ejection fraction were comparable between controls and IPF, while GLS was impaired in IPF (P < 0.01). RV end-diastolic diameters, wall thickness andmPAP were increased in IPF patients with PH. In addition, pulsed DMI detected in PH IPF impaired myocardial RV early diastolic (Em) peak velocity. Also peak systolic RV strain was reduced in basal and middle RV lateral free walls in IPF, as well as RV GLS (P < 0.0001). The impairment in RV wall strain was more evident when comparing controls with the no PH group than comparing the no PH group with the PH group. By multivariate analysis, independent association of RV strain with both six-minute walking test distance (P < 0.001), mPAP (P < 0.0001), as well as with forced vital capacity (FVC) % (P < 0.005) in IPF patients were observed.
Impaired RV diastolic and systolic myocardial function were present even in IPF patients without PH, which indicates an early impact on RV function and structure in patients with IPF.
阐明有无肺动脉高压（PH）的特发性肺纤维化（IPF）患者的右心室（RV）功能及其与该疾病其他特征的关系。
使用商用超声系统（MyLab Alpha，百胜），对52例IPF患者（66.5±8.5岁；27例男性）和45例年龄及性别匹配的对照者进行了临床评估、标准多普勒超声、多普勒心肌成像（DMI）以及RV间隔和侧壁的二维应变超声心动图（STE）检查。通过标准回声多普勒估计肺动脉平均压（mPAP）。通过平均RV局部应变计算RV整体纵向应变（RV GLS）。根据PH的无创评估将IPF患者分为两组：无PH（mPAP<25 mmHg；36例）和PH（mPAP≥25 mmHg；16例）。对照组和IPF患者的左心室直径和射血分数相当，而IPF患者的GLS受损（P<0.01）。有PH的IPF患者的RV舒张末期直径、壁厚和mPAP增加。此外，脉冲DMI检测到PH-IPF患者的RV心肌舒张早期（Em）峰值速度受损。IPF患者RV外侧游离壁基底段和中间段的收缩期峰值应变以及RV GLS也降低（P<0.0001）。与对照组和无PH组比较时，RV壁应变的损害比无PH组和PH组之间的比较更明显。多因素分析显示，IPF患者的RV应变与六分钟步行试验距离（P<0.001）、mPAP（P<0.0001）以及用力肺活量（FVC）%（P<0.005）均独立相关。
即使在无PH的IPF患者中也存在RV舒张期和收缩期心肌功能受损，这表明IPF患者的RV功能和结构受到早期影响。
