Kelemen Benjamin W, Mathai Stephen C, Tedford Ryan J, Damico Rachel L, Corona-Villalobos Cecilia, Kolb Todd M, Chaisson Neal F, Harris Traci Housten, Zimmerman Stefan L, Kamel Ihab R, Kass David A, Hassoun Paul M
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA ; These authors contributed equally to this article.
Division of Cardiology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Pulm Circ. 2015 Jun;5(2):327-34. doi: 10.1086/680356.
Patients with scleroderma (SSc)-associated pulmonary arterial hypertension (PAH) have worse survival than patients with idiopathic PAH (IPAH). We hypothesized that the right ventricle (RV) adapts differently in SSc-PAH versus IPAH. We used cardiac magnetic resonance imaging (cMRI) and hemodynamic characteristics to assess the relationship between RV morphology and RV load in patients with SSc-PAH and IPAH. In 53 patients with PAH (35 with SSc-PAH and 18 with IPAH) diagnosed by right heart catheterization (RHC), we examined cMRIs obtained within 48 hours of RHC and compared RV morphology between groups. Regression analysis was used to assess the association between diagnosis (IPAH vs. SSc-PAH) and RV measurements after adjusting for age, sex, race, body mass index (BMI), left ventricular (LV) mass, and RV load. There were no significant differences in unadjusted comparisons of cMRI measurements between the two groups. Univariable regression showed RV mass index (RVMI) was linearly associated with measures of RV load in both the overall cohort and within each group. Multivariable linear regression models revealed a significant interaction between disease type and RVMI adjusting for pulmonary vascular resistance (PVR), age, sex, race, BMI, and LV mass. This model showed a decreased slope in the relationship between RVMI and PVR in the SSc-PAH group compared with the IPAH group. RVMI varies linearly with measures of RV load. After adjusting for multiple potential confounders, patients with SSc-PAH demonstrated significantly less RV hypertrophy with increasing PVR than patients with IPAH. This difference in adaptive hypertrophy may in part explain previously observed decreased contractility and poorer survival in SSc-PAH.
硬皮病（SSc）相关肺动脉高压（PAH）患者的生存率低于特发性PAH（IPAH）患者。我们假设右心室（RV）在SSc-PAH和IPAH中的适应方式不同。我们使用心脏磁共振成像（cMRI）和血流动力学特征来评估SSc-PAH和IPAH患者RV形态与RV负荷之间的关系。在53例经右心导管检查（RHC）诊断为PAH的患者（35例SSc-PAH患者和18例IPAH患者）中，我们检查了在RHC后48小时内获得的cMRI，并比较了两组之间的RV形态。在调整年龄、性别、种族、体重指数（BMI）、左心室（LV）质量和RV负荷后，使用回归分析来评估诊断（IPAH与SSc-PAH）与RV测量值之间的关联。两组之间cMRI测量值的未调整比较无显著差异。单变量回归显示，在整个队列和每组中，RV质量指数（RVMI）与RV负荷测量值呈线性相关。多变量线性回归模型显示，在调整肺血管阻力（PVR）、年龄、性别、种族、BMI和LV质量后，疾病类型与RVMI之间存在显著交互作用。该模型显示，与IPAH组相比，SSc-PAH组中RVMI与PVR之间关系的斜率降低。RVMI与RV负荷测量值呈线性变化。在调整多个潜在混杂因素后，与IPAH患者相比，SSc-PAH患者随着PVR增加，RV肥厚明显较少。适应性肥厚的这种差异可能部分解释了先前观察到的SSc-PAH患者收缩力下降和生存率较低的情况。
