Mukherjee Monica, Mathai Stephen C, Jellis Christine, Freed Benjamin H, Yanek Lisa R, Agoglia Hannah, Chiu Caitlin, Jani Vivek P, Simpson Catherine E, Brittain Evan L, Tang W H Wilson, Park Margaret M, Hemnes Anna R, Rosenzweig Erika B, Rischard Franz P, Frantz Robert P, Hassoun Paul M, Beck Gerald, Hill Nicholas S, Erzurum Serpil, Thomas James D, Kwon Deborah, Leopold Jane A, Horn Evelyn M, Kim Jiwon
Johns Hopkins University School of Medicine, Baltimore, MD.
Cleveland Clinic Medical Center, Cleveland, OH.
Circ Cardiovasc Imaging. 2024 Oct;17(10). doi: 10.1161/circimaging.124.017074. Epub 2024 Oct 15.
Defining qualitative grades of echocardiographic metrics of right heart chamber size and function is critical for screening, clinical assessment, and measurement of therapeutic response in individuals with pulmonary vascular disease (PVD). In a population enriched for PVD, we sought to establish qualitative grades and prognostic value of right heart chamber size and function.
We investigated 1053 study participants in the Redefining Pulmonary Hypertension through PVD Phenomics program (PVDOMICS) to determine clinical and echocardiographic differences associated with increasing pulmonary vascular resistance (PVR) severity. Right heart chamber size and function were qualitatively assessed using a percentile-based approach above the median values to create a clinical grading system for right heart adaptation. The relationship between echocardiographic categories and all-cause mortality was examined using survival analyses adjusted for potential confounders.
A stepwise increase in adverse right heart remodeling was observed with a concomitant decrease in functional parameters by PVR strata (p<0.001 for all). Mild, moderate, and severe categories of right heart chamber size and dysfunction were defined using a percentile-based approach across the spectrum of PVD. During a median follow up of 2.07 years (interquartile range 1.23 - 3.01 years), 130 participants died (11.4%). Progressive PVR increase and 2DE evidence of right heart dysfunction inclusive of fractional area change, and right ventricular (RV) global longitudinal strain were independently associated with increased all-cause mortality risk in multivariate analysis adjusted for age, disease duration and male sex.
In this well-characterized sample of adults with diverse etiologies and varying PVD severity, we define categories of abnormal right heart chamber size and function. Further, we demonstrate a stepwise relationship between these categories of abnormal morphology and function and all-cause mortality. Defining grades of RV dysfunction in individuals with known PVD has important clinical implications for monitoring disease progression and response to therapies.
确定右心腔大小和功能的超声心动图指标的定性分级对于筛查、临床评估以及测量肺血管疾病（PVD）患者的治疗反应至关重要。在一个PVD患者富集的人群中，我们试图建立右心腔大小和功能的定性分级及预后价值。
我们在通过PVD表型组学重新定义肺动脉高压项目（PVDOMICS）中调查了1053名研究参与者，以确定与肺血管阻力（PVR）严重程度增加相关的临床和超声心动图差异。使用基于百分位数的方法对右心腔大小和功能进行定性评估，该方法基于中位数以上的值，以创建右心适应的临床分级系统。使用针对潜在混杂因素进行调整的生存分析来检查超声心动图类别与全因死亡率之间的关系。
随着PVR分层，观察到不良右心重塑逐步增加，同时功能参数下降（所有p<0.001）。通过基于百分位数的方法在整个PVD范围内定义了右心腔大小和功能障碍的轻度、中度和重度类别。在中位随访2.07年（四分位间距1.23 - 3.01年）期间，130名参与者死亡（11.4%）。在对年龄、疾病持续时间和男性性别进行调整的多变量分析中，PVR逐渐增加以及二维超声心动图显示的右心功能障碍证据（包括面积变化分数和右心室整体纵向应变）与全因死亡风险增加独立相关。
在这个病因多样且PVD严重程度各异的特征明确的成人样本中，我们定义了右心腔大小和功能异常的类别。此外，我们证明了这些异常形态和功能类别与全因死亡率之间的逐步关系。在已知PVD的个体中定义右心室功能障碍的分级对于监测疾病进展和治疗反应具有重要的临床意义。
