Echocardiographic characterization of combined postcapillary and precapillary pulmonary hypertension in systemic sclerosis with preserved ejection fraction
Abstract
Systemic sclerosis (SSc) is a multisystem autoimmune disease frequently complicated by pre- and post-capillary pulmonary hypertension (PH). Within SSc, progressive diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are key contributors, often presenting as isolated postcapillary PH (Ipc-PH) or combined pre- and postcapillary PH (Cpc-PH). The ability to differentiate these hemodynamic phenotypes is critical for risk stratification, yet echocardiographic markers specific to each subtype in SSc-HFpEF are poorly defined. We investigated 147 adults with SSc-HFpEF with echocardiograms and right heart catheterization (RHC) assessments performed within one year. Patients were classified as Ipc-PH (n = 46) or Cpc-PH (n = 101) based on guideline-defined hemodynamic criteria. Echocardiographic parameters, including conventional measures, strain indices, and coupling metrics were analyzed. A random forest (RF) classifier was used to identify top echocardiographic predictors of Cpc-PH, and further assessed using multivariable logistic regression. Separate RF models and Cox regression analyses were used to determine echocardiographic predictors of mortality within each group. Survival was assessed using Kaplan-Meier analysis. Patients with Cpc-PH exhibited significantly greater right heart remodeling, higher pulmonary pressures, and impaired right ventricle (RV)-pulmonary artery (PA) coupling. The top echocardiographic predictors of Cpc-PH included reduced RV…
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