Prognostic biomarkers and clinical parameters in adults with atrial septal defect-related pulmonary arterial hypertension treated with sildenafil and beraprost combination therapy

Background

Altered biological and environmental factors have been linked to the development of pulmonary vascular remodeling in pulmonary arterial hypertension (PAH) pathogenesis.

Objectives

We determined the association between prognostic biomarkers and clinical parameters of pulmonary arterial hypertension (PAH) in adult Atrial Septal Defect (ASD) patients with right heart failure who were treated with sildenafil and beraprost combination therapy.

Methods

We conducted an observational study using a cross-sectional design and examined the correlation between prognostic biomarkers (superoxide dismutase (SOD) and Platelet-derived growth factor-BB (PDGF-BB)) and clinical parameters (6 minute walking distance (6MWD) and echocardiography parameters (tricuspid annular plane systolic excursion/pulmonary artery systolic pressure (TAPSE/PASP)) in 41 adult patients with ASD diagnosed as pre-capillary pulmonary hypertension (WHO group 1) who were treated at the congenital outpatient clinic at Dr. Soetomo General Hospital Surabaya, Indonesia from October 1st to November 30th 2022. A Pearson correlation test was used to determine the correlation between normal distribution variables. Statistical analyses were done using SPSS software.

Results

The results indicated a moderately negative relationship between PDGF-BB and 6MWD (Pearson, r = −0.320, p = 0.041) and a medium significantly negative relationship between PDGF-BB and TAPSE/PASP (Pearson, r = −0.347, p = 0.026).

Conclusions

Lower circulating PDGF-BB is associated with better functional capacity and echocardiographic parameters in adult ASD-PAH patients receiving sildenafil and beraprost combination therapy. PDGF-BB may strongly be used as a treatment progress monitoring and prognostic biomarker.