Abstract
BACKGROUND: Research on the limits of compensatory right heart remodeling and the effects of pulmonary artery hypertension (PAH) targeted therapies on these mechanisms is limited. METHOD: Chest x-ray and echocardiographic data were collected from 143 deceased patients with PAH confirmed by right heart catheterization at their end-stage disease. Right heart remodeling was compared across different PAH treatment strategies. RESULTS: This study of 143 deceased PAH patients (49 ± 17 years, 74.1% female) characterized right heart remodeling at the time of death. Mean cardiothoracic ratio (CTR), right atrial area (RAA) and mid-cavity RV linear dimension (RVD) measured by echocardiography were 0.61 ± 0.09, 27 cm(2) (median 27, IQR 21-38), and 4.97 ± 0.97 cm, respectively, with extremes of 0.88, 102 cm(2), and 7.50 cm. Intensive therapy resulted in larger CTR (0.63 ± 0.08 vs. 0.60 ± 0.09, p = 0.016), RAA (30 [(24-40)] vs. 25 [(19-34)] cm(2), p = 0.020), and RVD (5.30 ± 0.97 vs. 4.65 ± 0.85 cm, p < 0.001) compared with non triple therapy. After adjusting for confounders, intensive therapy independently predicted increases in CTR (0.03, 95% CI 0.00-0.05, p = 0.054), RAA (6.63 cm(2), 95% CI 1.46-11.80, p = 0.013), and RVD (0.66 cm, 95% CI 0.34-0.98, p < 0.001). CONCLUSION: These findings suggest that more aggressive PAH treatment is associated with greater right heart remodeling, highlighting the complex relationship between therapeutic intervention and disease progression in PAH patients.