Right Heart Remodeling in End-Stage Pulmonary Arterial Hypertension and the Impact of Treatment Intensity
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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 cm² (median 27, IQR 21–38), and 4.97±0.97 cm, respectively, with extremes of 0.88, 102 cm², 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] cm², p=0.020), and RVD (5.30±0.97 vs. 4.65±0.85 cm, p<0.001) compared with monotherapy. 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 cm², 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.
