Abstract
Background: Recent studies have shown that pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in PAH. However, it remains unknown whether PAH-approved drugs also improve RV morphology and function in lung disease-associated pulmonary hypertension (group 3 PH).
Aim: To evaluate the RV morphology and systolic function in patients with group 3 PH treated with PAH-approved vasodilators.
Methods: Consecutive patients with severe group 3 PH, defined as mean pulmonary arterial pressure ≥ 35 mmHg or cardiac index < 2 L/min/m2, were studied. Right heart catheterization and cardiac magnetic resonance imaging were performed at baseline and at 3–4 months follow-up, during which the patients received an optimal medical treatment including oxygen plus sildenafil with or without other PAH-approved drug(s).
Results: A total of 10 patients were enrolled in the study (mean age, 66 ±15 years). Their underlying lung diseases were combined pulmonary fibrosis and emphysema (n = 5), interstitial pneumonia (n = 2), chronic obstructive pulmonary disease (n = 2), and cystic lung disease (n = 1). One patient died during the follow-up period. Compared with the baseline data, the follow-up data (n = 9) indicated significant decrease in RV end-diastolic volume index (125 ± 13 to 96 ± 7 mL/m2, p = 0.013) and pulmonary vascular resistance (11.8 ± 1.5 to 7.5 ±1.0 W.U., p = 0.014), and an increase in RV ejection fraction (24% ± 3% to 35% ± 4%, p = 0.015).
Conclusion: PAH-approved drug treatment improves RV dilatation and systolic function in patients with severe group 3 PH.
- Copyright ©ERS 2015
