Abstract
Background: Severe pulmonary hypertension (PH) due to COPD has dismal prognosis. PH-target-therapy may worsen ventilation-perfusion mismatch and is insufficiently studied.
Method: For COPD-PH-patients, PH-target-therapy, NYHA, 6MWD, SpO2 and QoL were noted at baseline,3,6,12&24 months.
Results: Data from 27 patients were analysed (7 females, 21 smokers, 23 emphysema) baseline median(quartiles):age 70(60;76), FEV1 60(46;78)%, FEV1/FVC 57(51;64)%, DLCO 42(36;59)%, mPAP 39(32;44)mmHg. Therapies included inhaled-iloprost(10), sc-treprostinil(2), iv-prostanoids(3), endothelin-receptor antagonists(15) and PDE-5-inhibitors(25). Under therapy, NYHA improved from 3.5(3;4) to 3(2;4) and 3(2;3.5) at 3&6 months (p= 0.02&0.008), the 6MWD from 373(236;452) to 395(339;472), 414(285;492)& 396(308;497)m at 3,6&12 months(p= 0.005,0.006&0.011) with unchanged resting-SpO2 of 92(86;94)% but decreased SpO2 after 6MW to 87(79;92) to 81(76;87), 83(76;88)& 80(82;89)% (p=0.014, 0.014&0.116). During median follow-up of 5.9(2.3;8.4)years, 10 died (after 3.1(2.3;4.3)years, 2 were transplanted (after 2.7(1.7;3.6)years) and 2 lost-to-follow-up after 3.3(2.2;4.4)years. Transplant-free-survival at 1,2,3 years was 92,69 and 54% and was similar for GOLD-stages 1-4, but worse for patients with mPAP≥40mmHg (p= 0.026), 6MWD<370m (p= 0.008), resting SpO2<92% (p= 0.02) and post-walk SpO2<87% (p= 0.012).
Conclusion: PH-target-therapy improved NYHA and 6MWD in selected COPD patients with severe PH with worsening resting SpO2. Poor exercise capacity, low SpO2 and high mPAP but not airflow obstruction were associated with unfavourable outcome.
- © 2014 ERS