Pulmonary hypertension ( PH ) due to COPD ( chronic obstructive pulmonary disease ) has dismal prognosis. Researchers reviewed the long-term effect of PH-target therapy in severe PH-COPD.

Patients attending our PH-clinic were reviewed for PH-COPD receiving PH-target therapy. Baseline characteristics, death / transplantation until 2014, therapy, NYHA functional class, 6 min walk distance ( 6MWD ) and oxygen saturation ( SpO2 ) at baseline, 3, 6, 12 and 24 months were analysed.

Of 48 PH-COPD identified 21 were excluded ( insufficient data, comorbidity ). 27 patients ( 7 females, 21 smokers, 23 emphysema ) with median baseline age 70 years, FEV1 60%, FEV1/FVC 57%, DLCO 42%, mean pulmonary artery pressure 39 mmHg under inhaled Iloprost ( 10 ), subcutaneous prostanoids ( 2 ), intravenous prostanoids ( 3 ), endothelin receptor antagonists ( 15 ) and phosphodiesterase-5-inhibitors ( 25 ) were included.

Under therapy, NYHA functional class improved from 3.5 to 3 after 3 months and 3 after 6 months ( p = 0.02 and 0.008 ).

The 6MWD improved from 373 to 395, 414 and 396 m at 3, 6 and 12 months ( p =0.005, 0.006 and 0.011 ) with unchanged resting-SpO2 but decreased peak-exercise SpO2.

During median follow-up of 5.9 years, 10 died, 2 were transplanted and 2 were lost to follow-up. Transplant-free survival at 1, 2, 3 years was 92, 69, 54 % and was similar for GOLD stages 1-4, but worse for patients with mPAP greater than or equal to 40 mmHg ( p = 0.026 ), 6MWD less than 370 m ( p = 0.008 ), resting SpO2 less than 92 % ( p = 0.02 ) and peak-walk SpO2 less than 87 % ( p = 0.012 ).

In conclusion, PH-target vasodilator therapy improved NYHA functional class and 6MWD up to one year in highly selected patients with severe PH-COPD.
Poor exercise capacity, low SpO2 and high mean pulmonary artery pressure at baseline but not airflow obstruction were associated with unfavourable outcome. ( Xagena )

Fossati L et al, Lung 2014;192:987-995

XagenaMedicine2014