title: Long-term effect of vasodilator therapy in pulmonary hypertension due to COPD: a retrospective analysis.
abstract: OBJECTIVE
  Pulmonary hypertension (PH) due to COPD has dismal prognosis. We reviewed the long-term effect of PH-target therapy in severe PH-COPD.
  
  
  METHODS
  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 (SpO(2)) at baseline, 3, 6, 12 and 24 months were analysed.
  
  
  RESULTS
  Of 48 PH-COPD identified 21 were excluded (insufficient data, comorbidity). 27 patients (7 females, 21 smokers, 23 emphysema) with median (quartiles) baseline age 70 (60; 76) years, FEV1 60 (46; 78) %, FEV1/FVC 57 (51; 64) %, DLCO 42 (36; 59) %, mean pulmonary artery pressure 39 (32;44) 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 (3; 4) to 3 (2; 4) after 3 months and 3 (2; 3.5) after 6 months (p = .02 and .008). The 6MWD improved from 373 (236; 452) to 395 (339; 472), 414 (285; 492) and 396 (308; 497)m at 3, 6 and 12 months (p = .005, .006 and .011) with unchanged resting-SpO(2) but decreased peak-exercise SpO(2). During median follow-up of 5.9 (2.3; 8.4) 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 ≥40 mmHg (p = .026), 6MWD <370 m (p = 0.008), resting SpO(2) <92 % (p = 0.02) and peak-walk SpO(2) <87 % (p = 0.012).
  
  
  CONCLUSIONS
  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 SpO(2) and high mean pulmonary artery pressure at baseline but not airflow obstruction were associated with unfavourable outcome.
authors.affiliation: Head of Pulmonary Hypertension Unit, Clinic for Pulmonology, University Hospital of Zurich, Raemistrasse 100, 8091, Zurich, Switzerland, laurafossati86@gmail.com.
authors.forename: Laura
authors.initials: L
authors.lastname: Fossati
authors.forename: Séverine
authors.initials: S
authors.lastname: Müller-Mottet
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Hasler
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Konrad E
authors.initials: KE
authors.lastname: Bloch
authors.forename: Lars C
authors.initials: LC
authors.lastname: Huber
authors.forename: Silvia
authors.initials: S
authors.lastname: Ulrich Somaini
mesh_terms: D000368:Aged; D015331:Cohort Studies; D016208:Databases, Factual; D018450:Disease Progression; D016503:Drug Delivery Systems / Q000379:methods; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008134:Long-Term Care; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D014665:Vasodilator Agents