title: Clinical worsening during long-term follow-up in inoperable chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Pulmonary endarterectomy is the treatment of choice in chronic thromboembolic pulmonary hypertension (CTEPH). Modern pulmonary vasoactive medication (like endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and prostacyclins) is used in patients with an inoperable disease and improved prognosis. We evaluate mortality and time to clinical worsening (TtCW) in inoperable CTEPH patients during long-term follow-up.
  
  
  METHODS
  All 32 patients with inoperable CTEPH were enrolled between June 2002 and January 2009. TtCW was defined as the combination of death, need for intravenous pulmonary arterial hypertension medication, or 15% decrease in 6-minute walk distance (6-MWD) without improvement in functional class. The Cox proportional hazard regression was used to identify predictors.
  
  
  RESULTS
  During a mean follow-up of 3.4 years (range = 0.2-10.2 years), 11 patients died (34%). The 1- and 3-year survival rates were 87 and 77%, respectively. Baseline functional class, 6-MWD, mean pulmonary artery pressure, and pulmonary vascular resistance were predictors for survival. Clinical worsening occurred in 16 patients (50%). The 1- and 3-year rates of freedom from clinical worsening were 74 and 60%, respectively. The only predictor for clinical worsening was the baseline 6-MWD.
  
  
  CONCLUSIONS
  Despite the improvement in medical treatment of inoperable CTEPH, the mortality rate is still high, and clinical worsening occurred in a substantial number of patients during a follow-up of more than 3 years.
authors.affiliation: Department of Cardiology, St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands.
authors.forename: Bastiaan E
authors.initials: BE
authors.lastname: Scholzel
authors.forename: Martijn C
authors.initials: MC
authors.lastname: Post
authors.forename: H W
authors.initials: HW
authors.lastname: Thijs Plokker
authors.forename: Repke J
authors.initials: RJ
authors.lastname: Snijder
mesh_terms: D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D016009:Chi-Square Distribution; D002908:Chronic Disease; D018450:Disease Progression; D018572:Disease-Free Survival; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D011655:Pulmonary Embolism / Q000150:complications; D012189:Retrospective Studies; D013997:Time Factors; D016138:Walking / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents