title: Use of pulmonary arterial hypertension-specific therapy in non-WHO group I pulmonary hypertension.
abstract: OBJECTIVE
  The development of pulmonary hypertension (PH) in non-World Health Organization group I PH adversely affects exercise capacity. It is unclear whether pulmonary artery hypertension (PAH)-specific drugs improve pulmonary hemodynamics and exercise capacity in such patients.
  
  
  METHODS
  We performed a retrospective chart review of consecutive patients with non-World Health Organization group I PH treated with PAH-specific therapy.
  
  
  RESULTS
  We identified 24 patients. The mean (standard deviation) age was 48 (14.8) years. Seventeen (71%) patients were women. The 6-minute walk distance improved significantly for the whole group in an initial follow-up period of 4.6 (2.3) months; however, the improvement was seen only in patients with obstructive sleep apnea (OSA) or severe PH and it was not sustained during a longer follow-up period of 11.5 (4.1) months, except in patients with OSA. PH was treated with a variety of PAH-specific drugs, including combination therapy in five patients.
  
  
  CONCLUSIONS
  The use of PAH-specific therapy in selected patients with PH secondary to lung diseases, OSA, or sarcoidosis may result in significant improvement in 6-minute walk distance, particularly in patients with OSA or severe PH.
authors.affiliation: From the Division of Pulmonary and Critical Care Medicine, University of Florida at Jacksonville.
authors.forename: Adil
authors.initials: A
authors.lastname: Shujaat
authors.affiliation: From the Division of Pulmonary and Critical Care Medicine, University of Florida at Jacksonville.
authors.forename: Jason
authors.initials: J
authors.lastname: Bellardini
authors.affiliation: From the Division of Pulmonary and Critical Care Medicine, University of Florida at Jacksonville.
authors.forename: James D
authors.initials: JD
authors.lastname: Cury
authors.affiliation: From the Division of Pulmonary and Critical Care Medicine, University of Florida at Jacksonville.
authors.forename: Abubakr A
authors.initials: AA
authors.lastname: Bajwa
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D015331:Cohort Studies; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D005080:Exercise Test; D017079:Exercise Tolerance / Q000502:physiology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D017563:Lung Diseases, Interstitial / Q000150:complications; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use*; D011453:Prostaglandins / Q000627:therapeutic use; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000150:complications; D012720:Severity of Illness Index; D020181:Sleep Apnea, Obstructive / Q000150:complications; D016896:Treatment Outcome; D014944:World Health Organization
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D011453:Prostaglandins; D011464:Epoprostenol; C427248:treprostinil