title: Pulmonary hypertension survival effects and treatment options in cystic fibrosis.
abstract: OBJECTIVE
  This review discusses the current impact of pulmonary hypertension on the outcome and treatment of cystic fibrosis (CF).
  
  
  RESULTS
  Pulmonary hypertension is commonly encountered in advanced lung diseases such as CF. The prevalence of pulmonary hypertension in CF patients varies based on disease severity and methodology used for diagnosis. Chronic alveolar hypoxia is the most likely cause. The majority of recent studies have shown worse survival in CF patients who develop pulmonary hypertension. The impact of pulmonary hypertension-specific therapies on symptomatology and outcomes in CF patients has not been well studied.
  
  
  CONCLUSIONS
  Pulmonary hypertension is common in patients with CF and it occurs largely because of hypoxemia. The presence of pulmonary hypertension in patients with CF is likely associated with worse outcome; however, it remains unknown whether treatment with pulmonary hypertension-specific therapies would be beneficial.
authors.affiliation: Department of Pulmonary, Allergy, and Critical Care Medicine, Respiratory Institute, Cleveland, Ohio, USA.
authors.forename: Adriano R
authors.initials: AR
authors.lastname: Tonelli
mesh_terms: D002245:Carbon Dioxide / Q000097:blood; D003550:Cystic Fibrosis / Q000097:blood / Q000150:complications / Q000401:mortality / Q000628:therapy*; D018450:Disease Progression; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood / Q000209:etiology / Q000401:mortality* / Q000628:therapy*; D000860:Hypoxia / Q000097:blood*; D016040:Lung Transplantation / Q000706:statistics & numerical data*; D008297:Male; D015995:Prevalence; D011379:Prognosis; D012307:Risk Factors; D012720:Severity of Illness Index; D015996:Survival Rate
chemical_list: D002245:Carbon Dioxide
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