title: Clinical implications of haemoptysis in patients with pulmonary arterial hypertension.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a disabling disease that may result in haemoptysis. Patients with congenital heart disease associated PAH (CHD-APAH) may have a survival advantage when compared with patients with other types of PAH presenting with haemoptysis. The effects of aetiology and subsequent management choice of haemoptysis in PAH patients is not well-defined.
  
  
  METHODS
  We conducted outcome analysis in CHD-APAH vs. all other subtypes of PAH patients presenting with haemoptysis to The Methodist Hospital. Twenty-one patients were identified, 13 patients in the CHD-APAH group and eight patients in the non-CHD group. We evaluated outcomes related to treatment (bronchial artery embolisation (BAE) vs. conservative management), hospital length of stay, mortality rates and survival in this cohort.
  
  
  RESULTS
  The CHD-APAH and non-CHD groups had similar baseline demographic, haemodynamic and laboratory values except BMI was higher in the non-CHD group and haematocrit was higher in the CHD-APAH group. Twenty-eight-day mortality (0% vs. 31%) and 1-year mortality (0% vs. 54%) was lower in the CHD-APAH patients as compared with non-CHD group. A statistically significant difference was found in the survival rate in favour of CHD-APAH group for the total follow-up period (p = 0.02). Although not statistically significant, patients treated with BAE had shorter length of stay (4.0 days ± 4.0 vs. 13.7 days ± 22.5; p = 0.26). There was recurrent haemoptysis in 43% of patients treated with BAE.
  
  
  CONCLUSIONS
  Haemoptysis in PAH patients is a serious event with a high mortality rate. CHD-APAH seems to confer a survival advantage, independent of therapy utilised. Termination of haemoptysis with BAE is rapid with relatively few complications except for frequent re-bleeding episodes. Further studies are needed to determine the risk factors that may predispose PAH patients to excessive mortality from haemoptysis and to identify an optimal therapeutic modality.
authors.affiliation: Division of Pulmonary-Critical Care Medicine, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Jose
authors.initials: J
authors.lastname: Cantu
authors.forename: Degang
authors.initials: D
authors.lastname: Wang
authors.forename: Zeenat
authors.initials: Z
authors.lastname: Safdar
mesh_terms: D000328:Adult; D000368:Aged; D004621:Embolization, Therapeutic; D005260:Female; D006469:Hemoptysis / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D015996:Survival Rate; D016896:Treatment Outcome