title: Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era.
abstract: BACKGROUND
  Novel therapies have recently become available for pulmonary arterial hypertension. We conducted a study to characterize mortality in a multicenter prospective cohort of patients diagnosed with idiopathic, familial, or anorexigen-associated pulmonary arterial hypertension in the modern management era.
  
  
  RESULTS
  Between October 2002 and October 2003, 354 consecutive adult patients with idiopathic, familial, or anorexigen-associated pulmonary arterial hypertension (56 incident and 298 prevalent cases) were prospectively enrolled. Patients were followed up for 3 years, and survival rates were analyzed. For incident cases, estimated survival (95% confidence intervals [CIs]) at 1, 2, and 3 years was 85.7% (95% CI, 76.5 to 94.9), 69.6% (95% CI, 57.6 to 81.6), and 54.9% (95% CI, 41.8 to 68.0), respectively. In a combined analysis population (incident patients and prevalent patients diagnosed within 3 years before study entry; n=190), 1-, 2-, and 3-year survival estimates were 82.9% (95% CI, 72.4 to 95.0), 67.1% (95% CI, 57.1 to 78.8), and 58.2% (95% CI, 49.0 to 69.3), respectively. Individual survival analysis identified the following as significantly and positively associated with survival: female gender, New York Heart Association functional class I/II, greater 6-minute walk distance, lower right atrial pressure, and higher cardiac output. Multivariable analysis showed that being female, having a greater 6-minute walk distance, and exhibiting higher cardiac output were jointly significantly associated with improved survival.
  
  
  CONCLUSIONS
  In the modern management era, idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension remains a progressive, fatal disease. Mortality is most closely associated with male gender, right ventricular hemodynamic function, and exercise limitation.
authors.affiliation: Service de Pneumologie et Réanimation Respiratoire, Faculté de Médecine, Hôpital Antoine Béclère, Assistance-Publique-Hôpitaux de Paris, Université Paris-Sud 11, 92140 Clamart, France. marc.humbert@abc.aphp.fr
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: Ari
authors.initials: A
authors.lastname: Chaouat
authors.forename: Michèle
authors.initials: M
authors.lastname: Bertocchi
authors.forename: Gilbert
authors.initials: G
authors.lastname: Habib
authors.forename: Virginie
authors.initials: V
authors.lastname: Gressin
authors.forename: Azzedine
authors.initials: A
authors.lastname: Yaïci
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Weitzenblum
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.forename: François
authors.initials: F
authors.lastname: Chabot
authors.forename: Claire
authors.initials: C
authors.lastname: Dromer
authors.forename: Christophe
authors.initials: C
authors.lastname: Pison
authors.forename: Martine
authors.initials: M
authors.lastname: Reynaud-Gaubert
authors.forename: Alain
authors.initials: A
authors.lastname: Haloun
authors.forename: Marcel
authors.initials: M
authors.lastname: Laurent
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Bruno
authors.initials: B
authors.lastname: Degano
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: David
authors.initials: D
authors.lastname: Montani
authors.forename: Rogério
authors.initials: R
authors.lastname: Souza
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D000328:Adult; D000368:Aged; D002302:Cardiac Output; D005260:Female; D005500:Follow-Up Studies; D030342:Genetic Diseases, Inborn / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology; D066298:In Vitro Techniques; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011446:Prospective Studies; D012737:Sex Factors; D015996:Survival Rate