Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era.
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.
Humbert
Marc
M
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
Sitbon
Olivier
O
Chaouat
Ari
A
Bertocchi
Michèle
M
Habib
Gilbert
G
Gressin
Virginie
V
Yaïci
Azzedine
A
Weitzenblum
Emmanuel
E
Cordier
Jean-François
JF
Chabot
François
F
Dromer
Claire
C
Pison
Christophe
C
Reynaud-Gaubert
Martine
M
Haloun
Alain
A
Laurent
Marcel
M
Hachulla
Eric
E
Cottin
Vincent
V
Degano
Bruno
B
Jaïs
Xavier
X
Montani
David
D
Souza
Rogério
R
Simonneau
Gérald
G
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