التفاصيل البيبلوغرافية
| العنوان: |
Survival in Patients With Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension in the Modern Management Era |
| المؤلفون: |
Humbert, Marc, Sitbon, Olivier, Chaouat, Ari, Bertocchi, Michèle, Habib, Gilbert, Gressin, Virginie, Yaïci, Azzedine, Weitzenblum, Emmanuel, Cordier, Jean-François, Chabot, François, Dromer, Claire, Pison, Christophe, Reynaud-Gaubert, Martine, Haloun, Alain, Laurent, Marcel, Hachulla, Eric, Cottin, Vincent, Degano, Bruno, Jaïs, Xavier, Montani, David, Souza, Rogério, Simonneau, Gérald |
| المصدر: |
Circulation ; volume 122, issue 2, page 156-163 ; ISSN 0009-7322 1524-4539 |
| بيانات النشر: |
Ovid Technologies (Wolters Kluwer Health) |
| سنة النشر: |
2010 |
| الوصف: |
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. Methods and 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. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1161/circulationaha.109.911818 |
| DOI: |
10.1161/CIRCULATIONAHA.109.911818 |
| الإتاحة: |
https://doi.org/10.1161/circulationaha.109.911818Test |
| رقم الانضمام: |
edsbas.86414B60 |
| قاعدة البيانات: |
BASE |
