Survival in Patients With Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension in the Modern Management Era

• Published in: Circulation (New York, N.Y.) Vol. 122; no. 2; pp. 156 - 163
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 13.07.2010
• Subjects: Adult; Aged; Biological and medical sciences; Blood and lymphatic vessels; Cardiac Output; Cardiology. Vascular system; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Female; Follow-Up Studies; Genetic Diseases, Inborn - drug therapy; Genetic Diseases, Inborn - mortality; Genetic Diseases, Inborn - physiopathology; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - mortality; Hypertension, Pulmonary - physiopathology; In Vitro Techniques; Incidence; Male; Medical sciences; Middle Aged; Pneumology; Prevalence; Prospective Studies; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Sex Factors; Survival Rate; Physical exercise; Human; Hypertension; risk factors; Respiratory disease; Mortality; Idiopathic; sex; Cardiovascular disease; exercise; pulmonary; Survival; Pulmonary hypertension; Clinical management; Risk factor
• ISSN: 0009-7322; 1524-4539; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.109.911818

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.