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. 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; nϭ190), 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. (Circulation. 2010;122:156-163.

Effects of the endothelin receptor antagonist bosentan on hemodynamics and exercise capacity in Japanese patients with mildly symptomatic pulmonary arterial hypertension

“…Effects of modern management are unsatisfactory and PAH still remains as a progressive and fatal disease with an estimated survival at 1, 2, and 3 years of 85.7, 69.6, and 54.9 %, respectively [4]. The most commonly reported predictors of survival are shown to be 6 MWD, WHO FC, and pulmonary hemodynamics [5].…”

“…Thereby, PAH patients in WHO FC I or II were shown to have significantly better long-term survival rates compared with those in WHO FC III or IV [4]. In China, 72 patients with IPAH and FPAH were enrolled from 1999 to 2004 and followed up for a mean duration of 40 months [9].…”

Current Treatment Approaches to Pulmonary Arterial Hypertension

“…A C C E P T E D ACCEPTED MANUSCRIPT 6 anticoagulants was not associated with a survival benefit in patients with other forms of PAH. Taken together, evidence in favour of oral anticoagulation is confined to patients with IPAH, heritable PAH, and PAH due to anorexigens only.…”

Advancing knowledge of right ventricular pathophysiology in chronic pressure overload: Insights from experimental studies

“…Actually, prolonged survival is related more to RV function than to pulmonary haemodynamics per se. In studies addressing haemodynamic variables and survival in PH, high mean right atrial pressures and low cardiac output are consistently associated with poorer survival [4][5][6]. Despite major improvements in pharmacological management over the last 15 years, PH patients still die from RVF.…”