Prognostic factors in severe pulmonary hypertension patients who need parenteral prostanoid therapy: The impact of late referral

BACKGROUND: Oral drugs have made the treatment of pulmonary hypertension (PH) feasible in non-expert centers, which could delay patient access to prostanoid therapy. METHODS: Fifty-seven consecutive patients with precapillary PH received a prostanoid in our center. Data at prostanoid initiation included modality of center referral, medical history, New York Heart Association [NYHA] class, exercise capacity, echocardiographic parameters, and hemodynamics. RESULTS: Overall survival at 1, 2, and 3 years was 85%, 69%, 55%, respectively. Non-survivors had worse NYHA class III/IV (17/12) than survivors (27/1; p < 0.01) and exercise capacity on 6-minute-walk distance (254 +/- 114 vs 354 +/- 91 meters; p < 0.01). Non-survivors were more frequently referred on oral therapy (83% vs 36%; p < 0.01) and had a higher rate of urgent prostanoid treatment (69% vs 17%;p < 0.0001). Multivariate analysis (hazard ratio [95% confidence interval]) found the independent prognostic factors were urgent prostanoid therapy (2.0 [1.1-3.9]) and NYHA class (3.5 [1.5-8.2]). Survivors had a significant response to prostanoid, improving NYHA class from 2.8 +/- 0.4 to 2.3 +/- 0.5 (p = 0.002), 6-minute walk distance from 354 +/- 91 to 426 +/- 82 meters (p = 0.0001), and pulmonary hemodynamics (pulmonary artery pressure from 56 +/- 13 to 44 +/- 18 mm Hg [p < 0.05]; cardiac index from 2.0 +/- 1.2 to 3.1 +/- 1.2 liters/min/m(2) [p = 0.002], and pulmonary vascular resistance from 17 +/- 10 to 8 +/- 6 WU [p = 0.001]). CONCLUSIONS: Referral of patients on oral treatment to a tertiary PH center is delayed and significantly affects prognosis. J Heart Lung Transplant 2012;31:364-72 (C) 2012 International Society for Heart and Lung Transplantation. All rights reserved.