Importance: Pulmonary hypertension (PH) is a fatal disease. Although the prognosis of pulmonary arterial hypertension (PAH) has improved with targeted therapies, the outcome is dependent on early detection and an accurate diagnosis. Objective: To determine the accuracy of PH diagnoses in patients referred to PH centers and the frequency of PAH-specific medication use despite an uncertain or incorrect diagnosis. Design: Multicenter, descriptive, cross-sectional study. During a 10-month period in 2010 and 2011, data on newly referred patients were collected and entered into a secure Internet database. Setting: Three large tertiary PH centers. Participants: One hundred forty consecutive patients newly referred to PH centers were invited to participate, and all consented to do so. Results: Of 140 patients referred with a mean age of 56 years, 95 (68%) were referred by cardiologists or pulmonologists and 86 (61%) had disease classified as World Health Organization functional class III or IV. Fifty-six of the prereferral diagnoses (40%) were PAH, 42 (30%)

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Background-The safety of β-blockers in patients with isolated right ventricular failure because of pulmonary arterial hypertension (PAH) is unclear. Methods and Results-We studied 564 PAH patients (total cohort) referred to our center from 1982 to 2013. Propensity scorematching was used to match pairs of PAH patients with and without β-blocker use (matched cohort). We compared all-cause mortality between the groups in the total cohort and the matched cohort using bootstrap validation, Kaplan-Meier, and Cox proportional hazard analyses. Seventy-one of the 564 patients in the total cohort were on β-blockers. They were older, had higher prevalence of comorbidities, and were more often on diuretics, digoxin, and angiotensin converting enzyme inhibitors. The severity of PAH and right ventricular failure was similar between those with and without β-blocker use. After propensity matching, 63 patients with β-blocker use were compared with 51 patients without β-blocker use. During a median follow-up time of 4.8 years, there were 339 (60%) deaths in the total cohort and 70 deaths (61%) in the matched cohort. There was no difference in absolute mortality between those with and without β-blockers (P=0.71). β-Blocker use was not associated with increased all-cause mortality in the total cohort after adjusting for propensity score (adjusted hazard ratio, 1.0; 95% confidence interval, 0.7-1.5) and in the matched cohort (hazard ratio, 1.2; 95% confidence interval, 0.8-2.0). Conclusions-There was no statistically significant difference in long-term mortality between propensity score-matched pairs of PAH patients with and without β-blocker use. These findings need further validation in prospective clinical trials. (Circ Heart Fail. 2014;7:903-910.)Key Words: adrenergic blockers ◼ heart failure ◼ pulmonary hypertension ◼ right ventricle

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