As compared with conventional therapy, the continuous intravenous infusion of epoprostenol produced symptomatic and hemodynamic improvement, as well as improved survival in patients with severe primary pulmonary hypertension.

The diagnosis and assessment of pulmonary arterial hypertension is a rapidly evolving area, with changes occurring in the definition of the disease, screening and diagnostic techniques, and staging and follow-up assessment. The definition of pulmonary hypertension has been simplified, and is now based on currently available evidence. There has been substantial progress in advancing the imaging techniques and biomarkers used to screen patients for the disease and to follow up their response to therapy. The importance of accurate assessment of right ventricular function in following up the clinical course and response to therapy is more fully appreciated. As new therapies are developed for pulmonary arterial hypertension, screening, prompt diagnosis, and accurate assessment of disease severity become increasingly important. A clear definition of pulmonary hypertension and the development of a rational approach to diagnostic assessment and follow-up using both conventional and new tools will be essential to deriving maximal benefit from our expanding therapeutic armamentarium.

These cases arouse concern that fenfluramine-phentermine therapy may be associated with valvular heart disease. Candidates for fenfluramine-phentermine therapy should be informed about serious potential adverse effects, including pulmonary hypertension and valvular heart disease.

Background-Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Methods and Results-Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early andLong-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance Ͼ32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men Ͼ60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. Conclusions-We Editorial see p 106 Clinical Perspective on p 172Although 6-minute walk distance (6MWD) and other end points are considered potential surrogates for survival of patients with PAH, they have never been thoroughly tested for their predictive abilities. However, these factors are often used to make critical decisions about the utility and efficacy of present-day therapeutics. 5 The Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) is a Methods REVEAL Study DesignREVEAL is an observational prospective registry study that consecutively enrolled patients with a diagnosis of WHO group I PAH meeting prespecified hemodynamic criteria at 54 geographically diverse community and university PAH specialty care facilities in the United States. 6 Both newly and previously diagnosed patients have been enrolled and will be followed up for at least 5 years unless discontinued from the study because of withdrawal of consent, death, or loss to follow-up. There are no protocol-mandated tests, treatments, or visit schedules. Study objectives and methods were prespecified in an Institutional Review Board-approved protocol, and all participants or their legal guardians gave written informed consent. Data from right heart catheterization were categorized as meeting traditional or expanded hemodynamic criteria for WHO group I PAH (ie, pulmonary capillary wedge pressure Յ15 versus 16 to 18 mm Hg); only patients meeting t...

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