Background: The 55-center, observational, US Registry to EValuate Early And Long-term pulmonary arterial hypertension (PAH) Disease Management (REVEAL) examines demographics, clinical course and management of patients (pts) diagnosed with PAH. Methods: Pts aged ≥3 months and ≤18 years at time of confirmatory diagnostic catheterization were followed at 26 sites. Data were analyzed to explore demographics, treatment and outcomes in the following PAH subgroups: idiopathic PAH (IPAH), familial PAH (FPAH), and PAH associated with congenital heart disease (APAH-CHD). Results: 3518 pts enrolled in REVEAL between 03/2006 and 12/2009; 77 APAH-CHD and 122 IPAH/FPAH had childhood-onset PAH; mean ± SD age at PAH diagnosis 6±6 years and 9±6 years ( P = 0.002), respectively. APAH-CHD had similar functional class (FC) at diagnosis compared with IPAH/FPAH (FC: I, 5%; II, 51%; III, 32%; and IV, 11% vs FC: I, 7%; II, 40%; III, 43%; and IV, 10%, respectively; P = 0.64). Hemodynamics were also similar at diagnosis. Similar proportions were treated with endothelin receptor antagonists (APAH-CHD, 40%; IPAH/FPAH, 45%; P = 0.53), though phosphodiesterase 5 inhibitors (45% vs 64%, respectively; P = 0.011) and prostacyclin (28% vs 50%, respectively; P = 0.003) analogs were used less in APAH-CHD. Five-year survival from diagnosis was similar for APAH-CHD and IPAH/FPAH (71±14% vs 75±7% vs, respectively; P = 0.53). Conclusion: Childhood-onset APAH-CHD and IPAH/FPAH have similar hemodynamics and FC and similar 5-year survival from diagnosis. REVEAL provides observational data on treatment and outcomes in childhood PAH that should prove useful in identifying prognostic parameters, thereby helping clinicians improve outcomes.

BACKGROUND Pulmonary arterial hypertension (PAH) is a life-threatening disease that affects more women than men. The reasons for the female preponderance are unclear, and there are limited data available for men with PAH. METHODS Data from the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) were analyzed to explore sex differences among patients with PAH with regard to 2-year survival from enrollment and 5-year survival from time of diagnosis. RESULTS The data set included 2,318 women and 651 men. More women had PAH associated with connective tissue disease (P < .001), and more men had portopulmonary hypertension (P < .001) and HIV-associated PAH (P < .001). More women had congenital heart disease-associated PAH (P = .017), thyroid disease (P < .001), and depression reported (P ≤ .001). At diagnosis, men had higher mean pulmonary artery pressure (53 ± 14 vs 51 ± 14.3 mm Hg; P = .013) and mean right atrial pressure (10 ± 6 vs 9 ± 6 mm Hg; P = .031). Women had better survival estimates for 2 years from enrollment and for 5 years from diagnosis. Stratifying by age showed that survival from enrollment was similar between men and women aged < 60 years at enrollment, whereas men aged ≥ 60 years have lower survival rates compared with women aged ≥ 60 years. CONCLUSIONS Our findings highlight similarities and differences between men and women with PAH, raising questions for future exploration regarding the role of hormones and sex in causation and survival in PAH. TRIAL REGISTRY ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.

BACKGROUND:REVEAL (The Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) provides current demographics of patients with group 1 pulmonary arterial hypertension (PAH) in the United States. METHODS:A total of 2,967 patients with PAH diagnosed based on right-sided heart catheterization were enrolled in REVEAL between March 2006 and September 2007. Demographics from the REVEAL patient cohort and REVEAL subpopulations (matched by inclusion criteria to other registries) were compared with historic US registry data and other contemporary US and non-US national PAH registries by inclusion criteria, including the National Institutes of Health (NIH) PAH registry and the French PAH registry. RESULTS:REVEAL patients matched to NIH registry patients were older at diagnosis (mean ± SE, 44.9 ± 0.6 years vs 36.4 ± 1.1 years; difference, 8.5 ± 1.4; P < .001) and more likely to be women (78.7 ± 1.2% vs 63.1 ± 3.5%; P < .001). REVEAL patients matched to French registry patients had similar age and severity at diagnosis, but REVEAL patients were more likely to be women (79.8 ± 0.8% vs 65.3 ± 1.8%; P < .001) and obese (BMI, ≥ 30 kg/m(2), 32.5 ± 1.0% vs 14.8 ± 1.4%; P < .001), whereas French patients were more likely to have HIV-associated PAH (6.2% vs 2.3%). The female preponderance is similar to that in other US-based contemporary registries. CONCLUSIONS:At diagnosis, REVEAL patients were older than NIH registry patients and similar in age to patients enrolled in contemporary registries. Compared with NIH and contemporary European and UK registries, there was a striking preponderance of women, and REVEAL patients were more likely to be obese. These observations and the difference in HIV-associated PAH between REVEAL and other non-US contemporary registries warrant further investigation. TRIAL REGISTRY:ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov.

The Registry to EValuate Early And Long-term PAH Disease Management (REVEAL) is a multicenter, observational, U.S. study designed to assess demographic, clinical and management data on patients diagnosed with pulmonary arterial hypertension (PAH).

The Registry to EValuate Early And Long-term PAH Disease Management (REVEAL), a multicenter, observational, U.S. study, is designed to meet the need for current and reliable information about demographics, clinical course and management of patients with pulmonary arterial hypertension (PAH).

The Registry to EValuate Early And Long-term PAH Disease Management (REVEAL) is a multicenter, observational, U.S. study designed to assess demographic, clinical and management data on patients diagnosed with pulmonary arterial hypertension (PAH). Strengths of the registry are the large number of patients enrolled and the ability to follow all patients a minimum of 5 years from enrollment.

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