Actelion Ltd.: REVEAL(R) RegistryData May Advance Patient Care in Pulmonary Arterial Hypertension

NEW ORLEANS, May 17 /PRNewswire-FirstCall/ --Actelion Ltd (SIX: ATLN) announced today that findings from the REVEAL® (Registry to EValuate Early And Long-term PAH Disease Management) Registry have confirmed the clinical utility of the REVEAL risk score calculator, a new tool designed to predict 1-year survival in patients with pulmonary arterial hypertension (PAH) according to data presented at the American Thoracic Society's (ATS) Annual International Conference. The predictive tool was developed and validated in patients enrolled in REVEAL, the largest prospective registry in PAH containing data from more than 3,500 patients.

"The REVEAL risk score calculator provides an innovative approach to better understand pulmonary arterial hypertension by looking at a unique combination of variables, that together, can help predict patient survival," said Raymond L. Benza, M.D., Medical Director of the Gerald McGinnis Cardiovascular of Advanced Heart Failure, Transplantation, Mechanical Circulatory Support and Pulmonary Hypertension Program, and Director of the Gerald McGinnis Cardiovascular Institute Center for Research and Innovation at Allegheny General Hospital, Pittsburgh. "We look forward to obtaining more data from the REVEAL Registry that can be used as an adjunct to the clinical experience and judgment of physicians who treat this chronic and life-threatening disease."

About "Use of REVEAL PAH Risk Score Calculator in Newly Diagnosed Patients"

Data from 2,717 newly and previously diagnosed PAH patients enrolled in REVEAL were used to develop a predictive tool from a stepwise model based on nineteen variables, including WHO Group I subgroup, mean right atrial pressure, pulmonary vascular resistance and functional class, that together predict 1-year survival. The final model was simplified into a risk score of 1-15 and tested in 398 newly diagnosed PAH patients. The 1-year survival of this cohort of patients with risk scores of 1 to 5 was 99.1%; 6 to 7 was 95.5%; 8 to 9 was 91.1%; 10 to 11 was 79.1%; and 12 to 15 was 50.4%.

About "Refining Definitions of Time to Clinical Worsening in Connective Tissue Disease Associated with PAH (CTD-PAH)

A second presentation today demonstrated that data from the REVEAL Registry may improve physicians' ability to determine the prognosis of patients with connective tissue disease associated pulmonary arterial hypertension (CTD-APAH). The study led by Roham T. Zamanian, M.D., Division of Pulmonary & Critical Care Medicine, Stanford University Medical Center, Stanford, Calif., found that current parameters that include all-cause hospitalization and addition of IV/SC prostacyclin therapies may not be appropriate for defining time to clinical worsening (TTCW) for patients with CTD-APAH.

The analysis included 586 newly diagnosed idiopathic PAH and CTD-APAH patients with a median follow up of 15 months, and compared two definitions of TTCW: major event, worsening functional class, or 15% reduction in 6-minute walk test versus major event, worsening functional class, 15% reduction in 6-minute walk test, all-cause hospitalization, or addition of IV/SC prostacyclin for any reason. Results showed TTCW defined by the first criteria predicted the worst outcomes in CTD-APAH patients, but when all-cause hospitalization and addition of prostacyclin therapies were added the predictive value of the model was no longer statistically significant.

Additional Data at ATS

Two additional posters from the REVEAL Registry will be presented at ATS on Tuesday, May 18:

  • HIV-APAH compared to IPAH: Baseline characteristics and 2 year outcomes from the REVEAL Registry by Gregg J. Stashenko, M.D., Duke University Medical Center, Durham, N.C.
  • Prognostic Value of Percent Predicted Six Minute Walk Test Distance in Idiopathic Pulmonary Arterial Hypertension Patients: an Analysis of the REVEAL Registry by Stephen C. Mathai, M.D., M.H.S., Johns Hopkins University School of Medicine, Department of Medicine, Division of Pulmonary and Critical Care Medicine, Baltimore.

About Pulmonary Arterial Hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder characterized by abnormally high blood pressure in the arteries between the heart and lungs of an affected individual. The function of the heart and lungs is severely compromised, manifested by a limited exercise capacity, and, ultimately, a reduced life expectancy. Approximately 100,000 people in Europe and the United States are afflicted with either primary or secondary forms of the disease related to conditions or tissue disorders that affect the lungs, such as scleroderma, lupus, HIV/AIDS or congenital heart disease.

PAH is associated with structural changes in both the pulmonary vasculature and the right ventricle. Recent advances [1] in the understanding of the pathogenic factors leading to the pulmonary vascular disease have led to the development of new therapies targeting specific pathways (the prostacyclin pathway; the endothelin pathway; and the nitric oxide pathway) [2]. The available therapies have shown positive treatment effects in patients with PAH, but they do not provide a cure, and in many patients the disease will progress. PAH remains a serious life-threatening condition [2,3]. Early recognition and an understanding of the selection and timing of therapeutic options remain critical elements in the optimal management of PAH patients.


1. Farber HW; Loscalzo J. Mechanisms of disease: pulmonary arterial hypertension. N. Eng. J. Med. 2004; 351:1655-65.

2. Humbert M; Sitbon O; Simonneau G. Treatment of pulmonary arterial hypertension. N. Eng. J. Med. 2004;351:1425-36.

3. Humbert M; Morrell NW; Archer SL; et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2004; 43: Suppl. 12: 13S-24S.

About the REVEAL® Registry

The REVEAL Registry is a multi-center, observational, U.S.-based study of the clinical course and disease management of patients with PAH. Approximately 3,500 previously and newly diagnosed patients from over 50 participating centers across the United States have been enrolled and tracked in the REVEAL Registry. Patients are followed for a minimum of five years from the time of enrollment. The registry has enrolled consenting newly diagnosed or previously diagnosed patients with World Health Organization (WHO) Group I PAH, who meet specific hemodynamic criteria based upon prior performance of right heart catheterization. The REVEAL Registry is sponsored by Actelion Pharmaceuticals. For more information, please visit or call 1-877-REVEAL5 (877-738-3255).

About Actelion Ltd

Actelion Ltd is a biopharmaceutical company headquartered in Allschwil/Basel, Switzerland. Actelion's first drug Tracleer®, an orally available dual endothelin receptor antagonist, has been approved as a therapy for pulmonary arterial hypertension. Actelion markets Tracleer® through its own subsidiaries in key markets worldwide, including the United States (based in South San Francisco), the European Union, Japan, Canada, Australia and Switzerland. Actelion, founded in late 1997, is a leading player in innovative science related to the endothelium - the single layer of cells separating every blood vessel from the blood stream. Actelion's 2000-plus employees focus on discovery, development and marketing of innovative drugs for significant unmet medical needs. Actelion shares are traded on the SIX Swiss Exchange (ticker symbol: ATLN) as part of the Swiss blue-chip index SMI (Swiss Market Index SMI®).

SOURCE Actelion Ltd

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