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AGH Study Advances Tool Used in Determining Treatment for Patients with Pulmonary Arterial Hypertension

Thursday, March 21, 2019

PITTSBURGH, Pa. – A study conducted by researchers at Allegheny General Hospital (AGH), part of Allegheny Health Network (AHN), has demonstrated the value of an innovative tool used to predict survival for patients with pulmonary arterial hypertension (PAH). The new PAH risk score calculator is expected to become the most commonly used risk prognostic tool for patients suffering from the rare but fatal disease, according to AHN cardiologist Raymond L. Benza, MD, who served as principal investigator of the study.
Published in the February issue of CHEST, the official publication of the American College of Chest Physicians, AHN’s research was tied to data from the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL), the world’s largest databank on PAH. The updated risk score system, known as REVEAL 2.0, takes into account additional data not previously factored into the REVEAL score, which includes information about other coexisting diseases, such as kidney disease; a 6-minute walk distance test; certain blood tests; pulmonary function tests; right heart catheterization; and information from previous hospitalizations.

The new research compared results from the updated REVEAL 2.0 risk score with the original REVEAL calculator as well as several European-based risk calculators. According to the study, scores derived from REVEAL 2.0 were more closely associated with an accurate survival risk than its predecessor or the European methods.

“By the time most patients suffering from PAH are diagnosed with the disease, their symptoms are severe and their life expectancy may only be a few years. So it is crucial to establish the proper course of treatment as quickly as possible in order offer the patient the best chance for a longer survival,” explained Dr. Benza, Medical Director for Advanced Heart Failure, Transplantation, Mechanical Circulatory Support, and Pulmonary Hypertension for the AHN Cardiovascular Institute. “Using these validated risk-based reassessments and treatment algorithms, cardiologists can work with their patients to guide treatment strategies so that patients receive timely, effective care and disease management.” 

There is no cure for PAH, which affects about 15 to 50 people per million in the U.S. Though one form of a broader condition known as pulmonary hypertension, PAH differs from more common forms of pulmonary hypertension, which are usually caused by other underlying diseases of the heart, lungs, or other organs. PAH occurs when the very small arteries throughout the lungs become narrow in diameter. This increases the resistance to blood flow through the lungs, and eventually the increased blood pressure can damage the heart.

AGH has earned Pulmonary Hypertension Care Center (PHCC) accreditation by the Pulmonary Hypertension Association (PHA), an accreditation that recognizes a healthcare facility’s pulmonary hypertension program that excels in diagnostic capabilities and has the capacity to appropriately and comprehensively manage pulmonary hypertension patients through a set of criteria established by the PHA. 

“AHN prides itself on providing leading-edge care for people with all forms of cardiovascular disease, including the rarest illnesses like pulmonary arterial hypertension,” said Srinivas Murali, MD, Co-Chairman, AHN Cardiovascular Institute and Interim Director, Department of Cardiovascular Medicine at AHN. “This work pioneered by Dr. Benza and his team offers fresh hope for people facing this challenging, life-limiting disease.”