PAH Mortality Risk ‘Unacceptably High’ in the Modern Era, Study Finds

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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Even with modern advances in treatments, many people in the U.S. with pulmonary arterial hypertension (PAH) are at an “unacceptably high” risk of mortality, a new study shows.

The study, “Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights From the Pulmonary Hypertension Association Registry,” was published in the Journal of the American Heart Association.

Estimates on mortality outcomes for people with PAH are based primarily on data from registries that enrolled participants before 2010. In more than a decade since those estimations were made, there have been marked improvements in PAH care, including several new treatments being approved.

A team led by scientists at the University of Minnesota sought to estimate more recent PAH mortality outcomes.

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“We aimed to determine mortality in PAH in the modern era in the United States using the PHAR (Pulmonary Hypertension Association Registry), an ongoing prospective, multicenter pulmonary hypertension registry that has been enrolling patients since 2015,” the scientists wrote.

The analysis included data for 935 people with PAH. The median age of enrollment into the registry was 56 years, and about three-quarters of the patients were women. The vast majority of patients were on one or more PAH treatments when they enrolled in the registry. About half had been enrolled for up to six months after diagnosis.

Over a median follow-up time of 489 days, 121 (12.9%) patients died. The overall one-year mortality rate was 8%. Two- and three-year mortality rates were 16% and 21%, respectively. Of patients with a known cause of death, the vast majority died due to pulmonary hypertension–related complications.

Each participant was determined to be at low, intermediate, or high risk from PAH using three different standardized measures — the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), the French Pulmonary Hypertension Registry (FPHR), and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL LITE 2.0).

Among low-risk patients, the one-year mortality rate was 1%, two-year mortality rates were 4%–6%, and three-year rates were 7%–11%. For intermediate-risk patients, the mortality rate at one year was 7%–8%, at two years it was 11%–16%, and at three years it was 18%–20%. Among high-risk patients, one-year mortality rates were 12%–19%, two-year rates were 22%–38%, and three-year rates were 28%–55%.

Generally similar results were seen in sub-analyses focused on patients with idiopathic (no identifiable cause) or heritable PAH.

“In an early analysis of this large observational registry, we found that the mortality at 1, 2, and 3 years among intermediate- and high-risk patients with PAH in general, as well as those specifically with idiopathic or heritable PAH, remains unacceptably high,” the researchers concluded.

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