Chang KY, et al. Come Together – Clinical Advances in Pulmonary Hypertension: Lessons from Best Abstracts. Presented at: American Thoracic Society International Conference; May 14-19, 2021 (virtual meeting).
Chang reports no relevant financial disclosures.
In the U.S., mortality trends in patients with pulmonary arterial hypertension continue to improve; however, rates remain high in those with intermediate- and high-risk characteristics, researchers reported.
“Currently, the mortality data for patients with PAH in the United States are based on registries that enrolled patients over a decade ago,” Kevin Y. Chang, MD, internal medicine resident in the division of cardiology at the University of Minnesota, said during a presentation at the American Thoracic Society International Conference. “With recent advancements in PAH-specific therapies, as well as a paradigm shift in how we treat PAH, there is a need to assess contemporary mortality rates in the United States.”
The researchers identified 935 patients with Group 1 PAH (mean age, 55 years; 76% women; 77% white; mean BMI, 29.5 kg/m2) from the Pulmonary Hypertension Association Registry, a currently-enrolling registry, prospective, multicenter registry that was launched in 2015. Forty percent of patients had idiopathic PAH and 32% had connective tissue disease-associated PAH. At baseline, the mean 6-minute walk distance was 335 m, 50% were in NYHA functional class III, and use of PDE5 inhibitors (88%) and endothelin receptor antagonists (76%) was high.
Researchers assessed 1-, 2- and 3-year mortality rates and mortality rates after stratifying patients based on time since PAH diagnosis.
During a median follow-up of 489 days, there were 121 deaths (12.9% of cohort), with 72% having a known cause, including 68 deaths from cardiopulmonary causes.
Among all patients with PAH, the mortality rate was 8% at 1 year, 16% at 2 years and 21% at 3 years, Chang said during the presentation.
When the researchers stratified by time since PAH diagnosis, they found no difference in mortality between those diagnosed with PAH less than 6 months from the time of enrollment and those diagnosed at least 6 months before enrollment (P = .59).
In addition, the researchers looked at PAH mortality by risk stratification: low risk (1-2% at 1 year, 4-6% at 2 years, 7-8% at 3 years), intermediate risk (7% at 1 year, 11-15% at 2 years, 18-20% at 3 years) or high risk (12-19% at 1 year, 22-37% at 2 years, 28-47% at 3 years).
Compared with mortality data from other PAH registries, such as COMPERA and REVEAL LITE 2.0, results suggested overall lower mortality rates in the current era, Chang said.
“Our study showed that mortality trends among patients diagnosed with PAH in the U.S. continue to improve,” Chang said. “However, mortality among those with intermediate- and high-risk characteristics remain unacceptably high, suggesting the importance of early diagnosis, aggressive utilization of available therapies to delay disease progression and a continued need for better therapeutics.”