Mortality in Pulmonary Arterial Hypertension in the Modern Era: Early Insights from the Pulmonary Hypertension Association Registry
Year: 2021
Type: conference-abstract
Abstract: Introduction:Current mortality data for pulmonary arterial hypertension (PAH) in the US are based on registries that enrolled patients prior to 2010. However, there have been many advancements in PAH therapeutics and development of evidence-based treatment paradigms in the last decade. Thus, we sought to determine mortality in PAH in the modern era using the Pulmonary Hypertension Association Registry (PHAR), which is a prospective, multicenter pulmonary hypertension registry in the US enrolling patients since 2015. Methods:We identified all adult patients with PAH enrolled in the PHAR between September 2015-2020 (N=935). Baseline demographics, disease characteristics and hemodynamic data were collected at enrollment. We used Kaplan-Meier survival analysis to assess mortality at 1-, 2-and 3-years. Patients were stratified based on prevalent vs. incident cases (diagnosed within 6 months of enrollment in PHAR) as well as disease severity by validated risk scores (REVEAL LITE 2.0, COMPERA and FPHR). Results:The mean age was 5516 years and 76% were women. Etiology of PAH was as follows: idiopathic (41%), heritable (3%), connective tissue disease (32%), drug-induced (11%), portopulmonary hypertension (6%), congenital heart disease (4%), HIV (2%), and pulmonary veno-occlusive disease (1%). Of the 935 patients, 483 (52%) were incident cases. There were 121 deaths (12.9%) during a median follow-up time of 489 (281-812) days. The 1-, 2-and 3-year mortality for all PAH were 8% (95% CI: 6-10%), 16% (95% CI: 13-19%) and 21% (95% CI: 17-25%), respectively. In patients with idiopathic and heritable PAH, the 1-, 2-, and 3-year mortality was 7% (95% CI: 5-10%), 17% (95% CI: 12-22%) and 20% (15-26%), respectively. There was no difference in mortality between incident vs. prevalent cases both in our PAH cohort (p=0.59) and in patients with idiopathic/heritable PAH (p=0.46). When stratified into low-, intermediate-and high-risk PAH by validated risk scores, the mortality at 1-, 2-and 3-years were 1-2%, 4-6% and 7-8% for low-risk, 7%, 11-15% and 18-20% for intermediate-risk, and 12-19%, 22-37% and 28-47% for high-risk, respectively. Similar trends were observed in patients with idiopathic and heritable PAH when stratified by risk categories. Conclusion:Mortality in PAH in the US continues to improve and is better now than compared to previous decades, similar to what was observed by the European registries (Figure). While overall survival is better, mortality in the intermediate and high-risk groups remain unacceptably high, suggesting the importance for early diagnosis, aggressive utilization of available therapies and the need for better therapeutics.
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
OpenAlex ID: W4231844486