Early and long-term outcomes and their predictors in incident pulmonary arterial hypertension: Results from the Database of Pulmonary Hypertension in Poland (BNP-PL)

BACKGROUND: Contemporary data on pulmonary arterial hypertension (PAH) from Central and Eastern Europe are scarce. AIMS: The aim of this study was to assess clinical characteristics, treatment, survival, and predictors of early improvement and long-term outcomes in newly diagnosed PAH in the BNP-PL database. METHODS: Consecutive patients with newly diagnosed, non-vasoreactive PAH between 2018 and 2023 were included. Changes in 4-strata risk category from diagnosis to the first available follow-up assessment within the first 12 months after diagnosis were analyzed. RESULTS: Among 806 patients, 445 had idiopathic PAH, 176 connective tissue disease-associated PAH, 147 congenital heart disease-associated PAH, and 38 other PAH subtypes. Kaplan-Meier estimated 12-month mortality was 13.5%. During a median follow-up of 16 (8-33) months, mortality was highest in connective tissue disease-associated PAH and lowest in congenital heart disease-associated PAH. First-year clinical improvement occurred in 45.8%. Improvement rates were 34.8% with upfront monotherapy, 49.2% with dual therapy, and 70.5% with triple therapy (P <0.001). After adjustment for baseline 4-strata risk, more initial PAH-specific drugs and fewer cardiovascular comorbidities were associated with clinical improvement. The baseline 4-strata model better discriminated 12-month mortality than the 3-strata model (area under the curve 0.73 vs. 0.63; P = 0.004). Among first-year survivors, worsening risk category, higher baseline risk, and PAH-related unplanned hospitalization within the first year independently predicted long-term mortality. CONCLUSIONS: Early combination therapy and fewer cardiovascular comorbidities were associated with greater early clinical improvement after adjustment for baseline 4-strata risk category. Baseline mortality risk, first-year change in risk category, and unplanned hospitalization were associated with subsequent mortality.