Early and long-term outcomes and their predictors in incident pulmonary arterial hypertension: Results from the Database of Pulmonary Hypertension in Poland (BNP-PL).
Contemporary data on pulmonary arterial hypertension (PAH) from Central and Eastern Europe are scarce.
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.
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.
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.
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.
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.
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.
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.
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.
Authors
Waligóra Waligóra, Kurzyna Kurzyna, Mularek-Kubzdela Mularek-Kubzdela, Skoczylas Skoczylas, Chrzanowski Chrzanowski, Błaszczak Błaszczak, Jaguszewski Jaguszewski, Kuśmierczyk Kuśmierczyk, Ptaszyńska Ptaszyńska, Grześk Grześk, Mizia-Stec Mizia-Stec, Malinowska Malinowska, Peregud-Pogorzelska Peregud-Pogorzelska, Lewicka Lewicka, Tomaszewski Tomaszewski, Barańska-Pawełczak Barańska-Pawełczak, Gąsior Gąsior, Pawlak Pawlak, Betkier-Lipińska Betkier-Lipińska, Pruszczyk Pruszczyk, Widejko Widejko, Winowska-Józwa Winowska-Józwa, Smukowska-Gorynia Smukowska-Gorynia, Florczyk Florczyk, Kopeć Kopeć
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