Add-on therapy with parenteral prostacyclin analogues in patients with pulmonary arterial hypertension: Insights from the COMPERA registry
Bibliographic record
Abstract
Parenteral prostacyclin analogues (PPA) are recommended for pulmonary arterial hypertension (PAH) patients at intermediate-high or high risk, yet supporting evidence remains limited. We retrospectively analyzed pretreated patients with idiopathic/heritable/drug-associated PAH (I/H/D-PAH), connective tissue disease-associated PAH (CTD-PAH), or congenital heart disease-associated PAH (CHD-PAH) from the COMPERA registry who received add-on PPA therapy. Among 495 patients, all pretreated with ≥1 PAH medication, add-on PPA treatment was associated with improvements in 6-minute walk distance, WHO functional class, and NT-proBNP across groups. However, mortality was high: Kaplan-Meier survival estimates at 5 years were 59% in I/H/D-PAH, 59% in CHD-PAH, and 31% in CTD-PAH. Independent predictors of mortality included older age, male sex, CTD, CHD, and intermediate-high or high risk at time of PPA initiation. These findings indicate short-term clinical improvements but high subsequent mortality with add-on PPA therapy in patients with PAH, particularly in those with CTD-PAH, underscoring the need for more effective therapies.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.003 | 0.005 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.001 | 0.002 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".