Balloon Pulmonary Angioplasty in Sickle Cell Disease with Chronic Thromboembolic Pulmonary Hypertension
Bibliographic record
Abstract
Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious complication of sickle cell disease (SCD). Patients with SCD often present with an inoperable form of CTEPH. In such cases, balloon pulmonary angioplasty (BPA) has emerged as a promising therapeutic option. Methods: This retrospective study evaluates BPA outcomes in SCD-associated CTEPH patients treated at the French National Reference Center for Pulmonary Hypertension from 2014 to 2023. Results: Fourteen SCD-CTEPH patients (10 HbSC, 3 HbSS, 1 S-β0 thalassemia) underwent BPA, 13 for inoperable lesions and 1 for persistent pulmonary hypertension (PH) post-endarterectomy. Pre-BPA, 10 patients received PH therapy. Significant functional and hemodynamic improvements were observed post-BPA (Table), leading to therapy reduction or discontinuation in 4 of 10. Compared to the broader CTEPH cohort (507 BPA-treated patients) during the same period, the hemodynamic response to BPA was comparable between groups (ΔPVR: 1.39 [0.8–2.6] vs. 2.6 [1.1–4.8]; p=0.091), and complication rates remained similar. Conclusion: BPA improved hemodynamics and exercise capacity in SCD-CTEPH patients, supporting its role as a viable treatment for inoperable cases. erj;66/suppl_69/PA920/TB1 T1 TB1 Variables Pre – BPA Median (IQR 25-75%) Post-BPA Median (IQR 25-75%) p value mPAP mmHg Cardiac Index (L/min/m2) PVR, WU 38 (30-47) 3.56 (2.86-3.86) 3.8 (3.0-6.6) 32 (22-37) 3.53 (3.09-4.39) 2.6 (1.6-3.8) 0.042 0.42 0.0016 6MWD, m WHO-FC I-II / III-IV 400 (355-443) 7 (50%)/7 (50%) 473 (431-512) 12(85%)/2 (15%) 0.008 0.1 Hemoglobin, g/dL 9.9 (9.3-10.8) 9.9 (9.1-10.4) 0.126 erj;66/suppl_69/PA920/TB2 T2 TB2 Group Non-SCD (N=507) SCD (N=14) p value BPA-related complications 195 (38.4%) 6 (42.9%) 0,470
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
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 | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 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".