Long-Term Outcomes of Transarterial Embolization for Pulmonary Arteriovenous Malformations in Pediatric Hereditary Hemorrhagic Telangiectasia
Bibliographic record
Abstract
## Introduction Pulmonary arteriovenous malformations (PAVMs) are a common manifestation of hereditary hemorrhagic telangiectasia (HHT), and may lead to significant morbidity in children. Transarterial embolization (TAE) is the standard treatment, but pediatric-specific long-term data remain limited. This study aimed to assess long-term clinical and imaging outcomes of TAE in pediatric HHT and to identify predictors of oxygenation response and lesion durability. ## Materials and Methods This retrospective cohort included 33 pediatric HHT patients (mean age: 8.3 years) who underwent TAE for 63 PAVMs between 2004 and 2024. Clinical outcome was assessed by peripheral oxygen saturation (SpO₂) at baseline and at 1 month, 1, 3, and 5 years. Lesion-level outcomes included persistence, reperfusion, and recanalization. Regression models were used to identify clinical and anatomical predictors. Technical success, embolic material, number and size of feeding and draining vessels, and adverse events (AEs) were recorded. AEs were classified using the CIRSE system. ## Results TAE had a technical success rate of 96.8%. Median SpO₂ improved from 95% at baseline to 98% at 1 month (p<0.001), and remained at 98% at 1 (p<0.001) and 3 years (p=0.002), and 97% at 5 years (p=0.03). Diffuse disease and HHT type 1 were associated with lower follow-up SpO₂ (p=0.024–0.033 and p=0.023). Lesion persistence, reperfusion, and recanalization occurred in 16%, 19%, and 6%, respectively. Persistence was associated with larger size (p=0.007), diffuse disease (p=0.008), and number of draining veins (p=0.009). Reperfusion was associated with lesion complexity (p=0.015) and number of feeding arteries (p=0.030); recanalization with number of feeding arteries (p=0.046). AE rate was 12%, mostly minor. ## Discussion TAE proved to be effective and safe for managing PAVMs in pediatric patients with HHT, with sustained oxygenation improvement and low complication rates. Most lesions remained occluded over time, and SpO₂ levels improved significantly at early follow-up and remained stable for up to 5 years. Importantly, our study identified diffuse PAVM disease and HHT type 1 as consistent predictors of lower oxygenation at follow-up, even after adjustment for baseline SpO₂. This supports prior observations that diffuse involvement may represent a distinct clinical phenotype associated with worse prognosis. Additionally, we found that lesion size, vascular complexity, and number of draining veins were significantly associated with persistence, while reperfusion and recanalization were linked to lesion complexity and feeding artery count. These findings suggest that anatomical characteristics influence embolization durability and should be considered when planning treatment and surveillance strategies. Although embolization alone may not prevent long-term complications in diffuse cases, early intervention may still offer clinical benefits, particularly in maintaining oxygenation and delaying progression. Our data reinforce the need for tailored follow-up in patients with unfavorable features, and suggest that adjunctive or staged strategies, including surgery, may be required in select cases. Compared with previous pediatric series, our study provides a more granular assessment of outcomes by combining longitudinal SpO₂ analysis and lesion-level modeling, offering clinically relevant insight into which patients are most likely to benefit from treatment and which may require more intensive management.
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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 | 0.001 | 0.002 |
| 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) | 0.001 | 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".