Long-Term Outcomes of Transarterial Embolization for Pulmonary Arteriovenous Malformations in Pediatric Hereditary Hemorrhagic Telangiectasia
Notice bibliographique
Résumé
## 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.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,002 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,001 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».