Alfredo Páez-Carpio, Adeline Y. L. Lim, Alessandro Gasparetto, Michelle Shaw, Felix Ratjen, João G. Amaral
Hospital for Sick Children and University of Toronto.
Canada
Cardiovascular and Interventional Radiology
Cardiovasc Intervent Radiol 2025;
DOI: 10.1007/s00270-025-04322-1
Abstract
Purpose: To assess long-term clinical and radiological outcomes of transarterial embolization (TAE) for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT).
Materials and methods: This retrospective single-center study included 29 pediatric HHT patients (62% male; mean age: 8 [SD: 3]) who underwent TAE for 63 PAVMs in 32 different events between 2004 and 2024. Clinical outcome was peripheral oxygen saturation (SpO2), measured at 1 month, 1, 3, and 5 years. Radiological outcomes included occlusion at final follow-up, persistence, incomplete treatment, reperfusion, and recanalization rates. Regression models were used to identify predictors of SpO2 response and radiological outcomes. Adverse events (AEs) were reported using the CIRSE classification.
Results: Median SpO2 significantly improved from 95% (IQR: 88-97) to 98% (IQR: 95-99) (p < 0.001), 98% (p < 0.001), 98% (p = 0.002), and 97% (p = 0.03) at 1 month, 1, 3 and 5 years. Diffuse disease (p = 0.024, 0.033) and HHT type 1 (p = 0.023) were associated with lower follow-up SpO2. Occlusion rate at final follow-up was 82.5% with persistence, incomplete treatment, reperfusion, and recanalization rates at 28.6%, 7.9%, 19.0% and 6.3%. Size (p = 0.021), diffuse lesion (p = 0.002), and draining vein number (p = 0.002) predicted persistence; diffuse lesion (p = 0.015) and feeding artery number (p = 0.030) predicted reperfusion; feeding artery number (p = 0.046) predicted recanalization; and size (p = 0.004), diffuse lesion (p < 0.001), feeding artery (p = 0.004), and draining vein number (p = 0.001) predicted incomplete occlusion at final follow-up. AE rate was 12.9%, with most events graded as minor.
Conclusion: TAE was effective and safe in pediatric HHT patients with PAVMs, achieving durable occlusion and oxygenation improvement.
Category
Pulmonary Arteriovenous Malformations
Genetic Factors Associated with Pulmonary Vascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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