Angiography and Prognosis of Bronchial Artery Malformations in Children with Pulmonary Hemorrhage of Unknown Origin

Background Pediatric pulmonary hemorrhage of unknown origin (PHUO) is rare but can cause severe morbidity. Idiopathic pulmonary hemosiderosis (IPH) is the primary diffuse PHUO, often recurring despite immunosuppressants. Bronchial artery embolization (BAE) has shown potential in treating hemorrhage associated with bronchial artery morphological abnormalities (BAMAs), but pediatric data are limited. Objective To assess the effectiveness of BAE and the prognostic impact of BAMAs on pediatric PHUO outcomes. Materials and Methods A retrospective cohort of 47 children (median age 47 months; 64% female) with PHUO refractory to medical treatment underwent bronchial arteriography at a tertiary Children's Hospital (March 2019–May 2024). The Anderson-Gill intensity model analyzed the relationship between BAMAs and post-BAE recurrence. Results Of 47 patients, 40 patients (85%) were diffuse PHUO (diagnosed as idiopathic pulmonary hemosiderosis [IPH]), and 7 patients (15%) were focal PHUO. All patients had bronchial artery morphological abnormalities, with the most common being hypervascularity (46 [98%]), followed by vascular dilation and tortuosity (38 [81%]), bronchial-pulmonary shunting (BPS) (19 [40%]), and contrast extravasation (17 [36%]). Clinical success after BAE was achieved in 41 patients (87%), of which 7 were focal PHUO and 34 were IPH. Fifty-eight recurrence events occurred in 29 patients at a median follow-up of 28 months (range, 2–60 months). On multivariable survival analyses, BPS and contrast extravasation were associated with lower recurrence rates in IPH patients after BAE (hazard ratio 0.36 [95% CI, 0.21–0.61] and 0.50 [0.24–0.90], respectively). Conclusion BAE demonstrates high success in pediatric PHUO, with BPS and contrast extravasation indicating favorable prognostic outcomes.