Anomalous Vascular Supply of Bronchial Circulation in Cystic Fibrosis Patients with Massive Hemoptysis

Background: Hemoptysis occurs commonly in patients with Cystic Fibrosis, and massive bleeding requiring emergent intervention occurs in a subset. Bronchial artery embolization (BAE) is recommended in clinically unstable CF patients with massive hemoptysis (MH); however, no consensus exists regarding selective versus extensive embolization, and recommendations of targets are scant. Objective: We sought to characterize the frequency of abnormal bronchial circulation in MH in CF in order to suggest interventional approach for BAE. Methods: A retrospective chart review of BAE for MH was performed. Characterization of angiography was described, and CF and non-CF patients were compared. Results: 10 CF and 7 non-CF patients with MH were identified. Dilated, tortuous bronchial vessels were ubiquitous in CF, and 70% had non-bronchial collaterals. CF patients required more extensive BAE and 50% had >1 collateral. Anomalous supplies were described, and a correlation between the volume of hemoptysis and number of collaterals was determined. Conclusion: Anomalous bronchial arteries origin and supply should be suspected in CF. Angiographic approach in CF should include arch aortograms to demonstrate entire supply of the bronchial arterial field.