Fate of the Arterial Origin of Major Aortopulmonary Collateral Arteries After Unifocalization

BACKGROUND: During unifocalization procedures for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, collateral arteries are either ligated or detached. Not much is known of the fate of the remaining arterial origins in the long term. Available computed tomography (CT) or magnetic resonance (MR) imaging of the intrathoracic arteries was examined to investigate possible abnormalities at the former position of the collateral arteries as well as ascending aortic diameters. METHODS: From 1989 to 2018, we performed 66 unifocalization procedures in 39 patients. One hundred and twenty-nine collateral arteries were ligated or detached. In 52% (15) of the surviving patients (with a total of 55 ligated or detached collaterals), sufficient imaging of the thoracic aorta from CT (11) and/or MR (9) was available for evaluation. RESULTS: The median interval between unifocalization procedure and imaging was 15 years (interquartile range [IQR]: 9-19 years). In 93% (14) of the scanned patients, 18 blunt ends were detected at the location of a former collateral artery. No aneurysm formation of the descending aorta was observed. The median diameter of the ascending aorta was 35 mm (IQR: 31-40 mm). During follow-up, no aortic dissection or rupture occurred. CONCLUSIONS: Aortic imaging late after unifocalization showed abnormalities in 93% of the scanned patients. Abnormalities consisted mostly of blunt ends of the former collateral artery. We recommend to include routine imaging of the aorta during late follow-up to detect eventual future abnormalities and monitor aortic diameters. Ascending aortic diameters showed slight dilatation with no clinical implications so far.