Unmasking a Bilateral Patent Ductus Arteriosus with Isolation of the Right Subclavian Artery after Device-Closure of a Left-Sided PDA as a Rare Case in Situs Inversus with Left-Sided Aortic Arch

Background: Isolation of the subclavian artery (ISA) is a rare finding usually associated with further cardiac anomalies. To our knowledge there are no reported cases of bilateral PDA and ISA in situs inversus.

Method: Case report: A 3-month-old boy with a body weight of 3 kg with situs inversus and cardiac congestion was referred for further preprocedural assessment. Echocardiography and cardiac catheterization displayed a situs inversus totalis, AVSD Rastelli type C, ASD II, V, azygos continuation, RSVC, LSVC, connection of the liver veins to the left-sided right atrium, and left-sided aortic arch with a left-sided PDA. Intraprocedural decision for PDA-closure, prior to implantation of a pacemaker due to congenital 2:1 AV block and central PA banding, unmasked an additional right-sided PDA with isolation of the right subclavian artery (right ISA).

Conclusion: A bilateral PDA, occurring in association with aortic arch anomalies, is very rare and even in these anomalies ISA is uncommon. Case reports of an ISA are scarce and mainly comprise a left-sided ISA with a right aortic arch. Our case of situs inversus shows the mirror image variant with a right ISA and a left aortic arch. Bilateral PDA and ISA may be explained by Edward hypothetical double aortic arch plan. Embryologic involution would occur on two levels: at the distal right dorsal aorta, creating a connection between the right subclavian artery and the pulmonary artery via the ductus arteriosus, and regression between the right common carotid artery and the right subclavian artery, isolating the right subclavian artery from the left-sided aorta.

Publication History

Article published online:
28 January 2023