Pitfalls of stenting coarctation of an angulated right circumflex aortic arch in Goldenhar syndrome

Abstract

We report stenting of coarctation of an angulated right circumflex aortic arch (RCAA) using four Cheatham Platinum stents in a child with Goldenhar syndrome. Difficulties in measuring the accurate length of the curved and narrowed transverse aortic arch marked discrepancy between the luminal diameters of the long narrow transverse arch and wide descending thoracic aorta, increased displacement force caused by the 90° bend between the two parts resulted in repeated stent migrations. We discuss the tips to avoid distal stent migration in the setting of an angulated RCAA.

Left aortic arch with right descending aorta and severe coarctation: an unusual “vascular clamp” with airway compression

“…Aortic arch anomalies may be associated with coarctation and hypoplasia. 6,7 They may also coexist with CHD. 1,8 Left pulmonary artery hypoplasia and unilateral lung hypoplasia are also described with circumflex aorta.…”

“…The ductal arch formed by the left sixth arch continued to maintain the descending aortic flows in the postnatal period and led to mild differential cyanosis. Aortic arch anomalies may be associated with coarctation and hypoplasia 6 7 They may also coexist with CHD 1 …”