Late complication rates after aortic coarctation repair in patients with or without a bicuspid aortic valve

Abstract

ObjectivePatients with previously repaired aortic coarctation (CoA) are at risk of developing late surgical complications. Many patients with CoA also have a bicuspid aortic valve (BAV). We sought to determine in patients with repaired CoA whether the presence of BAV is associated with more cardiovascular reinterventions during follow-up.MethodsAdults with previously repaired simple CoA were recruited from our Adult Congenital Heart Disease database (Sydney, Australia). The incidence of complications relating to the ‘CoA-site’ (descending aortic aneurysm or dissection, or recoarctation) and the ‘AV/AscAo’ (aortic valve or ascending aortic pathology) that required intervention was compared between those with BAV (‘CoA-BAV’) and without BAV (‘CoA-only’).ResultsOf 146 patients with repaired CoA, 101 (69%) had BAV. Age at CoA repair was similar (median 6.0 (IQR 0.5–14.0) years vs 5.0 (IQR 0.5–11.0) years, p=0.44), as was the distribution of repair types, with end-to-end repair the most common in both groups (45.9% vs 45.6%). At a median of 28 years following initial repair, a significantly higher proportion of patients with CoA-BAV required cardiovascular reintervention (45.5% vs 20.0%, p=0.003). Whereas ‘CoA-site’ complications were more common than ‘AV/AscAo’ complications in patients with CoA only (13.3% and 0%, respectively), patients with CoA-BAV had a high prevalence of both ‘CoA-site’ as well as ‘AV/AscAo’ complications (19.8% and 21.8%, respectively). Overall survival was similar (p=0.42).ConclusionIn adults with repaired CoA, patients with CoA-BAV are more than twice as likely to require cardiovascular reintervention by early-to-mid-adult life compared with those with CoA alone. Despite this, no difference in survival outcomes was observed.

Coarctation of the Aorta: Diagnosis and Management

“…BAV in itself is associated with a distinct aortopathy characterised by cystic medical necrosis and a higher risk of aneurysm formation. CoA and BAV therefore interact significantly to enhance the risk of aortic aneurysm formation in the ascending aorta, with aneurysmal dilatation of the ascending aorta being as common as CoA site complications in these patients [ 75 , 76 ]. Ascending aorta aneurysms are twice as likely to develop than those with isolated CoA.…”