Aortic Coarctation and Coronary Artery Disease

Abstract

Aortic coarctation (CoA) is a congenital defect involving a constriction of the aortic arch, usually occurring near the site of insertion of the ductus arteriosus. Surgical and endovascular repair techniques and success rates, measured by short-term survival, elimination of the gradient across the stricture, and normalization of systemic blood pressure, improved steadily over the last half of the 20th century. Despite apparently successful correction of the obstruction, however, individuals with a history of CoA demonstrate excess morbidity and premature mortality associated with hypertension (HTN), cerebrovascular accident, coronary artery disease, and aortic dissection/rupture.1–3 These adverse outcomes are to some extent independent of the severity of the original obstruction, type of treatment, restenosis, or presence of prosthetic grafts.2,4 Whereas blood pressure usually normalizes for a time after successful repair, ≈one third of CoA patients develop HTN by adolescence5 and ≈90% by middle age.2 The pathogenesis of the later onset HTN remains poorly understood.6 Normotensive children and young adults who had undergone successful CoA repair were found to have persistent endothelial dysfunction7 and impaired arterial reactivity,8 suggesting that intrinsic vascular abnormalities might contribute to the risk for premature coronary artery disease (CAD), independent HTN. Article see p 16 In this issue of Circulation , a well-designed cross-sectional study from McGill University specifically addresses the CAD risk in CoA.9 Roifman et al9 identified 756 individuals diagnosed with CoA and 6471 with ventriculoseptal defect between the years 1983 and 2005 from Quebec's Congenital Heart Disease Database. They compared the rate of cardiovascular diagnoses in age-matched CoA and ventriculoseptal defect cohorts (median age 30 years). The CoA group had significantly greater rates of HTN (45% versus 16%), CHF (15% versus 7%), and stroke (5.5% versus 2.6%; P <0.0001 for all). CoA patients also …