Aortic coarctation and coronary artery disease: the XY factor.

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.