Despite successful surgical repair, aortic coarctation is associated with unfavourable prognosis mainly due to cardiovascular disease. Late timing of repair and arterial hypertension represent adverse prognostic factors. Arterial hypertension can recur after coarctation repair, despite the absence of residual obstruction, with a prevalence of up to 45%. Furthermore, even subjects with normal blood pressure values at rest may show an abnormal blood pressure elevation during exercise and daily life activities. The pathophysiology of such abnormal blood pressure behaviour is unclear. Different mechanisms have been proposed: resetting of the renin-angiotensin system, neurological dysfunction and impaired vascular reactivity and/or elastic properties. Several studies have supported these hypotheses, although the suggestion of a causative role of vascular dysfunction persisting late after coarctation repair has recently become more popular. Further studies are needed to investigate this issue; this particular syndrome may represent an important study model for the understanding of systolic hypertension.