Objective: To investigate the incidence of clinical problems related to a bicuspid valve (aortic stenosis and regurgitation) and the incidence of ascending aorta and aortic arch pathology in combination with coarctation repair. Patients: 124 adult patients after surgical correction of aortic coarctation were studied. The incidence of aortic valve, ascending aorta, and aortic arch pathology was determined using echocardiography and magnetic resonance imaging. The median age at coarctation repair was 9 years and at last follow up 28 years. Results: Three patients died from aorta pathology. Aortic valve disease was found in 63% of the patients, requiring an intervention in 22%, at a median of 13 years after coarctation repair. Ascending aorta dilatation was observed in 28% and aortic arch abnormalities in 23%, among whom kinking of the aortic arch was found in 12%. Antihypertensive medication was used in 24%. In the patients with hypertension the age at operation and age at follow up were significantly higher (p = 0.0001 and p < 0.0001, respectively). Conclusion: In addition to the well known problems of hypertension and recoarctation, aortic valve and aortic arch pathology are commonly encountered in patients with previous coarctation repair. Aortic abnormalities may predispose to dilatation and dissection, thus necessitating careful lifelong attention in all patients with coarctation. C oarctation of the aorta accounts for 8% of congenital heart disease. In the past, coarctation was considered to be a simple "correctable lesion" and surgery, first performed in 1944, was believed to be curative. In keeping with this belief, normotensive patients were often discharged postoperatively without long term follow up. However, as increasing numbers of patients with surgically corrected coarctation reached maturity, it has become clear that almost a third of patients become or remain hypertensive, despite correction of their lesion, with an increased risk of accelerated atherosclerosis and end organ damage.1 Furthermore, recoarctation occurs in 3-35%.
2-4The association of a bicuspid aortic valve with coarctation of the aorta is well recognised, but estimates of bicuspid valve in patients with coarctation range from 25-85%.2 5 The clinical significance of a bicuspid valve in patients with coarctation is not well established.To address these issues, we retrospectively studied patients after coarctation repair seen in our department for adult congenital heart disease with emphasis on both aortic valve and aortic arch pathology.The specific purpose of this study was, firstly, to assess the prevalence of aortic stenosis or regurgitation in these patients; secondly, to assess how often dilatation of the ascending aorta and pathology of the aortic arch occur in patients with coarctation; and thirdly, to assess a possible correlation of aortic arch pathology with hypertension or recoarctation.
METHODSAll patients seen in our department for adult congenital heart disease with surgically corrected coarctation were reviewed. They w...