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Congenital heart disease
The adult with repaired coarctation of the aorta
  1. Eric Krieger1,
  2. Karen Stout2
  1. 1Boston Adult Congenital Heart Service, Children's Hospital Boston, Boston, USA
  2. 2Director Adult Congenital Heart Service, University of Washington, Seattle, USA
  1. Correspondence to Dr Eric Krieger, Boston Adult Congenital Heart Service, Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA; eric.krieger{at}cardio.chboston.org

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Coarctation of the aorta (CoA) is a common congenital cardiovascular defect which affects approximately 1 in 3000 live births. In the past, CoA was considered to be a simple congenital abnormality with a surgical cure. However, long term follow-up has shown that many patients with repaired CoA have substantial morbidity and premature mortality.1 w1 It is now clear that CoA is not simply an anatomic narrowing which can be cured by surgical repair or percutaneous dilation. Rather, CoA is a diffuse arteriopathy characterised by widespread perturbation in vascular structure and function which manifests as hypertension, premature cardiovascular disease, and aneurysmal disease. Each of these manifestations has an impact on the management of the adult with repaired CoA.

History

In the pre-surgical era, the prognosis of CoA was poor. The mean age of death was 34 years and only 5% of patients were alive at 60 years of age. The causes of death were predominantly related to uncontrolled hypertension: congestive heart failure (25%), aortic rupture (21%), and intracranial haemorrhage (12%).w2

Surgical palliation for CoA has been successfully performed for more than 60 years. Since the initial surgery, many authors have demonstrated that repair can be performed with low mortality, and that repair results in excellent haemodynamics and improvement in associated hypertension.w3 w4 There ensued a period of enthusiasm when it was believed that an anatomic repair would lead to normal longevity. Unfortunately, despite excellent anatomic repairs, late complications remain common and lifespan may be shorter than expected. In 1973, Maron et al reported on 248 patients with repaired CoA who were followed for up to 25 years after surgical repair. There was a 12% mortality with an average age at death of 34 years—an age which did not differ significantly from the preoperative era.w1 w2 In another series of 571 patients with repaired …

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