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2.1.10 Adults with congenital aortic isthmus stenosis
Marcus Rebel, Ali Dodge-Khatami, Ali Aydin, Yskert von Kodolitsch
2.1.10.1 Clinical pictures and epidemiology
Congenital aortic isthmus stenosis is also known as aortic coarctation and is a classic clinical picture in pediatric medicine. In most cases, adults with congenital aortic isthmus stenosis have undergone surgical correction while they were children. However, adults with corrected aortic isthmus stenosis have a number of medical problems and a first diagnosis of aortic isthmus stenosis is often only made in adults. This section aims to describe the specific vascular-medicine problems that arise in adults with corrected aortic isthmus stenosis or with a late first diagnosis of the condition. This approach is in accordance with current efforts to treat the situation faced by adults with congenital cardiac defects as a separate medical challenge (Schmaltz et al. 2008).
Aortic isthmus stenosis is defined as a congenital narrowing of the aorta in the area of the distal aortic arch, up to the start of the thoracic aorta at the level of the origin of the left subclavian artery and orifice of the ductus arteriosus; the isthmus is a physiological narrowing of the aorta in this area. The ductus arteriosus is the fetal “diversion” of the pulmonary circulation and links the pulmonary trunk with the aorta; within a few days to weeks of birth, it closes physiologically and regresses to a connective-tissue structure, the ligamentum arteriosum.
With an incidence of 0.2–0.6 per 1000 live births, aortic isthmus stenosis represents 5–8% of all congenital cardiac defects (Brickner et al. 2000; Krieger and Stout 2010).