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Coarctation of the aorta was described pathologically in the 1700s and clinical recognition occurred from the early 1900s.w1 w2 Postmortem data suggested a median age of death of 31 years—mainly from complications of the coarctation (cardiac failure in 26%, aortic rupture in 21%, bacterial endarteritis in 18%, and intracranial haemorrhage in 12%.w3 w4 The presurgical “natural” history data were similar accepting the case selection bias prevalent in that era.w5 Surgical repair was first described in 1945, balloon dilation in 1982, and stent implantation in 1991.w6–8
Currently, the clinical diagnosis (murmur, upper limb hypertension, and absent or diminished femoral pulses) can rapidly be confirmed non-invasively by echocardiography and magnetic resonance imaging (MRI), and monitored by these techniques following treatment (fig 1). Radio-femoral delay is a poor marker for follow up. 1w9 The vast majority of coarctations are identified and treated in the first year of life and adult presentation is becoming less frequent.
Coarctation of the aorta accounts for approximately 7% of all live births with congenital heart disease and is 1.7 times more common in males.w10 w11 Hypoplasia of the aortic arch to various degrees is a common accompanying feature.w12 There is a significant association with other cardiac lesions (ventricular septal defect, patent arterial duct, and bicuspid aortic valve (in up to 50%) commonly but also with complex defects); in some the prognosis depends more on the outcome of the associated lesions which requires simultaneous management with the coarctation while in others the associated lesion is mild and can be treated on its own merits. In Turner’s syndrome (46 XO) coarctation is present in 10% of …
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