Background: A 20-year-old male presented with a history of systemic hypertension. Examination revealed a systolic murmur with an early ejection click, and femoral pulses were markedly reduced.

Investigations: Physical examination, laboratory testing, electrocardiography, chest radiography, comprehensive echocardiography including pulsed-wave Doppler examination, and CT of the chest.

Diagnosis: Severe coarctation of the juxtaductal aorta accompanied by an ascending aortic aneurysm, a bicuspid aortic valve without evidence of hemodynamically significant stenosis or regurgitation, and an atrial septal defect.

Management: An ascending-descending intrapericardial aortic bypass graft, atrial septal defect closure, and ascending aorta replacement were all successfully performed. Lifelong follow-up will be required.

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http://dx.doi.org/10.1038/ncpcardio1258DOI Listing

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