Abstract
An active 29-year-old man was referred to our institution for a severe hypertension (170/100 mmHg) despite monotherapy with nicardipine. Physical examination documented symmetric blood pressure with a normal pulse examination. Transthoracic echocardiography showed normal left ventricular function without significant hypertrophy, a tricuspid aortic valve and a normal ascending aorta. He underwent an enhanced computed tomography angiography (CTA) to rule out renal artery stenosis or adrenal pathology (normal renal arteries; Panel A: asterisk). Three-dimensional CTA reconstructions revealed widespread abdominal arterial collaterals and a relatively small abdominal aorta (Panels A and B) measuring 17 mm. As the abdominal collateral arterial network appeared connected to thoracic arterial collateral and in particular the left epigastric artery (Panels A, B and C: arrows), we suspected an aortic coarctation (AC).
Therefore, we performed aortic magnetic resonance angiography (MRA) using a 1.5 T scanner (GE, USA), which confirmed a native AC with severe stenosis estimated at 75% in diameter (Panels D and F: arrowheads). No other indications of congenital heart disease were identified. Blood flow analysis of 4D flow MR phase contrast sequences using Arterys™ software (Arterys Inc., USA) demonstrated an abnormal decrease of systolic flow in the descending aorta compared to the ascending aorta. It also revealed high-velocity systolic–diastolic flows across the stenosis, with post-stenotic helical streamlines in the descending aorta (Panels E and F). Interestingly, 4D flow imaging documented enhanced flow in the left internal thoracic artery (Panel E: arrow).
AC is a frequent congenital cardiovascular disease accounting for 6–8% 1 of all congenital cardiovascular malformations. It is defined as a narrowing or constriction of the lumen of the aorta. Clinical presentation of affected patients depends on the site and extent of obstruction and associated cardiovascular anomalies. AC is typically diagnosed in early childhood, although identification may be delayed until adulthood when efficient compensatory collateral vessels exist. Survival to older age is rare owing to severe cardiovascular complications 2 such as heart failure, coronary artery disease, aortic rupture/dissection, concomitant aortic valve disease, infective endarteritis/endocarditis, or cerebral hemorrhage. The European Society of Cardiology guidelines recommend treatment with endovascular stenting if the non-invasive pressure difference is 20 mmHg with or without symptoms. 3
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Footnotes
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The authors received no financial support for the research, authorship, and/or publication of this article.