Circulation: Cardiovascular Imaging. 2008;1:e4-e6
doi: 10.1161/CIRCIMAGING.108.767947
Coarctation of the Abdominal Aorta
An Uncommon Cause of Arterial Hypertension and Stroke
Fernando Daghero, MD
;
Nora Bueno, MD
;
Alejandro Peirone, MD
;
José Ochoa, MD
;
Gustavo Foa Torres, MD
and
Javier Ganame, MD, PhD
From Pediatric Cardiology (F.D., N.B.) and the Radiology Department (J.O.), Hospital Infantil; Pediatric Cardiology (A.P.), Hospital Privado; and the Radiology Department (G.F.T.), Instituto Oulton, Cordoba, Argentina; and the Cardiology Department (J.G.), University Hospitals Leuven, Belgium.
Correspondence to Javier Ganame, Cardiology Department, University Hospitals Leuven, Herestraat 49, B-3000, Leuven, Belgium. E-mail javier.ganame{at}uzleuven.be
A 14-year old male with no previous medical history presented with headache, left third cranial nerve palsy, and generalized tonic-clonic seizures. Magnetic resonance imaging of the brain revealed subarachnoid hemorrhage secondary to an aneurysm of the left posterior communicating artery. The patient underwent successful transcatheter coil occlusion of the aneurysm, and his neurological symptoms improved (Figure 1).
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Figure 1. A, Cerebral angiogram demonstrating a large ruptured aneurysm (arrow) of the posterior communicating artery. B, Angiogram after endovascular coil embolization showing exclusion of the aneurysm.
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During admission, he remained hypertensive, with an arterial
blood pressure of 170/96 mm Hg in the upper limbs despite medical
treatment with atenolol, amlodipine, and enalapril. On physical
examination, an abdominal systolic bruit was heard, and weak
femoral pulses were noted. Blood pressure in the right and left
arms was similar, but there was a significant differential pressure
(50 mm Hg) compared with the lower limbs. Aortic coarctation
and renal artery stenosis were considered to be the causes of
arterial hypertension.
An echocardiogram showed moderate left ventricular hypertrophy and preserved systolic function. No abnormality in the aortic arch or proximal descending aorta was noted on echocardiography. An abdominal ultrasound showed a small abdominal aorta with diffuse narrowing and increased peak systolic velocity (509 cm/s). Presence of runoff flow throughout the entire diastole was also noted (Figure 2). Magnetic resonance angiography of the aorta confirmed the diagnosis of coarctation of the abdominal aorta and showed a narrowing of considerable length starting 5 cm below the diaphragm. This narrowing tapered to become a critical stenosis at the level of origin of the renal arteries (Figure 3). The abdominal aorta measured 2.8 mm at its smallest point and was consistently poorly opacified. Prominent collateral circulation from the mammary artery and the intercostal arteries to the abdomen was present.
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Figure 2. A, Abdominal ultrasound showing a prolonged narrowing of the abdominal aorta. B, Continuous Doppler signal showing peak velocity of 500 cm/s and runoff throughout the entire diastole.
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Figure 3. Magnetic resonance angiography of the aorta in sagittal (A) and coronal (B) planes showing a diffuse narrowing of the abdominal aorta that involved the origin of the celiac trunk, superior mesenteric artery, and both renal arteries. The stenosis was 5 cm long. The diameter of the aorta proximal to the stenosis was 10 mm; the minimum diameter of the aorta was 3 mm. Note the prominent mammary artery providing collateral circulation to the abdomen (upper arrow).
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At cardiac catheterization, there was a pullback gradient of
56 mm Hg from the ascending aorta to the descending abdominal
aorta under general anesthesia. Balloon angioplasty of the stenotic
segment was performed. Significant improvement in aortic and
renal arteries diameters was noted after the procedure. An aortogram
immediately after dilatation showed good opacification of the
entire abdominal aorta (
Figure 4). The gradient fell to 10 mm
Hg. The patient has remained normotensive since the procedure.
Coarctation of the abdominal aorta, also known as middle aortic
syndrome or mid-aortic dysplastic syndrome, is a clinical condition
caused by segmental narrowing of the abdominal or distal descending
thoracic aorta secondary either to a congenital anomaly in the
development of the abdominal aorta or to one of several acquired
conditions. Acquired conditions include neurofibromatosis, retroperitoneal
fibrosis, fibromuscular dysplasia, mucopolysaccharidosis, and
Takayasu’s arteritis, all of which may result in narrowing
of the abdominal aorta and other vessels.
1,2 In Takayasu’s
arteritis, the use of antiinflammatory agents may be useful.
This case highlights (1) the importance of a thorough physical examination, which in this case led to the discovery of an abdominal bruit and weak femoral pulses that raised the suspicion of aortic coarctation or renal artery stenosis as the cause of hypertension; (2) the association between anomalies of the aorta and cerebral arteries aneurysms (it has been reported that up to 10% of patients with aortic coarctation have cerebral aneurysms3); and (3) that coarctation of the abdominal aorta, although rare (2% of all coarctations of the aorta) can be the cause of secondary hypertension and should be considered during the diagnostic workup of hypertension, especially in young patients.
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Disclosures
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None.
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References
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1. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment.
Perspect Vasc Surg Endovasc Ther. 2005; 17: 187–203.
[Abstract/Free Full Text]2. Connolly JE, Wilson SE, Lawrence PL, Fujitani RM. Middle aortic syndrome: distal thoracic and abdominal coarctation: a disorder with multiple etiologies. J Am Coll Surg. 2002; 194: 774–781.[CrossRef][Medline]
3. Connolly HM, Huston J III, Brown RD Jr, Warnes CA, Ammash NM, Tajik AJ. Intracranial aneurysms in patients with coarctation of the aorta: a prospective magnetic resonance angiographic study of 100 patients. Mayo Clin Proc. 2003; 78: 1491–1499.[Abstract/Free Full Text]
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Disclosures
References