British Journal of Radiology (2004) 77, 335-337
© 2004 British Institute of Radiology
doi: 10.1259/bjr/84233974
Coarctation of the aorta associated with aneurysm of the left subclavian artery
N Hiller, MD
A Verstanding, MD
and
N Simanovsky, MD
Department of Radiology, Hadassah University Hospital, Jerusalem, Israel
 |
Abstract
|
|---|
Coarctation of the thoracic aorta is not often diagnosed after childhood. The combination of subclavian artery aneurysm in association with coarctation of the aorta is even less frequent. A review of the literature elicited only seven previously reported cases of such an association. Presented herein is a case of a young adult with coarctation of the aorta and secondary left subclavian artery aneurysm, diagnosed incidentally. We discuss the incidence of these rare conditions and possible causes for their co-existence.
|
Case report
|
|---|
A 19-year-old male was admitted with 1 week of fever, cough and headaches. On the day of admission he complained of chest pain radiating to the back. His previous medical history was unremarkable. On examination his body temperature was 40°C, blood pressure was 135/85 mmHg and the pulse 80 bps. Chest auscultation revealed crepitation at the upper right lung field and a left parasternal, 4/6 holosystolic murmur, radiating to the axillae and neck.
The femoral pulses were decreased as compared with the radial pulses. No obvious difference in blood pressure was registrated between the upper limbs. The rest of the examination was within normal limits. Laboratory tests revealed mild leukocytosis (9400 mm–3) but otherwise were normal. The electrocardiogram (ECG) displayed sinus rhythm and left ventricular hypertrophy. A chest radiograph (Figure 1
) showed a right upper lobe pulmonary infiltrate consistent with pneumonia and widening of the mediastinum. Mild rib notching was also seen. A contrast enhanced CT of the chest demonstrated coarctation of the aorta just distal to the origin of the left subclavian artery with rich collateral circulation and a saccular aneurysm of the proximal left subclavian artery (Figure 2). Angiography confirmed these findings (Figure 3), demonstrated the left vertebral artery arching distal to the aneurysm and elicited a systolic pressure gradient of 50 mmHg across the coarctation. CT of the head performed for the evaluation of headaches and to rule out intracranial aneurysms was unremarkable.
View larger version (137K):
[in this window]
[in a new window]
|
Figure 1. A chest radiograph demonstrating marked widening of the mediastinum and a faint lung infiltrate in the right upper lobe. Mild rib notching is also noted.
|
|
View larger version (80K):
[in this window]
[in a new window]
|
Figure 2. CT scan of the upper thorax showing a vascular dilatation corresponding to the left subclavian artery. Collateral vessels are seen (arrows).
|
|
View larger version (120K):
[in this window]
[in a new window]
|
Figure 3. Arteriography showing coarctation of the aorta at the typical site (arrows) and a saccular dilatation of the proximal left subclavian artery.
|
|
The patient was treated with antibiotics for the pulmonary infection. After 6 weeks an operation was performed with repair of the coarctation by vascular graft interposition between the aortic arch and the descending aorta, resection of the subclavian aneurysm and graft interposition between the aortic arch and the distal subclavian artery.
The post-operative course was uneventful except for a chylothorax that was treated with a low fat diet.
|
Discussion
|
|---|
Coarctation of the aorta is found in approximately 7% of patients with congenital heart disease. If untreated, 25% of patients will die by the end of the second decade, 49% die by the end of the third decade and approximately 90% will die by the age of 50 years [1]. The causes of death include congestive heart failure, aortic rupture and dissection, bacterial endocarditis and intracranial haemorrhage [1].
Clinicians are uncommonly aware of coarctation as a condition to be diagnosed in adult life.
Lewis [2] was the first to describe the method of diagnosis of coarctation by the differences in pulse between the wrists and femoral arteries. Although the diagnosis can usually be made on clinical grounds, imaging is necessary to assess the exact anatomy of the lesion, and to detect possible associated conditions such as cardiac malformations and cerebral aneurysms. Various imaging modalities can be used for diagnosis. Chest radiography can demonstrate dilatation of the aortic segment proximal to the coarctation site and rib notching caused by intercostals collateral circulation. Angiography has traditionally been the definitive pre-operative diagnostic procedure which also allows pressure gradient measurement across the coarctation, visualization of the collaterals and assessment of associated cardiac malformations, but this technique is invasive and enhances the risk for complications imposed by the coarctation.
Two-dimensional echocardiography with simultaneous pulse Doppler or transoesophageal echocardiography are good non-invasive methods to detect coarctation and to measure the pressure gradient across the coarctation [3–6] and have been used as the sole pre-operative imaging study [6].
Intravenous digital subtraction angiography [7] and CT [8, 9] are also useful methods for demonstrating the full extent of the coarctation and the collateral flow. MRI is considered an excellent imaging modality which provides a good delineation of the coarctation, post-stenotic dilatation and dilated collaterals [10].
This method is non-invasive, with no need for iodinated contrast media injection, and has the ability to yield information of the pressure gradient across the coarctation, which is an important indicator of the severity of the lesion.
The presence of aneurysms in patients with coarctation of the aorta is well known. Aneurysms that appear distal to the coarctation are secondary to the turbulated flow in the region of the coarctation. Berry aneurysms are present in 10% of patients with coarctation and are related both to high pressure of the vascular tree above the coarctation and to congenital defects of the vascular tree [11].
True aneurysms of the subclavian artery are rare in comparison with other peripheral aneurysms. On review of 1488 patients with arterial aneurysms only 2 had subclavian aneurysms [12]. The pathogenesis for true aneurysms includes atherosclerosis, post-stenotic dilatation, thoracic outlet obstruction, mycotic aneurysm and Ehrlers-Danlos syndrome [13]. The aetiology for pseudoaneurysms is usually traumatic [16]. The clinical presentation varies: in a series of 15 patients with a subclavian artery aneurysm only 6 patients presented with the classical sign of a pulsatile supraclavicular mass, 4 had Horner syndrome, 4 had upper chest pain, 1 patient suffered from limb ischaemia and 5 patients were asymptomatic [14].
Of these 15 cases only 2 were associated with thoracic coarctation.
Pairolero et al [13], in a 20-year retrospective study in Mayo Clinic found 31 patients with subclavian artery aneurysms. None of these patients had a coarctation of the aorta. A review of the English literature revealed only seven cases of aortic coarctation and associated subclavian artery aneurysm [14–19]. In one case the aetiology was probably hypertension and atherosclerosis in a 59-year-old patient with a very late diagnosis of aortic coarctation [15]. The typical location of the coarctation of the aorta is distal to the origin of the left subclavian artery. The mechanism for subclavian artery aneurysm formation in our patient, and in the former cases described in the literature, is probably the sheer stress of rapid high pressure pulsatile flow into the subclavian artery in addition to marked turbulence caused by the coarctation.
In situ thrombosis, antegrade and retrograde emboli can complicate the subclavian artery aneusysm with the danger of cerebral infarcts and limb ischaemia [14]. Rupture is also a dangerous complication of this condition.
A definite diagnosis is best made by arteriography but other imaging modalities including digital subtraction angiography, ultrasound, CT with multiplanar or volume rendering reformation, and MRI can also be used to demonstrate the pathology. Surgical treatment is recommended for the correction of aortic coarctation as well as subclavian artery aneurysm in young patients. In older patients surgery for aortic coarctation or subclavian artery aneurysm has more complications and a lesser therapeutic effect. In these patients the conservative strategy of using hypotensive drugs should be considered.
Received for publication November 25, 2002.
Revision received June 5, 2003.
Accepted for publication July 14, 2003.
|
References
|
|---|
- Campbell M. Natural history of coarctation of the aorta. Br Heart J 1970;32:633–40.[Abstract/Free Full Text]
- Lewis T. Material relating to coarctation of the aorta of the adult type. Heart 1933;16:205.
- Weyman AE, Caldwell RL, Hurwitz RA, et al. Cross-sectional echocardiography detection of aortic obstruction. 2.Coarctation of the aorta. Circulation 1978;57:498–502.[Abstract/Free Full Text]
- Sahn DJ, Allen HD, McDonald F, et al. Real time cross-sectional echocardiographic diagnosis of coarctation of the aorta: a prospective study of echocardiographic-angiographic correlation. Circulation 1977;56:762–9.[Abstract/Free Full Text]
- Hatle L, Angelsen B. Doppler ultrasound in cardiology. Philadelphia: Lea & Febiger, 1982.
- Smallhorn JF, Huhta JC, Adams PA, et al. Cross-sectional echocardiographic assessment of coarctation in sick neonate and infant. Br Heart J 1983;50:349–61.[Abstract/Free Full Text]
- Moodie DS, Yiannikas M, Gill CC, et al. Intravenous digital subtraction angiography in the evaluation of congenital abnormalities of the aorta and aortic arch. Am Heart J 1982;104:628–34.[CrossRef][Medline]
- Keyter S, Cholankeril MV. CT detection of coarctation of the aorta. CT 1981;5:355–8.
- Goodwin JD, Herfkens RJ, Brundage BH, et al. Evaluation of coarctation of the aorta by computed tomography. J Comput Assist Tomogr 1981;5:1253–6.
- Von Schulthess GK, Higashino SM, Higgins SS, et al. Coarctation of the aorta: MR imaging. Radiology 1986;158:469–74.[Abstract/Free Full Text]
- Morris MJH, McNamara DG. Structural congenital cardiovascular defects. In: Garson A Jr, Bricker JT, McNamara DG, editors. The science and practice of pediatric cardiology. Vol. II. Philadelphia/London: Lea and Febiger, 1990: 1366–7.
- Dent TL, Lindenauer SM, Ernst CB, et al. Multiple arteriosclerotic arterial aneurysms. Arch Surg 1972;105:338–43.[Abstract/Free Full Text]
- Pairolero PC, Walls JT, Payne WS, et al. Subclavian-axillary artery aneurysms. Surgery 1981;90:757–63.[Medline]
- McCollum CH, Da Gama AD, Noon GP, Debakey ME. Aneurysms of the subclavian artery. J Cardiovasc Surg 1979;20:159–64.[Medline]
- Clemente C, Vidal MT, Tornos MT, Soler-Soler J. Right subclavian artery aneurysm and coarctation of the aorta. Int J Cardiol 1993;43:199–201.
- Amah G, Milliez P, Blacher J, Girerd X, Couetil JP, Safar ME. Delayed diagnosis of aortic coarctation: the third medical visit. Circulation 1999;100:e51–2.[Free Full Text]
- De Mozzi P, Bortolotti U, Fracasso A, et al. Aortic coarctation with bilateral aneurysms of the subclavian arteries: surgical tratment. Chir Ital 1981;33:635–8.[Medline]
- Henderson RA, Ward C, Campbell C. Dissecting left subclavian aneurysm: an unusual presentation of coarctation of the aorta. Int J Cardiol 1993;40:69–70.[CrossRef][Medline]
- Aerichide N, Grondin P, Lepage G, et al. Coarctation of the aorta complicated by an aneurysm of the subclavian artery. Union Med Can 1965;94:1301–5.[Medline]
Top
Abstract
Case report
