O. Valdez-Davila et al.

Fortschr. Röntgenste. 130, 4

Fortschr. Rönrgenstr. 130, 4 (1979) 394-397

Aortenisthmusstenose der Aorta abdominalis

Coarctation of the abdominal aorta By O. Valdez-Davila, M.D., W. R. Castaneda-Zuniga, M.D., P. H. Nath, M.D., G. Velasquez, M.D., C. Zollikofer, M.D., A. Formanek, M.D. and K. Amplatz, M.D.

Department of Radiology, Unisersity of Minnesota Hospitals, Minneapolis, Minnesota

Die Aortenisthmusstenose der Bauchschlagader ist zwar selten, stellt jedoch eine wichtige Ursache systemischer Hypertension dar. Zur Diagnose und zur Feststellung des

Coarctation of the abdominal aorta is an uncommon process but an important cause of systemic hypertension. Angiography is the investigation of choice to establish a diagnosis and document the extent of involvement. However, angiography does not provide an etiological diagnosis; and it may not be possible to distinguish an acquired coarctation from a congenital process. Three patients with coarctation of the abdominal aorta are presented.

möglich sein, eine erworbene Aortenisthmusstenose von einer angeborenen bzw. ererbten zu unterscheiden. Der Artikel stellt drei Patienten mit Aortenisthmusstenose der Bauchschlagader vor.

3 Figures

Coarctation of the abdominal aorta (CAA) is an uncommon entity (approximately 2% of all coarctations of the aorta). It is an important cause of arterial hypertension; and if an adequate diagnosis is not well established pre-operatively, it can lead to the performance of inadequate surgical treatment. It is the purpose of this paper to report three cases of coarctation of the abdominal aorta which were studied and treated at the University of Minnesota.

Ausmaes der Erkrankung hat sich die Angiographie als das Mittel der Wahl bewährt. Jedoch liefert die Angiographie keine ätiologische Diagnose; es kann u.U. nicht

hypertension and progressive chronic renal failure in association with coarctation of the abdominal aorta. The patient had been subjected to multiple surgical procedures for the relief of the vascular problems associated with the coarctation, with the application of a graft from the proximal to the distal abdominal aorta at the level of the bifurca-

tion. He also underwent a left nephrectomy for a non-functioning hypoplastic kidney. Because of the persistence of severe hypertension and progressive renal failure, the patient was referred to the University of Minnesota for evaluation and treatment of his vascular problems.

An abdominal aortogram was performed which revealed two renal

Case reports Case 1: (D. T.) A seventeen year old white male was referred to the University of Minnesota for evaluation and treatment of long standing

0340-1618/79 0432-0394 $ 03.00 © 1979

arteries supplying the right kidney (Fig. 1); the upper pole renal artery was arising from the abdominal aorta with a 90% stenosis at its takeoff and the lower renal artery was arising from the unaffected distal abdominal aorta just above the bifurcation without evidence of nar-

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394

Coarctation of the abdominal aorta

395

(Case 1)

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Fig. 1

Fortschr. Röntgenstr. 130, 4

Abdominal aortogram. Diffuse coarctation of the abdominal aorta ¡s seen.

Contrast medium retluxes into the gratt (G) between the thoracic aorta and aortic bifurcation. There are two renal arteries on the right (arrow) and there is severe stenosis of the origin

ot the superior renal artery (small arrow). A = non-magnified angiogram, B = magnification angiogram.

Fig. 2

(Case 2)

PA view of the chest. Signs of left thoracotomy are seen. Ascending aorta is prominent. There is no poststenotic dilatation of the descending aorta. The gib and 1 0th posterior ribs on the right and the 10th rib on the left exhibit notching Fig. 2a

(arrow). Fig.

2b

Lateral view of an aorto-

gram. There is severe coarctation of

the abdominal aorta beginning above the level of the coeliac axis (arrow).

Superior mesenteric artery (S)

is

severly narrowed at its origin.

rowing. Severe narrowing of the abdominal aorta from about the level

of the origin of the coeliac trunk was noted which extended to just above the bifurcation of the abdominal aorta. A graft connecting the

high abdominal aorta above the area of narrowing to the distal abdominal aorta at the level of the bifurcation was demonstrated. An autotransplant of the right kidney was performed to the right lower quadrant with anastomosis of both renal arteries to the right hypogastric artery and vein. Case 2: (M. H.) A fourteen year old white female with a recent onset of hypertension and absent femoral pulses was referred to the University

of Minnesota Hospitals with a clinical diagnosis of coarctation of the thoracic aorta. A left thoracotomy was performed without aortography; the aorta was found to he normal during the surgical exploration of the thoracic segment. A follow-up catheterization demonstrated the presence of a coarctatlon of the abdominal aorta (Fig. 2). A second surgical exploration was then performed which confirmed the presence of narrowing of the abdominal aorta, just below the iaphragm with

gradual tapering involving the renal arteries; the left renal artery showed a severe narrowing at its take-off with marked post-stenotic dilatation. The right renal artery was normal. A bypass graft to the left renal artery was then performed. A follow-up aortogram revealed

O. Valdez-Davila et al.

Fortschr. Röntgenstr. 130, 4

Fig. 3

(Case 3). Aortogram.

Fig. 3a There is coarctation of the abdominal aorta from the level of the superior mesenterio artery to below

the renal arteries. There is an additional narrowing of the origin of the left renal artery (arrow).

Fig. 3b A late film from the same angiogram showing the extent of the coarctation.

stenosis at the take-off of all the major branches: coeliac trunk, superior mesenteric artery, renal arteries, and inferior mesenteric

Case 3: (K. W.) A previously healthy seven year old white female experienced an episode of left hemiparesis which was found to be

artery. The affected segment of the aorta extended down to lust above the bifurcation of the abdominal aorta. A new surgical procedure was then performed with the application of a teflon graft from the proximal to the distal abdominal aorta at the level of the bifurcation. AU the major branches: coeliac trunk, superior mesenteric artery, renal arteries, and inferior mesenteric artery were connected to the graft.

secondary to hpyertension. In the evaluation for etiology of hypertension an angiogram revealed a localized segment of narrowing of the abdominal aorta (Fig. 3( at the level of the renal arteries with involvement of both right and left renal arteries. The plasma renin level was elevated. An autotransplantation of the right kidney to the right iliac fossa was performed successfully together with a left nephrectomy.

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396

Coarctation of the abdominal aorta

Since the first case report by Quain in 1847, less than 150 cases of coarctation of the abdominal aorta have been reported (2, 7, 10).

Bahnson in 1949 was the first to report the angiographie changes associated with coarctation of the abdominal aorta in two cases (1). The coactation of the abdominal aorta is a rather uncommon entity which accounts for approximately 2% of all coarctations of the aorta. The classic coarctation of the aorta

at the isthmus is seen with more frequency in males (3: 1), while in the few reported cases of coarctation of the abdominal aorta there seems to be slight preponderance in females (2: 1) and particularly in young females (4). Although less common than in classic coarctation, coarctation of the abdominal aorta is also associated with intracranial aneurysms (3). There is no agreement concerning the etiology of the coarcta-

tion of the abdominal aorta. Some people believe this is a congenital process related to an inadequate development and fusion of both dorsal aortas; against this theory is the absence

of an intra-luminal septum in the surgical or pathological specimens. In favor of this theory is the fact that it manifests in

young people or children and is associated with multiple abnormalities and there is no histpathological evidence of inflammatory changes (2, 6).

Differentiation between Takayasu's arteritis and congenital coarctation can be difficult or impossible though the distinction is less important to clinical management since treatment in

both is bypass surgery in symptomatic patients. Both conditions affect younger patients with a predilection for females. Hypertension and abdominal bruit can be present in both. Other clinical features like occular symptoms, initial prodromal phase of fever, myalgia, etc. seen in Takayasu's arteritis

may not always be present. Absence of involvement of brachiocephalic vessels favors congenital coarctation, hut Takayasu's arteritis can also spare arch vessels. In Takayasu's disease transition from diseased to normal artery tends to be abrupt. Incidence of aneurysm formation is higher in arteritis and extremely rare in the congenital type. Segmental aortic or arterial calcification, when present, is strongly suggestive of Takayasu's arteritis. Arteritis is usually associated with abundant collateral formation which is less common in congenital coarctation. This factor is probably responsible for the higher incidence of symptoms in congenital coarctation; association of other intracardiac defects also favors congenital lesions.

The coarctation of the abdominal aorta may appear as an isolated entity as in our three cases or be associated with

Classical clinical manifestations of coarctation at the aortic isthmus are arterial hypertension with absent femoral pulses. When the coarctation involves the abdominal segment, the clinical manifestations are going to be related to the location of the narrowed segment. Only those cases in which the affected segment is below the renal arteries will the blood pressure be

normal (12). Other manifestations may include abdominal angina when the origins of the coeliac, superior mesenteric, and inferior mesenteric arteries are affected. Occasionally a bruit can he heard in the abdomen or lumbar area. Claudication has also been described in association with severe coarcta-

non of the abdominal aorta. If in the evaluation of a patient with classic clinical findings for

coarctation, such as hypertension and absent femoral pulses, the chest x-ray shows a normal aortic arch with no evidence of abnormalities in the barium swallow and absence of erosions of the costal margin in the first eight ribs and with erosions in the last three or four ribs and in whom a bruit is heard, the

diagnosis of coarctation of the abdominal aorta should be considered and an aortogram should be performed. Aortogra-

phy should include AP and lateral projections to evaluate origins of all major branches of the abdominal aorta adequately. If the extent of the

disease process is not accurately

documented, surgical therapy could be incomplete as happened in one of our patients (2, 8, 9). Underlying pathologic process tends to he progressive leading to recurrent and/or new symptoms which also requires angiographie evaluation.

Surgical treatment is essentially palliative and should be tai-

lored to individual patients depending upon the extent of involvement and resultant symptoms. Hence, angiography is essential in the management of these patients though it is not accurate in making an etiologic diagnosis. References I) Bahnson, H. T., R. N. Cooley, R.

Sloan: Coarctation of aorta at u nusual sites: Report of two cases D.

with angiographic and operative findings. Amer. Heart, j. 38 (1949) 905 E.

Korns: Coarctation of the abdomi-

ration of the abdominal aorta. Vase.

aorta. Arch. Pathol. 95 (1973)

nal 221

blizuak, j., j. D. Borgaincr: Coarc-

tation of the abdominal aorta with aneurysm of middle cerebral artery. Amer. j. Roentgenol. 122)1974)29 Bozer, A. Y., F. BoFe, A. Savlan'i,

Gursel: Infrarenal aortic coarcta tino, j. Cardiovasc. Siirg. 14 (1973)

Congenital coarctation of the andonii-

1.

aortic arch; 2. aortic isthmus: pre-

ductal, ductal, and post-ductal; 3. thoracic aorta; 4. abdominal aorta: supra-renal, inter-renal, and mfra-renal.

All of them, with the exception of the aortic isthmus, are considered as atypical sites (11). The cases of coarctation of the

thoracic and abdominal aorta have been classified, according to the extension of the narrowed segment, as (a) segmental and b) diffuse or hypoplastic (5).

127 )i976) 227 (8) Picrach, C. A., H. Katkov: Coarc-

Coarctations of the aorta have been described involving the following segments:

ing thoracic and abdominal aorta: A critical review. Amer. J. Roentgenol.

Ben-Shoshan, M., N. P. Rossi, M.

G.

defined

(7) Lande, A.: Takayasu's arteritis and congenital coarctarion of the descend-

(2)

syndromes of hypercalcemia, congenital rubella, or neurofibromatosis. In 10% of the cases the coarctation of the abdominal aorta is associated with other cardiovascular anomalies such as intracardiac defects and coarctation of the aorta in other places. clearly

397

452

D'Ahreu, A. L., C. G. Roh, J. F. Die coarctatio aortae abdominalis. Langehecks Arch. KIm. Vollinar:

Chir. 290 1959) 521

Doherneck, R.

C.,

R. L. Varco:

nal aorta, j. Lancet 88 (1968) i 43

P. H. Nath, M.D.

Department of Radiology University of Minnesota Hospitals 420 Delaware Street S. E. Minneapolis Minnesota 55455

Surg. 6 (1972) 159 (9) Pierce, W. S., W. R. Vincent,

E.

Fitzgerald, F. j. Miller: Coarctation of

the abdominal aorta with multiple aneurysms. Operative correction. Ann. Thorac. Stirg. 20 (1975) 687 (10) Quain, R.: Partial contraction of the abdominal aorta. Trans. Path. Soc. London 1)1848) 244 (11) Vollmor, J.: Reconstruktive Chirurgie der Arterien. Georg Thieme Verlag, Stuttgart (1967) 57 (12) Yagi, S., D. M. Kramsch, I. M. Madhoff, W. Hollander: Plasma renio activity in hypertension associated with coarctation of the aorta. Amer. j.

Physiol. 215 (1968) 605

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Discussion

Fortschr. Röntgenstr. 130, 4

Coarctation of the abdominal aorta.

O. Valdez-Davila et al. Fortschr. Röntgenste. 130, 4 Fortschr. Rönrgenstr. 130, 4 (1979) 394-397 Aortenisthmusstenose der Aorta abdominalis Coarct...
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