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Subclavian steal syndrome in right aortic arch with isolation of the left subclavian artery
Subclcavian steal syndrome in right with isolation of the left subclavian
aortic arch artery
W. H. Shuford, M.D. R. G. Sybevs, M.D., Ph.D. R. C. Schlant, M.D. Atltrntn, GCI.
C
and Reivich and associates2 first described patients with proximal subclavian artery obstruction in which circulation to the arm was maintained by retrograde flow down the vertebral artery with drainage of blood away from the basilar territory of the brain. This reversal of blood flow in the vertebral artery may cause basilar artery insufficiency, and the symptoms and signs resulting from ischemia of the brain stem have been termed “the subclavian steal syndrome.“3 Many causes of the subclavian steal syndrome have been described and include atherosclerosis,4*5 Takayasu’s arteritis,” tumor thrombus,’ embolic occlusion,* surgical ligation of the subclavian artery in the Rlalock-Taussig anastomosis,g and congenital atresia of the subclavian artery.“‘-l2 Right aortic arch with the left subclavian artery no longer connected to the aorta (isolation of the left subclavian artery) is a rare congenital anomaly,13 and an unusual cause of subclavian steal.14 For this reason it was considered of interest to describe such a case with angiographic demonstration of reversal of blood flow in the left vertebral artery. In addition, ontornil
From
the importance of extracerebral collateral channels as a source of blood supply to the left upper extremity in this patient will be emphasized. Case report L. D., W-36177, a 23-year-old soldier, was first seen at Grady Memorial Hospital at age 4 years with acute glomerulonephritis. Blood pressure in the right arm was 160/120 mm. Hg, and no information is available regarding pulse or blood pressure in the left arm. The patient responded well to treatment and on routine follow-up visits at age 7 chest x-rays disclosed a right aortic arch. The blood pressure in the right arm was 98/60 mm. Hg, and blood pressure was unobtainable in the left arm. He was asymptomatic with normal growth and development. The patient was subsequently followed in the cardiac clinic because of the aortic arch anomaly. At age 12 he first noticed that his left arm felt like it was going to sleep, and that it tired easily. As a result, he used his right hand for almost everything. However, he was able to actively participate in sports without difficulty. At age 18 the patient complained of numbness in the left upper extremity of two months’ duration which occurred when his arm was raised above the left shoulder. Except for easy fatigue in the left upper extremity there was no difference between his two arms in his ability to lift weights. There was no history of injury to his left arm, or symptoms
the Departments of Radiology and Medicine, Emory University School of Medicine pital, Atlanta, Ga. Supported by United States Public Health Training Grants HE 5494 and GM 1538. Received for publication June 15, 1970. Reprint requests to: Wade H. Shuford, M.D., Department of Radiology, Emory University Grady Memorial Hospital, 80 Butler Street, S. E., Atlanta, Ga. 30303.
9x
American Heart Journal
and
Grady
School
Memorial
of Medicine
Hos-
and
July, 1971 Vol. 52, No. 1, pp. 95-104
volume Number
82 1
Subclavian
steal syndrome
99
Cerebral arteriography with injection of the left common carotid artery revealed no evidence of arterial flow up the left internal carotid artery through the circle of Willis to supply the left arm (Fig. 3). Upon catheterizing the left brachial artery the catheter met resistance in the first or thoracic portion of the left subclavian artery. Hand injection of radiopaque material revealed this portion of the left subclavian artery to end blindly. There was no luminal connection of the left subclavian artery either with the aorta or with the pulmonary arterial system. The vertebral branch and costocervical and thyrocervical trunks of the left subclavian artery opacified for a short distance (Fig. 4, A and B). Catheter pressures in the ascending aorta were 75/40 mm. Hg with a mean of 60 mm. Hg, and in the left brachial artery 75/52 mm. Hg with a mean of 63 mm. Hg.
Discussion
Fig. 1. Aortogram shows opacilication of the left common carotid, right common carotid, and right subclavian arteries only from the arch. The aorta descends on the right. (From Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Amer., J. Roentgenol., May, 1970, published by Charles C Thomas, Publisher.) to suggest congenital heart disease or cerebral or vertebrobasilar insufficiency. The patient was admitted for aortography. He was well developed and appeared in good health. Auscultatory blood pressures were: right arm, 118/50 mm. Hg; arm raised to shoulder level, 1 lo/SO mm. Hg; left arm, SO/56 mm. Hg; arm raised to shoulder level, 70/60 mm. Hg; right leg, 140/80 mm. Hg; and left leg, 138/70 mm. Hg. The pulses in the left upper extremity were weaker than in the right and disappeared when the left arm was raised above the shoulder. Both carotid and femoral pulses were palpable equally. A continuous murmur was audible over the posterior triangle of the neck on the left side. Examination of the heart and the neurological examination revealed normal findings. The electrocardiogram was within normal limits. Chest roentgenograms disclosed a right aortic arch, a normal-sized heart, and normal pulmonary vasculature. Barium swallow revealed no abnormal indentation on the posterior esophageal wall. The aortogram showed the aorta to descend on the right side. The left common carotid artery arose as the first branch from the arch and was followed by the right common carotid and right subclavian arteries. The left subclavian artery did not opacify from the arch (Fig. 1). Selective injection of the left common carotid artery was then performed (Fig. 2, A). Two seconds following injection the distal left subclavian artery was opacified by collateral channels from the left external carotid artery through the vertebral artery and branches of the thyrocervical and costocervical trunks (Fig. 2, B and C).
In their monograph on malformations of the aortic arch, Stewart, Kincaid, and Edwards15 classify right aortic arch into three major types: (1) with mirror-image branching of the major arteries; (2) with an aberrant left subclavian artery; and (3) with the left subclavian artery isolated from the arch. Right aortic arch with isolation of the left subclavian artery is the least common of the three types. I3 Knowledge concerning this malformation has resulted chiefly from the study of anatomic specimens.15-17 In this entity the left common carotid artery arises as the first branch of the aorta to be followed by the right common carotid and the right subclavian arteries in that order. The left subclavian artery no longer has a connection with the aorta but arisesfrom the left pulmonary artery by way of a left ductus arteriosus (Fig. 5).15J7 Chest roentgenograms in these patients show the aortic arch to be anterior and to the right of the trachea and esophagus.18 The barium swallow shows no compression on the posterior esophagus. This anomaly is frequently associated with cyanotic congenital heart disease, especially the tetralogy of Fallot. 15*17 In our patient there was no evidence for a congenital malformation within the heart. When the first or thoracic portion of the left subclavian artery is absent or obstructed, blood may reach the left arm by one of several pathways. Retrograde flow down the left vertebral artery represents the final common pathway of many
Fig. 2B. Subtraction arteriogram t%o seconds after injection. The distal left subclavian artery opacifies by collateral channels from the left external carotid artery through the vertebral artery and branches of the thyrocervicnl and costocervical trunks. (From Shuford, LV. H., Sybers, li. G., and Schlant, 11. C.: Amer. J. Iioentgeno!., Llay, 1970, published by Charles C Thomas, Publisher.)
k-ix. 2.1. Selective injectiotl of the left ~mmo~~ (‘
\-ii L. ext.
carotid
a.-
Sup.
thyroid
o.
Deep
cervical
cervical
Thyrocervicol
Fig.
ZC. Composite
drawing
i -7,’ , ;i, ,,
o.
branch
Inf. thyroid
b. common
‘8
branch
Vertebral
Asc.
a.
‘; 1 i-g(i ,..j \i.‘. .’.
,-;1’
-0ccipilal
o.
onrverse
trunk carotid
of Fig.
stocervicol a.
2, A and B.
c:ervical trunk
branch
Volume Number
82 1
Fig. 3. Selective injection of the left common carotid artery. There is no evidence of flow through the circle of Willis contributing to the blood supply of the left subclavian artery.
collateral channels (Fig. 6).4~5s1s-23 The first of these is the right vertebral artery which communicates directly with the left vertebral artery at the vertebrobasilar junction. The second collateral route involves the circle of Willis. Blood from either the right or left internal carotid arteries may reach the basilar and left vertebral arteries through the respective right or left posterior communicating and posterior cerebral vessels. In addition to these pathways involving the cerebral and basilar artery circulations, a third route, the “cervical arterial collateral network,” utilizes the occipital branch of the external carotid artery, the muscular branches of the vertebral artery, and the thyrocervical and costocervical trunks of the subclavian artery. Anastomoses between the superior and inferior thyroid arteries and pathways between the thyroid arteries of the opposite side may occur. Finally, anastomoses by way of the internal mammary and intercostal arteries and anastomoses between the intercostal arteries and branches of the axillary artery
Subclavian
steal syndrome
101
Fig. 4A. Retrograde injection of the left subclavian artery with catheter tip in its first portion. (From Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Amer. J. Roentgenol., May, 1970, published by Charles C Thomas, Publisher.)
may be present, but are of lesser importance. In most patients with the subclavian steal syndrome the right vertebral artery directly supplies the left vertebral artery at the vertebrobasilar junction.5 In our patient contrast studies of the right vertebral and right carotid arteries were not performed. For this reason, the amount of blood flow, if any, through the vertebrobasilar-vertebral route and/or crossover filling from the right carotid artery is not known. Selective injection of the left common carotid artery revealed no flow from the left internal carotid artery through the circle of Willis to the basilar artery and hence to the left arm via the left vertebral artery. Arteriographic studies clearly indicate that a principal source of blood to the left upper extremity was from the left external carotid artery. Selective injection of the left common carotid artery showed blood reaching the distal left subclavian artery via anastomoses between the occipital branches of the external carotid artery and muscular branches of the vertebral artery, and anastomoses between the occipital branches of the external
102
Shuford,
Sybers, and S’chlunt
Asc. cervical
Transverse
Thyrocervicol Costocervicol
cervical
trunk trunk
Lot. thorocic
Fig. 4B. No connection
branch
of the left subclavian
artery
with
carotid artery and the thyrocervical and costocervical trunks of the left subclavian artery. In addition, the left superior thyroid artery anastomosed with the inferior thyroid artery to supply the thyrocervical trunk. Bosniakrg has emphasized the importance of these cervical arterial collateral pathways in patients with brachiocephalic occlusive disease. Virtually all of the possible collateral routes in the neck were functioning in this patient. With a difference in blood pressure between the aorta and the left arm there exists in this patient a situation similar, in reverse, to that found in coarctation of the aorta. Theoretically, the congenital collateral pathway that might be expected to be well developed was not present-or, at least, not demonstrated by contrast studies. Aortography showed no blood reaching the left subclavian artery through the aortic intercostal-internal mammary route. This extracerebral pathway generally is not a source for blood reaching the left arm in the subclavian steal syndrome due to atherosclerosis. However, in subclavian steal resulting from the Blalock-Taussig pulmonary-subclavian artery anastomosis, the posterior intercostals may be important pathways for collateral flow through
o
the aorta
or the left pulmonary
artery
is demons1 :rated
R.C.C.A. RS
Fig. 5. Right aortic arch and isolation subclavian artery. The left subclavian connected to the left pulmonary artery ductus arteriosus.
of the artery by the
left is left
their communications with the thoracic and subscapular branches of the axillary artery.24-26 On occasion, rib notching on the side of the anastomosis may be seen in these patients.2* Pate1 and Toole5 have listed the important features of the subclavian steal syndrome. Most prominent among these are cerebral symptoms (vertigo, dizziness,
.
Volume Number
82 1
Subclavian
R. ont.
R. post.
cerebral
steal syndrome
103
o./
communicotin
R. post.
cerebral
R. int. carotid
L. int.
carotid
L. ext.
carotid
a.
Deep
cervical
Asc. cervical R. common
R. thyrocervicol R. subclavian
a.
carotid
a.
branch branch
o.
trunk
L. thyrocervical
trunk
L. costocervical
trunk
a. L. subclovian
a.
int. mammary Lat.
thoracic
Intercostal
Fig. 6. Diagram of collateral circulation cl&ian artery.
a. a.
a.
in the presence of occlusion or absence of first portion of the left sub-
blurred vision, headaches), arm symptoms (pain, cramps, numbness), a weak or absent radial pulse, a supraclavicular bruit, and a blood pressure difference between the two arms. The only complaints experienced by this patient were secondary to inadequate blood flow into the left arm. These symptoms were of minor nature and did not interfere with his performing duties as a combat soldier. This is not surprising as numerous Blalock-Taussig operations have well established that the subclavian artery can be ligated almost without complication.26 Also, symptoms of brain-stem circulatory ischemia were absent. Undoubtedly, the absence of symptoms of basilar artery
insufficiency was due to the rich extracerebral collateral network which developed early in life. It is of interest that in most of the reported cases of subclavian steal syndrome of congenital cause cerebral symptoms have been absent.i0J2J4 With the exception of the recent report by Maranhao and associates,14this case represents the first angiographic demonstration of subclavian steal in this type of congenital malformation of the aortic arch. Summary
A patient with a right aortic arch and isolation of the left subclavian artery and the subclavian steal syndrome is pre-
sented. The clinical
ieatures
resulted
frOIII
a relative ischemia of tlje left arm, and there were no findings to suggest cereln-ovascular insufficiency or congenital heart disease. The esophagrams revealed no defect on the posterior aspect of the esophagus. Collateral channels from the left external carotid artery opacified the distal left subclavian artery. The causes of subclavian steal syndrome are reviejved, together with the pathways for collateral circulation to the left arm when the proximal portion of the left subclavian artery is obstructed.
REFERENCES 1. Contorni, L.: Circolo collaterale vertebrovertebrale nella obliterazione dell’arteria succlavia alla sua origine, Minerva Chir. 15:268, 1960. M., Helling, H. E., Roberts, B., and 2. Reivich, Toole, J. F.: Reversal of blood flow through the vertebral artery and its effect on the cerebral circulation, New Eng. J. Med. 265:878, 1961. A new vascular syndrome in “the 3. Editorial: subclavian steal,” New Eng. J. Med. 265:912, 1961. R. R., Fields, ‘IV. S., DeBakey, M. E., 4. North, and Crawford, E. S.: Brachial-basilar insufficiency syndrome, Neurology 12:810, 1962. 5. Pate], A., and Toole, J. F.: Subclavian steal syndrome-Reversal of cephalic blood flow, Medicine 44:289, 1965. J. H., Jr., and Hanafee, W.: The 6. Grolhnan, roentgen diagnosis of Takayasu’s arteritis, Radioloev 83:387. 1964. 7. Agee, OY’F.: Two unusual cases of subclavian steal syndrome, Amer. J. Roentgenol. 97:447, 1966. H., Gensler, S., Stern, W. Z., and 8. Dardik, Glotzer, P.: Subclavian steal syndrome secondary to embolism: First reported case, Ann. Surg. 164:171, 1964. G. M., and Shah, K. D.: Subclavian 9. Folger, steal in patients with Blalock-Taussig anastomosis, Circulation 31:241, 1965. A. U., and Ottesen, 0. E.: Collateral 10. Antia, circulation in subclavian stenosis or atresia, Amer. J. Cardiol. 18:599, 1966. S., Serfas, L. S., and Ruskinko, A.: 11. Levine, Right aortic arch with subclavian steal syndrome (atresia of left common carotid and left
Massumi, R. .A.: The congenital variety of the “subclavian steal” svndrome, Circulation 28: 1149, 1963. 13. Stewart, J. Ii., Kin&d, 0. \V., and Titus, J. I,.: Right aortic arch: Plain film diagnosis and significance, Amer. J. Roentgenol. 97:377, 1966. 14. Maranhao, V., Gooch, A. S., Ablaza, S. G. G., Nakhjavan, F. K., and Goldberg, H.: Congenital subclavian steal syndrome associated with right aortic arch, Brit. Heart J. 30:875, 1968. J. R., Kincaid, 0. W’., and Edwards, 1.5. Stewart, J. E.: An atlas of vascular rings and related malformations of the aortic arch system, Springfield, Ill., 1964, Charles C Thomas, Publisher, pp. 8-13 and 124-129. J. D., Bregman, E. H., and Edwards, 16. Barger, J. E.: Bilateral ductus arteriosus with right nortic arch and right-sided descending aorta, Amer. J. Roentgenol. 76:758,19.56. A.: Ueber eine seltene Entwicklung17. Ghon. storung des Gefassystems, Verhandl. D. De;tsch. Path. Gesellsch. 12:242, 1908. 18. Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Right aortic arch with isolation of the left subclavian artery, Amer. J. Roentgenol. 109:75, 1970. 19. Bosniak, M. A.: Cervical arterial pathways associated with brachiocephalic occlusive disease. Amer. I. Roenteenol. 91:1232. 1964. 20. Edwards, J. E:, Clagetty 0. T., Drake; R. L., and Christensen, N. A.: Collateral circulation in coarctation of the aorta, Proc. Mayo Clin. 23:333, 1948. 21. Newton, T. H., and Wylie, E. J.: Collateral circulation associated with occlusion of the proximal subclavian and innominate arteries, Amer. I. Roentgenol. 91:394. 1964. 22. Peabody, C. N, and O’Brien, B.: Subclavian steal from the circle of Willis, Angiology 17:148, 1966. RI., Ravinov, I(., and Harenstein, S.: 23. Simon, Proximal subclavian artery occlusion and reversed vertebral blood flow to the arm, Clin. Radio]. 13:201, 1962. 24. Boone, M. L., Swenson, B. E., and Felson, B.: Rib notching: Its many causes, Amer. J. Roentgenol. 91:1075, 1964. 25. Campbell, M.: Unilateral rib-notching from collateral circulation after division of subclavian artery, Brit. Heart J. 20:253, 1958. 26. Webb, W. R., and Burford, T. H.: Gangrene of the arm following use of the subclavian artery in a pulmonosystemic (Blalock) anastomosis, J. Thorac. Surg. 23:199, 1952. 12.
aortic arch artery
W. H. Shuford, M.D. R. G. Sybevs, M.D., Ph.D. R. C. Schlant, M.D. Atltrntn, GCI.
C
and Reivich and associates2 first described patients with proximal subclavian artery obstruction in which circulation to the arm was maintained by retrograde flow down the vertebral artery with drainage of blood away from the basilar territory of the brain. This reversal of blood flow in the vertebral artery may cause basilar artery insufficiency, and the symptoms and signs resulting from ischemia of the brain stem have been termed “the subclavian steal syndrome.“3 Many causes of the subclavian steal syndrome have been described and include atherosclerosis,4*5 Takayasu’s arteritis,” tumor thrombus,’ embolic occlusion,* surgical ligation of the subclavian artery in the Rlalock-Taussig anastomosis,g and congenital atresia of the subclavian artery.“‘-l2 Right aortic arch with the left subclavian artery no longer connected to the aorta (isolation of the left subclavian artery) is a rare congenital anomaly,13 and an unusual cause of subclavian steal.14 For this reason it was considered of interest to describe such a case with angiographic demonstration of reversal of blood flow in the left vertebral artery. In addition, ontornil
From
the importance of extracerebral collateral channels as a source of blood supply to the left upper extremity in this patient will be emphasized. Case report L. D., W-36177, a 23-year-old soldier, was first seen at Grady Memorial Hospital at age 4 years with acute glomerulonephritis. Blood pressure in the right arm was 160/120 mm. Hg, and no information is available regarding pulse or blood pressure in the left arm. The patient responded well to treatment and on routine follow-up visits at age 7 chest x-rays disclosed a right aortic arch. The blood pressure in the right arm was 98/60 mm. Hg, and blood pressure was unobtainable in the left arm. He was asymptomatic with normal growth and development. The patient was subsequently followed in the cardiac clinic because of the aortic arch anomaly. At age 12 he first noticed that his left arm felt like it was going to sleep, and that it tired easily. As a result, he used his right hand for almost everything. However, he was able to actively participate in sports without difficulty. At age 18 the patient complained of numbness in the left upper extremity of two months’ duration which occurred when his arm was raised above the left shoulder. Except for easy fatigue in the left upper extremity there was no difference between his two arms in his ability to lift weights. There was no history of injury to his left arm, or symptoms
the Departments of Radiology and Medicine, Emory University School of Medicine pital, Atlanta, Ga. Supported by United States Public Health Training Grants HE 5494 and GM 1538. Received for publication June 15, 1970. Reprint requests to: Wade H. Shuford, M.D., Department of Radiology, Emory University Grady Memorial Hospital, 80 Butler Street, S. E., Atlanta, Ga. 30303.
9x
American Heart Journal
and
Grady
School
Memorial
of Medicine
Hos-
and
July, 1971 Vol. 52, No. 1, pp. 95-104
volume Number
82 1
Subclavian
steal syndrome
99
Cerebral arteriography with injection of the left common carotid artery revealed no evidence of arterial flow up the left internal carotid artery through the circle of Willis to supply the left arm (Fig. 3). Upon catheterizing the left brachial artery the catheter met resistance in the first or thoracic portion of the left subclavian artery. Hand injection of radiopaque material revealed this portion of the left subclavian artery to end blindly. There was no luminal connection of the left subclavian artery either with the aorta or with the pulmonary arterial system. The vertebral branch and costocervical and thyrocervical trunks of the left subclavian artery opacified for a short distance (Fig. 4, A and B). Catheter pressures in the ascending aorta were 75/40 mm. Hg with a mean of 60 mm. Hg, and in the left brachial artery 75/52 mm. Hg with a mean of 63 mm. Hg.
Discussion
Fig. 1. Aortogram shows opacilication of the left common carotid, right common carotid, and right subclavian arteries only from the arch. The aorta descends on the right. (From Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Amer., J. Roentgenol., May, 1970, published by Charles C Thomas, Publisher.) to suggest congenital heart disease or cerebral or vertebrobasilar insufficiency. The patient was admitted for aortography. He was well developed and appeared in good health. Auscultatory blood pressures were: right arm, 118/50 mm. Hg; arm raised to shoulder level, 1 lo/SO mm. Hg; left arm, SO/56 mm. Hg; arm raised to shoulder level, 70/60 mm. Hg; right leg, 140/80 mm. Hg; and left leg, 138/70 mm. Hg. The pulses in the left upper extremity were weaker than in the right and disappeared when the left arm was raised above the shoulder. Both carotid and femoral pulses were palpable equally. A continuous murmur was audible over the posterior triangle of the neck on the left side. Examination of the heart and the neurological examination revealed normal findings. The electrocardiogram was within normal limits. Chest roentgenograms disclosed a right aortic arch, a normal-sized heart, and normal pulmonary vasculature. Barium swallow revealed no abnormal indentation on the posterior esophageal wall. The aortogram showed the aorta to descend on the right side. The left common carotid artery arose as the first branch from the arch and was followed by the right common carotid and right subclavian arteries. The left subclavian artery did not opacify from the arch (Fig. 1). Selective injection of the left common carotid artery was then performed (Fig. 2, A). Two seconds following injection the distal left subclavian artery was opacified by collateral channels from the left external carotid artery through the vertebral artery and branches of the thyrocervical and costocervical trunks (Fig. 2, B and C).
In their monograph on malformations of the aortic arch, Stewart, Kincaid, and Edwards15 classify right aortic arch into three major types: (1) with mirror-image branching of the major arteries; (2) with an aberrant left subclavian artery; and (3) with the left subclavian artery isolated from the arch. Right aortic arch with isolation of the left subclavian artery is the least common of the three types. I3 Knowledge concerning this malformation has resulted chiefly from the study of anatomic specimens.15-17 In this entity the left common carotid artery arises as the first branch of the aorta to be followed by the right common carotid and the right subclavian arteries in that order. The left subclavian artery no longer has a connection with the aorta but arisesfrom the left pulmonary artery by way of a left ductus arteriosus (Fig. 5).15J7 Chest roentgenograms in these patients show the aortic arch to be anterior and to the right of the trachea and esophagus.18 The barium swallow shows no compression on the posterior esophagus. This anomaly is frequently associated with cyanotic congenital heart disease, especially the tetralogy of Fallot. 15*17 In our patient there was no evidence for a congenital malformation within the heart. When the first or thoracic portion of the left subclavian artery is absent or obstructed, blood may reach the left arm by one of several pathways. Retrograde flow down the left vertebral artery represents the final common pathway of many
Fig. 2B. Subtraction arteriogram t%o seconds after injection. The distal left subclavian artery opacifies by collateral channels from the left external carotid artery through the vertebral artery and branches of the thyrocervicnl and costocervical trunks. (From Shuford, LV. H., Sybers, li. G., and Schlant, 11. C.: Amer. J. Iioentgeno!., Llay, 1970, published by Charles C Thomas, Publisher.)
k-ix. 2.1. Selective injectiotl of the left ~mmo~~ (‘
\-ii L. ext.
carotid
a.-
Sup.
thyroid
o.
Deep
cervical
cervical
Thyrocervicol
Fig.
ZC. Composite
drawing
i -7,’ , ;i, ,,
o.
branch
Inf. thyroid
b. common
‘8
branch
Vertebral
Asc.
a.
‘; 1 i-g(i ,..j \i.‘. .’.
,-;1’
-0ccipilal
o.
onrverse
trunk carotid
of Fig.
stocervicol a.
2, A and B.
c:ervical trunk
branch
Volume Number
82 1
Fig. 3. Selective injection of the left common carotid artery. There is no evidence of flow through the circle of Willis contributing to the blood supply of the left subclavian artery.
collateral channels (Fig. 6).4~5s1s-23 The first of these is the right vertebral artery which communicates directly with the left vertebral artery at the vertebrobasilar junction. The second collateral route involves the circle of Willis. Blood from either the right or left internal carotid arteries may reach the basilar and left vertebral arteries through the respective right or left posterior communicating and posterior cerebral vessels. In addition to these pathways involving the cerebral and basilar artery circulations, a third route, the “cervical arterial collateral network,” utilizes the occipital branch of the external carotid artery, the muscular branches of the vertebral artery, and the thyrocervical and costocervical trunks of the subclavian artery. Anastomoses between the superior and inferior thyroid arteries and pathways between the thyroid arteries of the opposite side may occur. Finally, anastomoses by way of the internal mammary and intercostal arteries and anastomoses between the intercostal arteries and branches of the axillary artery
Subclavian
steal syndrome
101
Fig. 4A. Retrograde injection of the left subclavian artery with catheter tip in its first portion. (From Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Amer. J. Roentgenol., May, 1970, published by Charles C Thomas, Publisher.)
may be present, but are of lesser importance. In most patients with the subclavian steal syndrome the right vertebral artery directly supplies the left vertebral artery at the vertebrobasilar junction.5 In our patient contrast studies of the right vertebral and right carotid arteries were not performed. For this reason, the amount of blood flow, if any, through the vertebrobasilar-vertebral route and/or crossover filling from the right carotid artery is not known. Selective injection of the left common carotid artery revealed no flow from the left internal carotid artery through the circle of Willis to the basilar artery and hence to the left arm via the left vertebral artery. Arteriographic studies clearly indicate that a principal source of blood to the left upper extremity was from the left external carotid artery. Selective injection of the left common carotid artery showed blood reaching the distal left subclavian artery via anastomoses between the occipital branches of the external carotid artery and muscular branches of the vertebral artery, and anastomoses between the occipital branches of the external
102
Shuford,
Sybers, and S’chlunt
Asc. cervical
Transverse
Thyrocervicol Costocervicol
cervical
trunk trunk
Lot. thorocic
Fig. 4B. No connection
branch
of the left subclavian
artery
with
carotid artery and the thyrocervical and costocervical trunks of the left subclavian artery. In addition, the left superior thyroid artery anastomosed with the inferior thyroid artery to supply the thyrocervical trunk. Bosniakrg has emphasized the importance of these cervical arterial collateral pathways in patients with brachiocephalic occlusive disease. Virtually all of the possible collateral routes in the neck were functioning in this patient. With a difference in blood pressure between the aorta and the left arm there exists in this patient a situation similar, in reverse, to that found in coarctation of the aorta. Theoretically, the congenital collateral pathway that might be expected to be well developed was not present-or, at least, not demonstrated by contrast studies. Aortography showed no blood reaching the left subclavian artery through the aortic intercostal-internal mammary route. This extracerebral pathway generally is not a source for blood reaching the left arm in the subclavian steal syndrome due to atherosclerosis. However, in subclavian steal resulting from the Blalock-Taussig pulmonary-subclavian artery anastomosis, the posterior intercostals may be important pathways for collateral flow through
o
the aorta
or the left pulmonary
artery
is demons1 :rated
R.C.C.A. RS
Fig. 5. Right aortic arch and isolation subclavian artery. The left subclavian connected to the left pulmonary artery ductus arteriosus.
of the artery by the
left is left
their communications with the thoracic and subscapular branches of the axillary artery.24-26 On occasion, rib notching on the side of the anastomosis may be seen in these patients.2* Pate1 and Toole5 have listed the important features of the subclavian steal syndrome. Most prominent among these are cerebral symptoms (vertigo, dizziness,
.
Volume Number
82 1
Subclavian
R. ont.
R. post.
cerebral
steal syndrome
103
o./
communicotin
R. post.
cerebral
R. int. carotid
L. int.
carotid
L. ext.
carotid
a.
Deep
cervical
Asc. cervical R. common
R. thyrocervicol R. subclavian
a.
carotid
a.
branch branch
o.
trunk
L. thyrocervical
trunk
L. costocervical
trunk
a. L. subclovian
a.
int. mammary Lat.
thoracic
Intercostal
Fig. 6. Diagram of collateral circulation cl&ian artery.
a. a.
a.
in the presence of occlusion or absence of first portion of the left sub-
blurred vision, headaches), arm symptoms (pain, cramps, numbness), a weak or absent radial pulse, a supraclavicular bruit, and a blood pressure difference between the two arms. The only complaints experienced by this patient were secondary to inadequate blood flow into the left arm. These symptoms were of minor nature and did not interfere with his performing duties as a combat soldier. This is not surprising as numerous Blalock-Taussig operations have well established that the subclavian artery can be ligated almost without complication.26 Also, symptoms of brain-stem circulatory ischemia were absent. Undoubtedly, the absence of symptoms of basilar artery
insufficiency was due to the rich extracerebral collateral network which developed early in life. It is of interest that in most of the reported cases of subclavian steal syndrome of congenital cause cerebral symptoms have been absent.i0J2J4 With the exception of the recent report by Maranhao and associates,14this case represents the first angiographic demonstration of subclavian steal in this type of congenital malformation of the aortic arch. Summary
A patient with a right aortic arch and isolation of the left subclavian artery and the subclavian steal syndrome is pre-
sented. The clinical
ieatures
resulted
frOIII
a relative ischemia of tlje left arm, and there were no findings to suggest cereln-ovascular insufficiency or congenital heart disease. The esophagrams revealed no defect on the posterior aspect of the esophagus. Collateral channels from the left external carotid artery opacified the distal left subclavian artery. The causes of subclavian steal syndrome are reviejved, together with the pathways for collateral circulation to the left arm when the proximal portion of the left subclavian artery is obstructed.
REFERENCES 1. Contorni, L.: Circolo collaterale vertebrovertebrale nella obliterazione dell’arteria succlavia alla sua origine, Minerva Chir. 15:268, 1960. M., Helling, H. E., Roberts, B., and 2. Reivich, Toole, J. F.: Reversal of blood flow through the vertebral artery and its effect on the cerebral circulation, New Eng. J. Med. 265:878, 1961. A new vascular syndrome in “the 3. Editorial: subclavian steal,” New Eng. J. Med. 265:912, 1961. R. R., Fields, ‘IV. S., DeBakey, M. E., 4. North, and Crawford, E. S.: Brachial-basilar insufficiency syndrome, Neurology 12:810, 1962. 5. Pate], A., and Toole, J. F.: Subclavian steal syndrome-Reversal of cephalic blood flow, Medicine 44:289, 1965. J. H., Jr., and Hanafee, W.: The 6. Grolhnan, roentgen diagnosis of Takayasu’s arteritis, Radioloev 83:387. 1964. 7. Agee, OY’F.: Two unusual cases of subclavian steal syndrome, Amer. J. Roentgenol. 97:447, 1966. H., Gensler, S., Stern, W. Z., and 8. Dardik, Glotzer, P.: Subclavian steal syndrome secondary to embolism: First reported case, Ann. Surg. 164:171, 1964. G. M., and Shah, K. D.: Subclavian 9. Folger, steal in patients with Blalock-Taussig anastomosis, Circulation 31:241, 1965. A. U., and Ottesen, 0. E.: Collateral 10. Antia, circulation in subclavian stenosis or atresia, Amer. J. Cardiol. 18:599, 1966. S., Serfas, L. S., and Ruskinko, A.: 11. Levine, Right aortic arch with subclavian steal syndrome (atresia of left common carotid and left
Massumi, R. .A.: The congenital variety of the “subclavian steal” svndrome, Circulation 28: 1149, 1963. 13. Stewart, J. Ii., Kin&d, 0. \V., and Titus, J. I,.: Right aortic arch: Plain film diagnosis and significance, Amer. J. Roentgenol. 97:377, 1966. 14. Maranhao, V., Gooch, A. S., Ablaza, S. G. G., Nakhjavan, F. K., and Goldberg, H.: Congenital subclavian steal syndrome associated with right aortic arch, Brit. Heart J. 30:875, 1968. J. R., Kincaid, 0. W’., and Edwards, 1.5. Stewart, J. E.: An atlas of vascular rings and related malformations of the aortic arch system, Springfield, Ill., 1964, Charles C Thomas, Publisher, pp. 8-13 and 124-129. J. D., Bregman, E. H., and Edwards, 16. Barger, J. E.: Bilateral ductus arteriosus with right nortic arch and right-sided descending aorta, Amer. J. Roentgenol. 76:758,19.56. A.: Ueber eine seltene Entwicklung17. Ghon. storung des Gefassystems, Verhandl. D. De;tsch. Path. Gesellsch. 12:242, 1908. 18. Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Right aortic arch with isolation of the left subclavian artery, Amer. J. Roentgenol. 109:75, 1970. 19. Bosniak, M. A.: Cervical arterial pathways associated with brachiocephalic occlusive disease. Amer. I. Roenteenol. 91:1232. 1964. 20. Edwards, J. E:, Clagetty 0. T., Drake; R. L., and Christensen, N. A.: Collateral circulation in coarctation of the aorta, Proc. Mayo Clin. 23:333, 1948. 21. Newton, T. H., and Wylie, E. J.: Collateral circulation associated with occlusion of the proximal subclavian and innominate arteries, Amer. I. Roentgenol. 91:394. 1964. 22. Peabody, C. N, and O’Brien, B.: Subclavian steal from the circle of Willis, Angiology 17:148, 1966. RI., Ravinov, I(., and Harenstein, S.: 23. Simon, Proximal subclavian artery occlusion and reversed vertebral blood flow to the arm, Clin. Radio]. 13:201, 1962. 24. Boone, M. L., Swenson, B. E., and Felson, B.: Rib notching: Its many causes, Amer. J. Roentgenol. 91:1075, 1964. 25. Campbell, M.: Unilateral rib-notching from collateral circulation after division of subclavian artery, Brit. Heart J. 20:253, 1958. 26. Webb, W. R., and Burford, T. H.: Gangrene of the arm following use of the subclavian artery in a pulmonosystemic (Blalock) anastomosis, J. Thorac. Surg. 23:199, 1952. 12.