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Congenital subclavian steal: Report of a case occurring in a neonate and review of the literature
Congenital subclavian steal: Report of a case occurring in a neonate and review of the literature Daniel R. Pieroni, M.D. Sidney T. Brodsky, M.D. Richard D. Rowe, M.D., F.R.C.P. Baltimore, Md.
A
lthough the subclavian steal is a well appreciated affliction of the elderly patient, it has seldom been reported in childhood and rarely recognized in the newborn. It results from occlusion of the proximal subclavian artery with subsequent retrograde blood Ilow to the distal subclavian by way of the ipsilateral vertebral artery. Review of the literature reveals the number of cases of congenital subclavian steal to be rapidly increasing, indicating that the occurrence is not as rare as previously thought. Our case demonstrates that the congenital subclavian steal may present in the newborn period and can be easily diagnosed. It discusses the typical clinical signs one might expect to encounter and describes the early development of collateral pathways. This review should alert the clinician to the associated cardiovascular lesions and present a glimpse of the natural history of the defect with a view toward timely surgical intervention. Case history When this 3,600 gram newborn developed severe congestive heart failure at 15 days of age he was transferred to Johns Hopkins Hospital for definitive cardiac evaluation.
(Ed-in.)
On physical examination he was a dusky infant with marked tachypnea. The pulse rate was 156 per minute and the respirations were 80 per minute. A discrepancy was noted in both the blood pressure determinations and the pulse amplitudes. The right arm and leg pressures were loo,‘65 mm. Hg and the left arm was 80/60 mm. Hg. The left brachial and radial pulses were indistinct to palpation. The precordium was hyperdynamic and there was a gallop rhythm. The first heart sound was normal and the second showed pulmonary accentuation. Neither a bruit nor a murmur was noted on auscultation. Gross hepatomegaly was found without evidence of splenic enlargement. Neurologic examination was normal. The initial electrocardiograms (ECG’s) showed biatrial enlargement and right ventricular hypertrophy with an indeterminate axis. There was right ventricular enlargement and pulmonary plethora on the chest film with a small posterior indentation of the esophagram. Cardiac catheterization was performed after 48 hours of successful anticongestive therapy. The hemodynamic data revealed a large ventricular septal defect with elevated pulmonary arteriolar resistance. The cineangiocardiogram demonstrated a ventricular septal defect and a right aortic arch. The left and right common carotid arteries were the first brachiocephalic branches (Fig. l/I). These were followed by the right subclavian artery which gave off the right vertebral and internal mammary arteries (Fig. 1B). A blind diverticulum opacified on the distal arch (Fig. 1C). Although the proximal left subclavian arterv could not be identified. filling of the distal left subclavian artery took place in a
From the Division of Pediatric Cardiology. Department Supported by United States Public Health Service grant Project grant 12 H201. Received for publication June 5. 1972. Reprint requests to: Daniel R. Pieroni. M.D., The Helen pital. Baltimore, Md. 21205.
Vol. 84, No. 6, pp. 801-807
December, 1972
of Pediatrics. 5ROl HE10718
B. Taussig
The Johns Hopkins and The Maternal
Children’s
Cardiac
Hospital. and Child
Center.
Johns
Baltimore. Md. Health Service
Hopkins
American Heart Journal
Has-
801
802
Pieroni,
Brodsky,
Am. Heart J. 1972 December,
and Rowe
Fig. I, A through D. A, Retrograde right subclavian arteriogram in the LAO position revealed the left @CC), right common carotid (RCC), and right subclavian arteries (RSC), respectively. B, Origin of the right internal mammary (RIM) and tortuous vertebral @VA) arteries from the right subclnvian (RSC). C, A conspicuous left subclavian filling. D, Late descending aortic arch diverticulum (D) was noted in the absence of proximal phase retrograde filling of the distal left subclavian (UC) and internal mammary arteries (MM) by way of the left vertebral artery &VA). The blind stump of the distal subclavian approximated the level of the diverticulum.
retrograde fashion by way of the left vertebral artery. Both the left subclavian and internal mammary arteries could be seen in the late frames of the cineangiogram (Fig. 1D). By 18 months of age a barium study continued to show a conspicuous posterior indentation (Fig. 2), while roentgenograms of the upper extremities were normal. Reassessment of the blood pressures re-
vealed a right arm pressure of 100/70 mm. Hg, leg pressures of 120/70 mm. Hg, and 90/70 mm. Hg in the left arm. Recatheterization in preparation for definitive cardiac surgery showed a large left-to-right shunt at the ventricular level with slight elevation of the pulmonary arteriotar resistapce. Biplane aortography again revealed the right aortic arch and clearly .1 I i/_II,,
volume~84
Ntmber
Congenital szcbclavian steal
6
Fig. 2. Lateral esophagram enlargement and a posterior the aortic diverticulum.
illustrates indentation
803
left atria1 caused by
allowed definition of the left common carotid artery as the first vessel, followed by the right common carotid and right subclavian arteries, respectively. On the distal arch a prominent blind diverticulum opacified and an unusual collateral artery was visualized reaching for the left arm (Fig. 3). Careful examination of the late phase films in the region of the left clavicle revealed retrograde filling of the left subclnvian artery by way of the left vertebral artery and thyrocervical trunk (Fig. 4). A subcristal ventricular septal defect was successfully closed at open heart surgery. No intervention was deemed necessary for the obliterated proximal subclnvian artery. The patient has done well since surgery.
Discussion
The subclavian steal phenomenon was first described by Contornir in 1960. When the proximal portion of the subclavian artery was obstructed, retrograde blood flow occurred in the ipsilateral vertebral artery into the low pressure distal subclavian system. Soon afterwards, Reivich and associates2reported a clinical syndrome produced from occlusion of the proximal subclavian artery. Blood siphoned from the basilar area of the brain by the vertebral artery produced symptoms of cerebral insufficiency.3~4 The combination of hemo-
Fig. 3, A and B. A, Anteroposterior view. B, Lateral view. Antegrade aortogram revealing a mirror image right aortic arch with left &CC), right (RCC) common carotid, and right subclavian (RSC) arteries in sequence. A prominent diverticulum, (D) opacified on the descending arch followed by an unusual collateral artery (CA). The proximal left subclavian artery failed to visualize.
dynamic phenomena and clinical symptoms was descriptively designated the “Subclavian Steal Syndrome” in honor of the piratical subclavian artery.j While atherosclerosis is by far the most
so4
Pieroni,
Brodsky,
Am. Heovt I. Dccembcr. 1972
and Rowe
Fig. 4. Magnified left cervical-clavicular retrograde filling of the left subclavian by way of the left vertebral artery thyrocervical trunk (TT).
area artery
(LVA)
with (LSC) and the
common cause,3 other etiologies have included thrombus formation,fi as well as spontaneous emboli from the left heart’ or following traunla.8 Folger and Shah9 reported 12 cases following Rlalock-Taussig anastomosis when the vertebral artery was left intact; Bloodwell and colleaguesrO described it during bypass with failure to perfuse the subclavian artery, and others inadvertently created the syndrome. Intrauterine acquired diseases such as the hypercalcemic syndrome may also cause proximal subclavian obstruction.“-I4 The 26 cases of angiographically documented congenital sul)clavian steal gleaned from the literature are summarized in Table I. Seventeen cases occurred with a right aortic arch and nine with some form of interruption of a left aortic arch. The point of interest is that those patients with a left aortic arch usually have a coarctation or total interruption of the aorta involving the origin of the subclavian, whereas patients with a right aortic arch often have only a localized hypoplasia, atresia, or isolation of the subclavian artery itself with a normal aortic arcll.20~3Z~35 A comprehensive study of the embryologic patterns has been published32 and the anatomic potentials for a subclavian steal outlined in great detail by Becker and co-workers.35 When the subclavian artery arises distal to the coarctation instead of being directly included in the constriction, then blood flow to the lower extremity may be supplied in retrograde fashion through the vertebral-subclavian system, a condition referred to as a “subclavian-aortic stea1.“28 Bilateral subclavian-aortic steals have been
observed in patients with interruption of the aortic arch when the left subclavian and anomalous right subclavian arteries empty distal to the aortic obstruction.18~25~34 As evidenced by our own experience, there are many more steals with coarctations and interruptions than have been reported. Classic neurologic symptoms have never been seen in a newborn patient and are rarely described in childhood. Only 5 patients with the congenital subclavian steal were clearly noted to have associated complaints during childhood.13~22*23~28J3 The voungest patient was 11 years old at the Eime of onset.13 Complaints are usually confined to parasthesias and exercise intolerance of the ipsilateral arm. Cerebral symptoms have not been seen in early childhood. Neurologic expression depends on the delicate balance between the siphon and other collateral circuits supplying the ipsilateral extremity.“s3’ The development of additional extracranial collateral channels in utero and the absence of atherosclerosis are thought to be the main reasons for the lack of symptoms in childhood.“0 This w-ould indicate that elective surgical intervention should not be considered before the second decade at the earliest. Repair shortly before the onset of adolescence should avoid growth retardation of the ipsilateral extremity. Fortunately, the anatomy is much more amenable to reconstructive surgery 1)); that time. Classical collateral pathways involving the internal and external carotids, cervicals, thyroids, occipitals, vertebrals, muscular branches of the vertebrals, epigastrics, and intercostals are frequently identified by angiography even in childhood.20~3s~3g Several collateral pathways were well estaljlished in our patient before a year of age. Besides the vertebral artery and thryocervical trunk, a prominent right internal mammary artery communicated with the left subclavian by way of the intercostal system. The unusual collateral artery distal to the diverticulum (Fig. 3) is similar to that described by Abrams40 in a patient with coarctation of the aorta. The ability of the infant to form early extensive collateral circuits is no longer in question. The parallel process has been well documented in infants with coarctation of the aorta.41 The pooling of data revealed that pa-
Congenital szibclnvian steal
Table I. Summary steal syndrome Author
of 1. 2. 3. 4. 5. 6.
and study
of cases of angiographically
Comnents
18
1954 1963 1964 1964 1964 1964
3,162 > 43 17 days
7. 8. 9. 10. 11. 12. 13.
Pillsbury?’ Levinez2 Bradley23 Antia% Antia Gerbe+ Zetterquistz5
1964 1966 1966 1966 1966 1967 1967
16 33 21 6 12 8 10
14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26.
Replogle2” Lansingz7 Lansing Lovez8 MaranhaosO Ska!pe30 Grossma# Vi!!arr3 Victorica3z Victorica Shufords3 Shuford Morgan”
1968 1968 1968 1968 1968 1969 1969 1969 1970 1970 1970 1970 1970
2 6 3/12 30 19 7 14 11
1972
*Abbreviations: anomalous
congenital subclavian
year
Viannar6 Massumi16~3~ Davesrr Bosniakr* Gro!!manrQ StewartzO
27. Pieroni
docwnented
805
+ -F T + + : 0 0
if
0 0 0 0 0 0 0 T 0 * 0 0
2,122 23 34 19
+" 0 0
15 days
0
0
tients with a congenital subclavian steal have a higher incidence of associated cardiovascular defects. When found in conjunction with a left arch and coarctation, lesions are confined largely to either a patent ductus arteriosus and/or a ventricular septal defect,42 whereas defects with a right arch depend on the particular type of right aortic arch. Several authors43-45 have firmly established the connection between intracardiac defects and a mirror-image right aortic arch. Although the number of cases was small, this correlation was observed in the literature reviewed by us. Tetralogy of Fallot was the most common lesion found with a mirror-image right arch (Table I). Prediction of an associated congenital heart lesion may be made upon identifying the orientation of the aorta with respect to
VSD,
steal
ASD, PDA Interrupted
hypoplastic
aortic
aortic
arch
Supravalve
aortic
stenosis
Hygercalcemic syndrome Interruption aortic arch, steals Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot
0 0 0 0 0 0 + 0 0 0 0 0 f
ASD = atria1 septal defect; Coarct. = coarctation pulmonary venous return; PDA = patent ductus
Right cervical arch VSD, PDA Bilateral subclavian
0 0 : 0 0
Tetralogy
isthmus,
bilateral
bilateral
subclaviaa
bilateral
subclavian
of Fallot
Supravalve aortic stenosis Tetralogy of Fallot VSD. PAPVR
i 0
of aorta; arteriosus;
Interruption steals VSD
aortic
arch,
L = left; R = right: Sx = symptoms; VSD = ventricular septal defect.
PAPVR
= partial
the esophagus. A retroesophageal right aortic arch produces a wide concavity on the barium-filled esophagus. This is not to be confused with the smaller indentation made by an anomalous right subclavian artery from a left aortic arch.32 Therefore, a barium study proves to be an invaluable diagnostic aid in patients with a right aortic arch. Careful examination of both brachial pulses as well as the femorals should be performed routinely on every newborn infant suspected of having congenital heart disease. In the presence of a right aortic arch this simple maneuver may be particularly rewarding. A perceptible difference in the brachial pulse amplitudes should alert the clinician to the possibility of a subclavian steal.
SO6
Pieroni,
Brodsky,
Am. Heart J. December, 1972
and Rowe
in a When aortography is performed patient with tetralogy of Fallot and a right . aortic arch, attention should be focused upon the mode of filling of the left subclavian artery as well as the specific anatomy of the brachiocephalic vessels. Failure to recognize the existence of an atretic proximal left subclavian may eventuate in a futile attempt at a Blalock-Taussig anastomosis with little hope for another palliative procedure from the left thoracotomy approach.
6.
7.
8.
9.
10.
Summary
This report is a reminder that the congenital subclavian steal can occur in the newborn and can be diagnosed clinically. Increased awareness of the phenomenon has been partially responsible for the recognition of 26 cases since the first described in 1960. This would indicate that the entity is not as rare as previously thought. Careful palpation of both brachial pulses as well as the femorals should be performed in all newborns, especially in infants with a right aortic arch or coarctation of the aorta. A discernible bra&al pulse discrepancy should alert the clinician to the possibility of a subclavian steal. While associated cardiovascular lesions are not uncommon, neurologic symptoms are infrequently seen in early childhood. Extensive extracranial collaterals and the .absence of atherosclerosis are thought to be the main reasons for the lack of symptoms in childhood. Elective surgical intervention is, therefore, not usually necessary before the second decade. The authors wish to extend thanks Haroutunian and Mrs. D. Mitchell for preparing the manuscript.
5.
11.
12.
13.
14.
15.
16.
17. 18.
to Dr. L. their aid in 19.
REFERENCES 1.
2.
3.
4.
Contorni, L.: II circolo collaterale vertebralvertebrale nella obliterazione dell ‘arteria subclavia alle sue origini, Minerva Chir. 15:268, 1960. Reivich, M., Helling, H. E., Roberts, B., and Tolle. ”I. F.: Reversal of blood flow through the vertebral artery and its effect on cerebral circulation, N. Engl. J. Med. 265:878, 1961. Piccone, V. A., Jr., and LeVeen, H. H.: The subclavian steal syndrome, Ann. Thorac. Surg. 951, 1970. Henzel, J. H., Pories, W. S., Burget, D. E., and Smith, J. L.: Subclavian steal: evaluation and report of three cases, Am. Surg. 32:.591, 1966.
20.
21.
22.
23.
Editorial: A new vascular syndrome-“The subN. Engl. J. Med. 265:912, clavian steal,” 1961. Bertelsen, S.: Occlusive diseases in the central part of the subclavian artery, Stand. J. Thorac. Cardiovasc. Surg. 2:112, 1968. Gorman, J. F., Navarre, J. R., and McLean, H.: Subclavian steal syndrome, Arch. Surg. 88:350, 1964. Rojas, R., Levitsky, S., and Stansel, H.: Acute traumatic subclavian steal syndrome, J. Thorac. Cardiovnsc. Surg. 51:113, 1966. Folger, G. M., Jr., and Shah, K. D.: Subclavian steal in patients with Blalock-Taussig anastomosis, Circulation 31:241, 1965. Bloodwell, R. D., Hallman, G. I,., and Cooley, D. A.: Total replacement of the aortic arch and the “subclavian steal” phenomena, Ann. Thorac. Surg. 5:236, 1968. Ottesen, O., Antia, A., and Rowe, Ii. D.: Peripheral vascular anomalies associated with the supravalvular aortic stenosis syndrome, Radiology 86:430, 1966. Gerber, N.: Congenital atresia of the subclavian artery: Producing the “subclavian steal syndrome,” Am. J. Dis. Child 113:709, 1967. Villar do Valle, P., Barcia, A., Bargeron, L. M., Jr., Knrp, R. B., and Kirklin, J. W.: Angiographic study of supravalvular aortic stenosis and associated lesions. Report of five cases and review of literature, Ann. Radiol. 12:779, 1969. Varghese. P. 1.. Izukawa. T.. and Rowe. R. D.: Supyavalvula; aortic stenosis as part of’rubella syndrome with discussion of pathogenesis, Br. Heart J. 31:59, 1969. Vianna, F. C., Borges, S., Bocanegra, J., Guinades, R. de F., Gallucci, C., Yunis, C. B., Algranti, N., Balko, R., Casanova, R., and Forte, V.: CoarctacZo a6rtiea corn subclAvia eoqurda phscoartac%o, Arq. Bras. Cardiol. 14:261, 1954. Massumi, R. A.: The congenital variety of the “subclnvian steal” syndrome, Circulation 28:1149, 1963. Daves, M. L., and Treger, A.: Vertebral grand larceny, Circulation 29:911. 1964. Bosniak, M. A.: Cervical arterial pathways associated with brachiocephalic occlusive disease, Am. J. Roentgenol. 91:1232, 1964. Grollman, J. H., and Horns, J. W.: The collateral circulation in coarctation of the aorta with a distal subclavian artery, Radio]. 83:622, 1964. Stewart, J. R., Kincaid, 0. W., and Edwards, J. E.: An atlas of vascular rings and related malformations of the aortic arch system, Springfield, Ill., 1964, Charles C Thomas, pp. 77-79. Pillsbury, R. C., Lower, R. R., and S~umway, N. E.: Atresia of the aortic arch. Circulation 30:749, 1964. Levine, S., Serfas, I,. S., and Rusinko, A.: Right aortic arch with subclavian steal syndrome (atresia of left common carotid and !eft subclavian arteries), Am. J. Surg. 3:632, 1966. Bradley, W. G.: Congenital aortic arch abnormalities with “subclavian steal” pattern of blood flow, Br. Heart J. 28:718, 1966.
Volume Numfxr
21.
25.
26.
27.
28.
29.
30.
31.
32.
33.
34.
83 6
Antia, A., and Ottesen, 0.: Collateral circulation in subclavian stenosis or atresia, Am. J. Cardiol. 18:599, 1966. Zetterquist, P.: A typical coarctation of the aorta with bilateral vertebral subclavian pathway, Stand. J. Thorac. Cardiovasc. Surg. 1:68, 1967. Replogle, R. L.: Left subclavian artery arising from obliterated ductus arteriosus, Ann. Thorac. Surg. 5:153, 1968. Lansing, A. M., and Murphy, J.: Origin of the left subclavian artery from the pulmonary artery with congenital subclavian steal, Ann. Thorac. Surg. 5:146, 1968. Love, J. W.: Varieties of congenital subclavian steal with and without the syndrome, J. Cardiovast. Surg. 9:3.58, 1968. Maranhao, V., Gooch, A., Ablaza, S., Nakhjavan, F., and Goldberg, H.: Congenital subclavian steal syndrome associated with right aortic arch, Br. Heart J. 30:875, 1968. Skalpe, I. O., and Semb, G. S.: Collateral circulation in subclavian atresia, Stand. J. Thorac. Cardiovasc. Surg. 3:207, 1969. Grossman, M., and Jacoby, W.: Right aortic arch and coarctation of the aorta, Dis. Chest. 56:158, 1969. Victorica, B. E., Van Mierop, L. H. S., and Elliott, L. P.: Right aortic arch associated with contralateral congenital subclavian steal syndrome, Radiology 108:582, 1970. Shuford, W. H., Sybers, R. G., and Schlant, R. C.: Right aortic arch with isolation of the left subclavian artery, Am. J. Roentgenol. 109:75, 1970. Morgan, J. R., Forker, A. D., Fosburg, R. G., Neugebauer, M. K., Rogers, A. K., and Bemiller, C.: Interruption of the aortic arch without a patent ductus arteriosus, Circulation 42:961, 1970.
Congenital
35.
36.
37.
38.
39.
40.
41.
42.
43. 44. 45.
sz~bcluvinn steal
x07
Becker, A. E., Becker, M. J., and Edwards, J. E.: Congenital anatomic potentials for subclavian steal, Chest 60:4, 1971. Massumi, R., Wiener, L., and Charif, I’.: The syndrome of cervical aorta, Am. J. Cardiol. 11:678. 1963. Ekestrom, S., and Retamal, E.: Haemodynamic condition in the subclavian steal syndrome, Stand. I. Thorac. Cardiovasc. Sure. 1:161. 1967. Newton, T., and Wylie, E.: Colliteral circula: tion associated with occlusion of the proximal subclavian and innominate arteries, Am. J. Roentgenol. 91:394, 1964. Di Giacomo, V., Neri, A., Carmenini, G., et al.: Fenomena della succlavia ladra. Basi fisiopatologiche e rassegna dei casi da anomalie vascolari congenite, Boll. Sot. Ital. Cardiol. 14:169, 1969. Abrams, H., editor: Angiography, Boston, 1971, Little, Brown & Company, Vol. 1, p. 338, fig. 21-6. Edwards, J. E., Clagett, 0. T., Drake, R. L., and Christensen, N. A.: The collateral circulation in coarctation of the aorta, Mayo Clin. Proc. 23:333, 1948. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The MacMillan CO., pp. 214-215. Felson, B., and Palayew, M.: The two types of right aortic arch, Radiology 81:74.5, 1963. Hastreiter, A. R., D’Cruz, I. .4., and Cantez, T.: Right sided aorta, Br. Heart J. 28:722, 1966. Stewart, J. R., Owing, W. K., and Titus, J. L.: Right aortic arch, plain film diagnosis and significance: 295 cases of right aortic arch with levocardia representing 10 years experience (1954-64) at the Mayo Clinic, Am. J. Roentgenol. 97:377, 1966.
A
lthough the subclavian steal is a well appreciated affliction of the elderly patient, it has seldom been reported in childhood and rarely recognized in the newborn. It results from occlusion of the proximal subclavian artery with subsequent retrograde blood Ilow to the distal subclavian by way of the ipsilateral vertebral artery. Review of the literature reveals the number of cases of congenital subclavian steal to be rapidly increasing, indicating that the occurrence is not as rare as previously thought. Our case demonstrates that the congenital subclavian steal may present in the newborn period and can be easily diagnosed. It discusses the typical clinical signs one might expect to encounter and describes the early development of collateral pathways. This review should alert the clinician to the associated cardiovascular lesions and present a glimpse of the natural history of the defect with a view toward timely surgical intervention. Case history When this 3,600 gram newborn developed severe congestive heart failure at 15 days of age he was transferred to Johns Hopkins Hospital for definitive cardiac evaluation.
(Ed-in.)
On physical examination he was a dusky infant with marked tachypnea. The pulse rate was 156 per minute and the respirations were 80 per minute. A discrepancy was noted in both the blood pressure determinations and the pulse amplitudes. The right arm and leg pressures were loo,‘65 mm. Hg and the left arm was 80/60 mm. Hg. The left brachial and radial pulses were indistinct to palpation. The precordium was hyperdynamic and there was a gallop rhythm. The first heart sound was normal and the second showed pulmonary accentuation. Neither a bruit nor a murmur was noted on auscultation. Gross hepatomegaly was found without evidence of splenic enlargement. Neurologic examination was normal. The initial electrocardiograms (ECG’s) showed biatrial enlargement and right ventricular hypertrophy with an indeterminate axis. There was right ventricular enlargement and pulmonary plethora on the chest film with a small posterior indentation of the esophagram. Cardiac catheterization was performed after 48 hours of successful anticongestive therapy. The hemodynamic data revealed a large ventricular septal defect with elevated pulmonary arteriolar resistance. The cineangiocardiogram demonstrated a ventricular septal defect and a right aortic arch. The left and right common carotid arteries were the first brachiocephalic branches (Fig. l/I). These were followed by the right subclavian artery which gave off the right vertebral and internal mammary arteries (Fig. 1B). A blind diverticulum opacified on the distal arch (Fig. 1C). Although the proximal left subclavian arterv could not be identified. filling of the distal left subclavian artery took place in a
From the Division of Pediatric Cardiology. Department Supported by United States Public Health Service grant Project grant 12 H201. Received for publication June 5. 1972. Reprint requests to: Daniel R. Pieroni. M.D., The Helen pital. Baltimore, Md. 21205.
Vol. 84, No. 6, pp. 801-807
December, 1972
of Pediatrics. 5ROl HE10718
B. Taussig
The Johns Hopkins and The Maternal
Children’s
Cardiac
Hospital. and Child
Center.
Johns
Baltimore. Md. Health Service
Hopkins
American Heart Journal
Has-
801
802
Pieroni,
Brodsky,
Am. Heart J. 1972 December,
and Rowe
Fig. I, A through D. A, Retrograde right subclavian arteriogram in the LAO position revealed the left @CC), right common carotid (RCC), and right subclavian arteries (RSC), respectively. B, Origin of the right internal mammary (RIM) and tortuous vertebral @VA) arteries from the right subclnvian (RSC). C, A conspicuous left subclavian filling. D, Late descending aortic arch diverticulum (D) was noted in the absence of proximal phase retrograde filling of the distal left subclavian (UC) and internal mammary arteries (MM) by way of the left vertebral artery &VA). The blind stump of the distal subclavian approximated the level of the diverticulum.
retrograde fashion by way of the left vertebral artery. Both the left subclavian and internal mammary arteries could be seen in the late frames of the cineangiogram (Fig. 1D). By 18 months of age a barium study continued to show a conspicuous posterior indentation (Fig. 2), while roentgenograms of the upper extremities were normal. Reassessment of the blood pressures re-
vealed a right arm pressure of 100/70 mm. Hg, leg pressures of 120/70 mm. Hg, and 90/70 mm. Hg in the left arm. Recatheterization in preparation for definitive cardiac surgery showed a large left-to-right shunt at the ventricular level with slight elevation of the pulmonary arteriotar resistapce. Biplane aortography again revealed the right aortic arch and clearly .1 I i/_II,,
volume~84
Ntmber
Congenital szcbclavian steal
6
Fig. 2. Lateral esophagram enlargement and a posterior the aortic diverticulum.
illustrates indentation
803
left atria1 caused by
allowed definition of the left common carotid artery as the first vessel, followed by the right common carotid and right subclavian arteries, respectively. On the distal arch a prominent blind diverticulum opacified and an unusual collateral artery was visualized reaching for the left arm (Fig. 3). Careful examination of the late phase films in the region of the left clavicle revealed retrograde filling of the left subclnvian artery by way of the left vertebral artery and thyrocervical trunk (Fig. 4). A subcristal ventricular septal defect was successfully closed at open heart surgery. No intervention was deemed necessary for the obliterated proximal subclnvian artery. The patient has done well since surgery.
Discussion
The subclavian steal phenomenon was first described by Contornir in 1960. When the proximal portion of the subclavian artery was obstructed, retrograde blood flow occurred in the ipsilateral vertebral artery into the low pressure distal subclavian system. Soon afterwards, Reivich and associates2reported a clinical syndrome produced from occlusion of the proximal subclavian artery. Blood siphoned from the basilar area of the brain by the vertebral artery produced symptoms of cerebral insufficiency.3~4 The combination of hemo-
Fig. 3, A and B. A, Anteroposterior view. B, Lateral view. Antegrade aortogram revealing a mirror image right aortic arch with left &CC), right (RCC) common carotid, and right subclavian (RSC) arteries in sequence. A prominent diverticulum, (D) opacified on the descending arch followed by an unusual collateral artery (CA). The proximal left subclavian artery failed to visualize.
dynamic phenomena and clinical symptoms was descriptively designated the “Subclavian Steal Syndrome” in honor of the piratical subclavian artery.j While atherosclerosis is by far the most
so4
Pieroni,
Brodsky,
Am. Heovt I. Dccembcr. 1972
and Rowe
Fig. 4. Magnified left cervical-clavicular retrograde filling of the left subclavian by way of the left vertebral artery thyrocervical trunk (TT).
area artery
(LVA)
with (LSC) and the
common cause,3 other etiologies have included thrombus formation,fi as well as spontaneous emboli from the left heart’ or following traunla.8 Folger and Shah9 reported 12 cases following Rlalock-Taussig anastomosis when the vertebral artery was left intact; Bloodwell and colleaguesrO described it during bypass with failure to perfuse the subclavian artery, and others inadvertently created the syndrome. Intrauterine acquired diseases such as the hypercalcemic syndrome may also cause proximal subclavian obstruction.“-I4 The 26 cases of angiographically documented congenital sul)clavian steal gleaned from the literature are summarized in Table I. Seventeen cases occurred with a right aortic arch and nine with some form of interruption of a left aortic arch. The point of interest is that those patients with a left aortic arch usually have a coarctation or total interruption of the aorta involving the origin of the subclavian, whereas patients with a right aortic arch often have only a localized hypoplasia, atresia, or isolation of the subclavian artery itself with a normal aortic arcll.20~3Z~35 A comprehensive study of the embryologic patterns has been published32 and the anatomic potentials for a subclavian steal outlined in great detail by Becker and co-workers.35 When the subclavian artery arises distal to the coarctation instead of being directly included in the constriction, then blood flow to the lower extremity may be supplied in retrograde fashion through the vertebral-subclavian system, a condition referred to as a “subclavian-aortic stea1.“28 Bilateral subclavian-aortic steals have been
observed in patients with interruption of the aortic arch when the left subclavian and anomalous right subclavian arteries empty distal to the aortic obstruction.18~25~34 As evidenced by our own experience, there are many more steals with coarctations and interruptions than have been reported. Classic neurologic symptoms have never been seen in a newborn patient and are rarely described in childhood. Only 5 patients with the congenital subclavian steal were clearly noted to have associated complaints during childhood.13~22*23~28J3 The voungest patient was 11 years old at the Eime of onset.13 Complaints are usually confined to parasthesias and exercise intolerance of the ipsilateral arm. Cerebral symptoms have not been seen in early childhood. Neurologic expression depends on the delicate balance between the siphon and other collateral circuits supplying the ipsilateral extremity.“s3’ The development of additional extracranial collateral channels in utero and the absence of atherosclerosis are thought to be the main reasons for the lack of symptoms in childhood.“0 This w-ould indicate that elective surgical intervention should not be considered before the second decade at the earliest. Repair shortly before the onset of adolescence should avoid growth retardation of the ipsilateral extremity. Fortunately, the anatomy is much more amenable to reconstructive surgery 1)); that time. Classical collateral pathways involving the internal and external carotids, cervicals, thyroids, occipitals, vertebrals, muscular branches of the vertebrals, epigastrics, and intercostals are frequently identified by angiography even in childhood.20~3s~3g Several collateral pathways were well estaljlished in our patient before a year of age. Besides the vertebral artery and thryocervical trunk, a prominent right internal mammary artery communicated with the left subclavian by way of the intercostal system. The unusual collateral artery distal to the diverticulum (Fig. 3) is similar to that described by Abrams40 in a patient with coarctation of the aorta. The ability of the infant to form early extensive collateral circuits is no longer in question. The parallel process has been well documented in infants with coarctation of the aorta.41 The pooling of data revealed that pa-
Congenital szibclnvian steal
Table I. Summary steal syndrome Author
of 1. 2. 3. 4. 5. 6.
and study
of cases of angiographically
Comnents
18
1954 1963 1964 1964 1964 1964
3,162 > 43 17 days
7. 8. 9. 10. 11. 12. 13.
Pillsbury?’ Levinez2 Bradley23 Antia% Antia Gerbe+ Zetterquistz5
1964 1966 1966 1966 1966 1967 1967
16 33 21 6 12 8 10
14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26.
Replogle2” Lansingz7 Lansing Lovez8 MaranhaosO Ska!pe30 Grossma# Vi!!arr3 Victorica3z Victorica Shufords3 Shuford Morgan”
1968 1968 1968 1968 1968 1969 1969 1969 1970 1970 1970 1970 1970
2 6 3/12 30 19 7 14 11
1972
*Abbreviations: anomalous
congenital subclavian
year
Viannar6 Massumi16~3~ Davesrr Bosniakr* Gro!!manrQ StewartzO
27. Pieroni
docwnented
805
+ -F T + + : 0 0
if
0 0 0 0 0 0 0 T 0 * 0 0
2,122 23 34 19
+" 0 0
15 days
0
0
tients with a congenital subclavian steal have a higher incidence of associated cardiovascular defects. When found in conjunction with a left arch and coarctation, lesions are confined largely to either a patent ductus arteriosus and/or a ventricular septal defect,42 whereas defects with a right arch depend on the particular type of right aortic arch. Several authors43-45 have firmly established the connection between intracardiac defects and a mirror-image right aortic arch. Although the number of cases was small, this correlation was observed in the literature reviewed by us. Tetralogy of Fallot was the most common lesion found with a mirror-image right arch (Table I). Prediction of an associated congenital heart lesion may be made upon identifying the orientation of the aorta with respect to
VSD,
steal
ASD, PDA Interrupted
hypoplastic
aortic
aortic
arch
Supravalve
aortic
stenosis
Hygercalcemic syndrome Interruption aortic arch, steals Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot
0 0 0 0 0 0 + 0 0 0 0 0 f
ASD = atria1 septal defect; Coarct. = coarctation pulmonary venous return; PDA = patent ductus
Right cervical arch VSD, PDA Bilateral subclavian
0 0 : 0 0
Tetralogy
isthmus,
bilateral
bilateral
subclaviaa
bilateral
subclavian
of Fallot
Supravalve aortic stenosis Tetralogy of Fallot VSD. PAPVR
i 0
of aorta; arteriosus;
Interruption steals VSD
aortic
arch,
L = left; R = right: Sx = symptoms; VSD = ventricular septal defect.
PAPVR
= partial
the esophagus. A retroesophageal right aortic arch produces a wide concavity on the barium-filled esophagus. This is not to be confused with the smaller indentation made by an anomalous right subclavian artery from a left aortic arch.32 Therefore, a barium study proves to be an invaluable diagnostic aid in patients with a right aortic arch. Careful examination of both brachial pulses as well as the femorals should be performed routinely on every newborn infant suspected of having congenital heart disease. In the presence of a right aortic arch this simple maneuver may be particularly rewarding. A perceptible difference in the brachial pulse amplitudes should alert the clinician to the possibility of a subclavian steal.
SO6
Pieroni,
Brodsky,
Am. Heart J. December, 1972
and Rowe
in a When aortography is performed patient with tetralogy of Fallot and a right . aortic arch, attention should be focused upon the mode of filling of the left subclavian artery as well as the specific anatomy of the brachiocephalic vessels. Failure to recognize the existence of an atretic proximal left subclavian may eventuate in a futile attempt at a Blalock-Taussig anastomosis with little hope for another palliative procedure from the left thoracotomy approach.
6.
7.
8.
9.
10.
Summary
This report is a reminder that the congenital subclavian steal can occur in the newborn and can be diagnosed clinically. Increased awareness of the phenomenon has been partially responsible for the recognition of 26 cases since the first described in 1960. This would indicate that the entity is not as rare as previously thought. Careful palpation of both brachial pulses as well as the femorals should be performed in all newborns, especially in infants with a right aortic arch or coarctation of the aorta. A discernible bra&al pulse discrepancy should alert the clinician to the possibility of a subclavian steal. While associated cardiovascular lesions are not uncommon, neurologic symptoms are infrequently seen in early childhood. Extensive extracranial collaterals and the .absence of atherosclerosis are thought to be the main reasons for the lack of symptoms in childhood. Elective surgical intervention is, therefore, not usually necessary before the second decade. The authors wish to extend thanks Haroutunian and Mrs. D. Mitchell for preparing the manuscript.
5.
11.
12.
13.
14.
15.
16.
17. 18.
to Dr. L. their aid in 19.
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2.
3.
4.
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Volume Numfxr
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31.
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33.
34.
83 6
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Congenital
35.
36.
37.
38.
39.
40.
41.
42.
43. 44. 45.
sz~bcluvinn steal
x07
Becker, A. E., Becker, M. J., and Edwards, J. E.: Congenital anatomic potentials for subclavian steal, Chest 60:4, 1971. Massumi, R., Wiener, L., and Charif, I’.: The syndrome of cervical aorta, Am. J. Cardiol. 11:678. 1963. Ekestrom, S., and Retamal, E.: Haemodynamic condition in the subclavian steal syndrome, Stand. I. Thorac. Cardiovasc. Sure. 1:161. 1967. Newton, T., and Wylie, E.: Colliteral circula: tion associated with occlusion of the proximal subclavian and innominate arteries, Am. J. Roentgenol. 91:394, 1964. Di Giacomo, V., Neri, A., Carmenini, G., et al.: Fenomena della succlavia ladra. Basi fisiopatologiche e rassegna dei casi da anomalie vascolari congenite, Boll. Sot. Ital. Cardiol. 14:169, 1969. Abrams, H., editor: Angiography, Boston, 1971, Little, Brown & Company, Vol. 1, p. 338, fig. 21-6. Edwards, J. E., Clagett, 0. T., Drake, R. L., and Christensen, N. A.: The collateral circulation in coarctation of the aorta, Mayo Clin. Proc. 23:333, 1948. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The MacMillan CO., pp. 214-215. Felson, B., and Palayew, M.: The two types of right aortic arch, Radiology 81:74.5, 1963. Hastreiter, A. R., D’Cruz, I. .4., and Cantez, T.: Right sided aorta, Br. Heart J. 28:722, 1966. Stewart, J. R., Owing, W. K., and Titus, J. L.: Right aortic arch, plain film diagnosis and significance: 295 cases of right aortic arch with levocardia representing 10 years experience (1954-64) at the Mayo Clinic, Am. J. Roentgenol. 97:377, 1966.