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Congenital Subclavian Steal Syndrome; Anatomy, Physiology, Pathology and Surgical Correction
DISCUSSION
In mirror-image dextrocardia, which is the most common condition of right-sided heart, the anterior-posterior relationship of the various parts of the heart are normal, but their right-to-Ieft orientation is reversed. The heart exists as a mirror image of normal. The venae cavae are on the left as is the systemic venous atrium. The venous
ventricle is anterior and the systemic, posterior. The
aortic arch is usually on the right. This condition is easily recognized because it is almost uniformly associated with some degree of abdominal situs inversus. The electrocardiographic changes are diagnostic: inverted P, QRS and T waves in lead 1. Rarely, this condition may be associated with cardiac abnormalities. According to Grand, 1 the associated cardiac malformations occur in 5 percent of cases and in these the most common anomalies are atrial septal defect, ventricular septal defect, pulmonary valvular stenosis, patent ductus arteriosus and tetralogy of Fallot. Mirror-image dextrocardia with situs inversus tends to be associated with simple and correctable cardiac malformations, whereas the rest of the cardiac malpositions are usually associated with more complicated and often uncorrectable anomalies. Billig8 reported 17 patients with mirror-image dextrocardia and cardiac anomalies recently. Of those, the cardiac malformations were complicated or uncorrectable in only three patients. The incidence of cardiac malformation in the general population is considered to be about 8 to 10 percent. 9 The frequency of occurrence of coarctation of the aorta among patients with congenital cardiac disease is 6 percent, 9 which amounts to a frequency of aortic coarctation among the general population of 1 in 6,000. If one takes the average frequency of mirror-image dextrocardia with situs inversus in the general population as 1:33,000,6 one can speculate and anticipate finding coarctation of the aorta in association with mirror-image dextrocardia and situs inversus occurring with an average frequency of 1: 198,000,000 persons. In other words, in our country with its present rate of 70,000 births per annum, one could expect to encounter this complex of the two anomalies in one person only once in about 2,828 years, which justified our designating it rare.
treatment of cardiac defects associated with variations in cardiac position. J Thorac Cardiovasc Surg 55:80, 1968 9 Edwards F: Congenital Cardiac Disease. Philadelphia, W B Saunders Co, 1962, pp 20-44
Congenital Subclavian Steal
Syndrome: Anatomy, Physiology, Pathology and Surgical Corredion* Roque PifarrB, M.D., F.C.C.P. and Richard G. Rouse, M.D.
A 22-year old man with right aortic arch and congenital subclavian steal syndrome secondary to hypoplastic malformation of the left subclavian artery proximal to the ostium of the vertebral artery is presented. Surgical correction of the hypoplastic segment was performed with the use of knitted Dacron graft. The patient bas subsequently been asymptomatic, and the graft • patent as demonstrated by postoperative aortography. The term subclavian steal syndrome was coined in 1961 after Reivich and co-workers 1 reported two such cases. 2 Early cases of this syndrome were reported in adults with subclavian obstruction secondary to atherosclerosis. However, in 1963 Massumi! presented the case of a six-year old boy with arteriographic evidence of congenital subclavian steal but without signs or symptoms of cerebral vascular insufficiency. In the litera°From the Department of Surgery, Loyola University Medical Center, Maywood, Dlinois, and the Cardiopulmonary Surgery Section, VA Hospital, Hines, Dlinois. Reprint requests: Dr. Pifarre, Lovola University Medtcal Center, Maywood, Illinois 00153
REFERENCES
1 Wood GW, Blalock A: Situs inversus totalis and disease of biliary tract: Survey of literature and report of case. Arch Surg 40:885, 1940 2 Lineback PE: An extraordinary case of situs inversus totalis. JAMA 75:1775, 1920 3 Johnson JR: Situs inversus with associated abnonnalities: Review of literature and report of three cases. Arch Surg 58: 149, 1949 4 Cleveland M: Situs inversus viscerum: Anatomic study. Arch Surg 13:342, 1926 5 Le Wald LT: Complete transposition of the viscera. A report of 29 cases with remarks on etiology. JAMA 84:261, 1925 6 Sherk HH: Total transposition of the viscera. Surg Gyneeol Obstet 35:53, 1922 7 Grant RP: The syndrome of dextroversion of the heart. Circulation 18:25, 1958 8 Billig OM, Hallman GL, Bloodwell RD, et al: Surgical
CHEST, 66: 3, SEPTEMBER, 1974
FIGURE 1. Aortic root injection via the right subclavian artery demonstrates a right aortic arch with a left common carotid, right common carotid and right subclavian arteries originating from the aorta but no visualization of the ieft subclavian artery.
CONGENITAL SUBCLAVIAN STEAL SYNDROME 299
L.\lA. R.C.C.A. R.S.A. ~~", ~~
-Fibrous cord
-Dacron graft ' ....~ L.C.C.A.
FIGURE 2. The left subclavian artery fllling late and retrograde from the left vertebral artery.
ture, we have found 27 cases of congenital subclavian steal. 8 - 20 TWO·,12 of these had the subclavian steal syndrome and were corrected surgically. A third case of surgical correction of congenital subclavian steal syndrome is presented below with a discussion of the literature review. CASE
REPoRT
This 22-year-old white man was admitted with the chief complaint of weakness in his left hand on exercise for the past four years. Recently he had experienced episodes of dizziness with strenuous work and use of his left hand. Physical examination was normal except for a blood pressure of 70/50 in the left arm with pressures of 104/58 in the right arm and 140/80 in both legs. No bruits were heard in the neclc. The pulses were: both carotids 3+ (scale of 1 to 4), right brachial 4+, left brachial 1+, right radial 4+, left radial 1+, and both femorals 3+. Chest x-ray film revealed a right aortic arch. An aortic root injection showed a right aortic arch, with left common carotid, right common carotid and right subclavian branches in that order (Fig 1). The left subclavian artery did not visualize immediately but filled late and retrograde from the left vertebral artery (Fig 2). A thoracotomy was performed through a midstemal splitting incision. The left pleural space was opened and' the junction of the left subclavian and left vertebral arteries was located. There was a fibrous cord 1.5 em in length from the aortic arch to the subclavian-vertebral junction (Fig 3). The subclavian artery distal to the vertebral artery was normal. An 8 mm knitted Dacron graft was anastamosed end-to-side distal to the junction of the cord to the left subclavian artery with 5-0 silky Polydek suture. The graft was anastamosed end-to-side to the ascending aorta with 4-0 silky Polydek (Fig 3). Postoperatively, the patient has been asymptomatic. The postoperative aortogram shows the graft to be patent between the ascending aorta and the second part of the subclavian artery with a normal amount of blood flow to the left arm (Fig 4). The subclavian steal is no longer present.
•
PlFARRE, ROUSE
FIGURE 3. The 8 mm knitted Dacron graft anastamosed from the ascending aorta to the junction of the fibrous cord and left subclavian artery at the origin of the left vertebral artery. RSA, right subclavian artery; RCCA, right common carotid artery; LVA, left vertebral artery; LCCA, left common carotid artery; LSA, left subclavian· artery; PA, main pulmonary artery. DISCUSSION
In 1971, Becker and colleagues 21 presented a comprehensive review of the congenital anatomic potentials for subclavian steal. With this classification, it is possible to describe all cases of congenital subclavian steal based on: 1) position of the aortic arch, whether right or left, 2) presence or absence of posterior esophageal compression, and 3) the subclavian system which bears a low
FIGURE 4. Postoperative aortogram showing the Dacron graft to be patent and filling the left subclavian and left vertebral arteries from the ascending aorta. The subclavian steal is no longer present.
CHEST, 66: 3, SEPTEMBER, 1974
Table l---€ompariwn 01 Sur«ieal Correction 01 Coqeniaal Subelm1ian Steal Syndrome
Levine, et al4
Loven
History
33 yr old white woman. Spots before eyes and light-headedness since child; numbness and pain left arm; absent pulses for 14 years; left arm 1.5 cm shorter than right arm.
30 yr old white woman. Dizziness with exercise of left arm; intermittent numbness and easy fatigability left arm for several weeks.
with strenuous work and use of left hand; weakness of left hand with exercise for 5 years.
Physical
Right arm 110/70; left arm 80/70 left axillary, brachial, and radial pulses absent; decreased L. Carotid pulse; right arch with RSA and RCC both off RINA; RINA the only branch off the aorta; retrograde fill of LCC and LVA with distal LSA filling from LVA and/or LCC retrograde.
Right arm 136/84; left arm 104/82; right arch; left desc aorta; RSA, RCC, LCC, but no LSA off arch; retrograde filling LVA into distal LSA.
Right arm 104/58; left arm 70/50 legs 140/80; decreased pulses left arm; right arch; LCC, RCC, RSA; but no LSA off arch; retrograde filling LVA into distal LSA.
Operation and Results
Median sternotomy; atretic cord from arch to junct LCC and LSA containing atretic LCC and LSA; ligamentum art. from main P.A. to LSA: 10 mm knitted Dacron graft from junct. LCC and LSA to ascending aorta; P08top: no pulses palpable in left arm or LCC; no pain or numbness in left arm; LCC and LSA visualized simultaneously with aorta; graft not visualized, but assumed patent; blood pressure, left arm 90/60, amelioration symptoms of lightheadedness and spots before eyes.
Left posterolateral thoracotomy resect 4th rib; coart. LSA from origin to LSA-LVA junct; distal LSA normal; ligamentum arterioswn compressing esophagus; 6 mm knitted Dacron graft replaced proximal LSA with the coarctation; P08top: aortogr&m at three months shows graft patent; free of symptoms blood pressure, right arm, 120/80, left arm, 110/92
Midsternotomy; fibrous cord from descending thoracic aorta to LSALVA junction; no ligamentum arterioswn; distal LSA normal; 8 mm knitted Dacron graft ascending aorta to junction of cord-LSA: cord intact for support; P08top: aortogr&m; graft patent, normal blood flow to LSA; no steal present; patient asymptomatic; blood pressures are equal in both arms.
RSA, right subclavian artery; RCC, right common carotid artery. LSA, left subclavian artery; LCC, left common carotid artery. pressure. If the congenital subclavian steal is of such a magnitude that it causes transient cerebral ischemic attacks, the condition is designated as congenital subclavian steal syndrome. The cerebral symptoms of this syndrome are vertigo, dizziness, blurred vision, and headaches. The arm symptoms of this syndrome are pain, cramps, numbness, weak pulses, supraclavicular bruit and blood pressure diHerences between the two arms. 23 The occurrence of the syndrome in the congenital form of subclavian steal has been reported only in adults. 4 •12 This is most likely because of the development of adequate collateral circulation before birth. 13•21 The patient may take years to develop the syndrome from the gradual development of atherosclerotic changes which compromise the cerebral vessels in addition to the underlying congenital subclavian steal. s Therefore, it might be suggested that congenital subclavian steal need not be corrected unless it is associated with other congenital cardiovascular malformations or until the syndrome develops, which may take years. As far as we are aware, this is the 28th reported case of a subclavian steal on a congenital basis. 3-20 It is the 11 th reported case of hypoplastic malformation of the left subclavian artery proximal to the ostium of the vertebral artery.12-20 This is only the third reported case (Table 1) of a surgical correction of a congenital subclavian steal syndrome. 4 - 12 Although congenital
CHEST, 66: 3, SEPTEMBER, 1974
Present Case Report 22 yr old white man. Dizziness
P.A. pulmonary artery; RINA, right innominate artery. LVA, left vertebral artery. subclavian steal is associated with an increased incidence of cardiac malformation,22 cardiac catheterization studies were not performed in this patient because he was asymptomatic.
fu:FEBENCES 1 Reivich M, Holling HE, Roberts B, et al: Reversal of blood flow through the vertebral artery and its effect on cerebral circulation. New Eng J Moo 265:878, 1961 2 Editorial: A new vascular syndrome-"the subclavian steal." N Eng! J Moo 265:912, 1961 3 Massumi RA: The congenital variety of the "subclavian steal" syndrome. Circulation 28:1149,1963 4 Levine S, Serfas LS, Rusinko A: Right aortic arch with subclavian steal syndrome (atresia of left common carotid and left subclavian arteries). Am J Surg 111:632, 1966 5 Lansing AM, Murphy J: Origin of the left subclavian artery from the pulmonary artery with congenital subclavian steal. Surgical implications in cyanotic patients. Ann Thorac Surg 5:146,1968 6 Clagett OT, Kirklin }W, Edwards IE: Anatomic variations and pathologic changes in coarctation of the aorta. A study of 124 cases. Surg Gynec Obstet 98: 103, 1954 7 Bosniak MA: Cervical arterial pathways associated with brachiocephalic occlusive disease. Am J Roentgenol 91: 1232, 1964 8 Gerber N: Congenital atresia of the subclavian artery, producing the "subclavian steal syndrome." Am J Dis Child 113:709, 1967 9 Maranhao V, Gooch AS, Ablaza sec, et a1: Congenital
CONGENITAL SUBCLAVIAN STEAL SYNDROME 301
10 11 12 13 14
15 16
17
18 19 20 21 22 23
subclavian steal syndrome associated with right aortic arch. Br Heart J 30:875, 1968 Bradley WG: Congenital aortic arch abnormalities with the "subclavian steal" pattern of blood flow. Br Heart J 28:718, 1966 Masswni R, Wiener L, Charif P: The syndrome of cervical aorta. Report of a case and review of the previous cases. Am J Cardiolll:678, 1963 Love JW: Varieties of congenital subclavian steal with and without the syndrome. J Cardiovac Surg 9:358, 1968 Shuford WH, Sybers RG, Schlant RC: Subclavian steal syndrome in right aortic arch with isolation of the left subclavian artery. Am Heart J 82:98, 1971 Skalpe 10, Semb GS: Collateral circluation in subclavian atresia. Report of a case with right aortic arch and tetralogy of Fallot. Scand J Thorac Cardiovasc Surg 3:207,1969 Grossman M, Jacoby WJ Jr: Right aortic arch and coarctation of the aorta. Dis Chest 56: 158, 1969 Antia AJ, Ottensen OE: Collateral circulation in subclavian stenosis or atresia. Angiographic demonstration of retrograde vertebral-subclavian flow in two cases with right aortic arch. Am J Cardiol 18:599, 1966 Oi Giacomo V, Neri A, Carmenini G, et al: Fenomena della succlavia ladra. Basi 6siopatologiche e rassegna dei case da anomalie vascolari congenite. Boll Soc ltal Cardiol 14:169,1969 Victorica BE, Van Mierop LHS, Elliott LP: Right aortic arch associated with contralateral congenital subclavian steal syndrome. Radiology 108:582, 1970 Ghon A: Ueber eine seltene Entwicklungstorung des Gefassystems. Verhandl D Deutsch Path Gesellsch 12: 242, 1908. Quoted by Shuford, WH, et al20 Shuford WH, Sybers RG, Schlant RC: Right aortic arch with isolation of the left subclavian. artery. Am J Roentgenoll09:75, 1970 Becker AE, Becker AJ, Edwards JE: Congenital anatomic potentials for subclavian steal. Chest 60:4, 1971 Patel A, Toole JF: Subclavian steal syndrome-reversal of cephalic blood flow. Medicine 44:289,1965 Skalpe 10, Semb GS: Congenital bilateral "subclavian steal" blood flow pattern. Scand J Thorac Cardiovasc Surg 4:153, 1970: reviewing Gasul Arcilla and Lev, 19662.: and LeRoux and Williams. 1968.28
atrial myxomas, while rare may present in a L eftvariety of fonns including fever of unknown origin, symptoms related to peripheral embolization, acute congestive heart failure, symptoms indistinguishable from collagen-vascular diseases, or symptoms referable to the mechanical movements of the mass within the left atrium and left ventricle. We report a 12-year-old girl in whom left atrial myxoma occurred with clinical findings suggestive of acute rheumatic fever and mitral insufficiency. CASE REpORT
A 12-year-old Spanish-American girl was first seen at age 11 years for evaluation of frequent, severe headaches of five years' duration. A throat culture obtained two weelcs prior to her initial visit was positive for Lancefield group A-beta hemolytic Streptococcus, and oral penicillin therapy was provided. She had previously been in good health and her family history was noncontributory. Initial physical examination revealed a chronically-ill appearing girl with a 2/6 apical pansystolic murmur interPreted as that of mitral insufficiency. She was anemic, and had an erythrocyte sedimentation rate ( ESR) of 52 mm/hr, a "positive" C-reactive protein (CRP), and an anti-streptolysin-O (ASO) titer of 166 Todd units. A preswnptive diagnosis of rhewnatic fever with carditis was made and she was placed on daily penicillin prophylaxis.
I I u
Left Atrial Myxoma Presenting as Rheumatic Fever*
...
Randall H. Lortscher, M.D.;" Warren H. Toews, M.D.;" lames I. Nora, M.D.;-- Robert R .Wolfe, M.D.;t and Richard D. Spangler, M.D.,: F.C.C.P.
w.
A 12-year-01d girl, with a diagnosis of previous rheumatic fever and murmur of mitral insufficiency, is reported. Echocardiognphic examination performed to assess mitnl valve function. Documentation of left atrial myxoma end its subsequent growth over a threemonth period recorded.
w.
-From the Departments of Medicine and Pediatrics, University of Colorado Medical Center, Denver. IIFellow, Pediatric Cardiology. "Professor of Pediatrics. t Assistant Professor of Pediatrics. :j:Assistant Clinical Professor. Reprint requests: Tk. Nora, 4200 East Ninth, Denver 80220
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FIGURE 1. ECG and cardiac catheterization data. The cineangiogram shows the myxoma outlined within the left atriwn. Catheterization results demonstrate the obstructive quality of the twnor and corresponding increased pulmonary artery wedge pressure.
CHEST, 66: 3, SEPTEMBER, 1974
In mirror-image dextrocardia, which is the most common condition of right-sided heart, the anterior-posterior relationship of the various parts of the heart are normal, but their right-to-Ieft orientation is reversed. The heart exists as a mirror image of normal. The venae cavae are on the left as is the systemic venous atrium. The venous
ventricle is anterior and the systemic, posterior. The
aortic arch is usually on the right. This condition is easily recognized because it is almost uniformly associated with some degree of abdominal situs inversus. The electrocardiographic changes are diagnostic: inverted P, QRS and T waves in lead 1. Rarely, this condition may be associated with cardiac abnormalities. According to Grand, 1 the associated cardiac malformations occur in 5 percent of cases and in these the most common anomalies are atrial septal defect, ventricular septal defect, pulmonary valvular stenosis, patent ductus arteriosus and tetralogy of Fallot. Mirror-image dextrocardia with situs inversus tends to be associated with simple and correctable cardiac malformations, whereas the rest of the cardiac malpositions are usually associated with more complicated and often uncorrectable anomalies. Billig8 reported 17 patients with mirror-image dextrocardia and cardiac anomalies recently. Of those, the cardiac malformations were complicated or uncorrectable in only three patients. The incidence of cardiac malformation in the general population is considered to be about 8 to 10 percent. 9 The frequency of occurrence of coarctation of the aorta among patients with congenital cardiac disease is 6 percent, 9 which amounts to a frequency of aortic coarctation among the general population of 1 in 6,000. If one takes the average frequency of mirror-image dextrocardia with situs inversus in the general population as 1:33,000,6 one can speculate and anticipate finding coarctation of the aorta in association with mirror-image dextrocardia and situs inversus occurring with an average frequency of 1: 198,000,000 persons. In other words, in our country with its present rate of 70,000 births per annum, one could expect to encounter this complex of the two anomalies in one person only once in about 2,828 years, which justified our designating it rare.
treatment of cardiac defects associated with variations in cardiac position. J Thorac Cardiovasc Surg 55:80, 1968 9 Edwards F: Congenital Cardiac Disease. Philadelphia, W B Saunders Co, 1962, pp 20-44
Congenital Subclavian Steal
Syndrome: Anatomy, Physiology, Pathology and Surgical Corredion* Roque PifarrB, M.D., F.C.C.P. and Richard G. Rouse, M.D.
A 22-year old man with right aortic arch and congenital subclavian steal syndrome secondary to hypoplastic malformation of the left subclavian artery proximal to the ostium of the vertebral artery is presented. Surgical correction of the hypoplastic segment was performed with the use of knitted Dacron graft. The patient bas subsequently been asymptomatic, and the graft • patent as demonstrated by postoperative aortography. The term subclavian steal syndrome was coined in 1961 after Reivich and co-workers 1 reported two such cases. 2 Early cases of this syndrome were reported in adults with subclavian obstruction secondary to atherosclerosis. However, in 1963 Massumi! presented the case of a six-year old boy with arteriographic evidence of congenital subclavian steal but without signs or symptoms of cerebral vascular insufficiency. In the litera°From the Department of Surgery, Loyola University Medical Center, Maywood, Dlinois, and the Cardiopulmonary Surgery Section, VA Hospital, Hines, Dlinois. Reprint requests: Dr. Pifarre, Lovola University Medtcal Center, Maywood, Illinois 00153
REFERENCES
1 Wood GW, Blalock A: Situs inversus totalis and disease of biliary tract: Survey of literature and report of case. Arch Surg 40:885, 1940 2 Lineback PE: An extraordinary case of situs inversus totalis. JAMA 75:1775, 1920 3 Johnson JR: Situs inversus with associated abnonnalities: Review of literature and report of three cases. Arch Surg 58: 149, 1949 4 Cleveland M: Situs inversus viscerum: Anatomic study. Arch Surg 13:342, 1926 5 Le Wald LT: Complete transposition of the viscera. A report of 29 cases with remarks on etiology. JAMA 84:261, 1925 6 Sherk HH: Total transposition of the viscera. Surg Gyneeol Obstet 35:53, 1922 7 Grant RP: The syndrome of dextroversion of the heart. Circulation 18:25, 1958 8 Billig OM, Hallman GL, Bloodwell RD, et al: Surgical
CHEST, 66: 3, SEPTEMBER, 1974
FIGURE 1. Aortic root injection via the right subclavian artery demonstrates a right aortic arch with a left common carotid, right common carotid and right subclavian arteries originating from the aorta but no visualization of the ieft subclavian artery.
CONGENITAL SUBCLAVIAN STEAL SYNDROME 299
L.\lA. R.C.C.A. R.S.A. ~~", ~~
-Fibrous cord
-Dacron graft ' ....~ L.C.C.A.
FIGURE 2. The left subclavian artery fllling late and retrograde from the left vertebral artery.
ture, we have found 27 cases of congenital subclavian steal. 8 - 20 TWO·,12 of these had the subclavian steal syndrome and were corrected surgically. A third case of surgical correction of congenital subclavian steal syndrome is presented below with a discussion of the literature review. CASE
REPoRT
This 22-year-old white man was admitted with the chief complaint of weakness in his left hand on exercise for the past four years. Recently he had experienced episodes of dizziness with strenuous work and use of his left hand. Physical examination was normal except for a blood pressure of 70/50 in the left arm with pressures of 104/58 in the right arm and 140/80 in both legs. No bruits were heard in the neclc. The pulses were: both carotids 3+ (scale of 1 to 4), right brachial 4+, left brachial 1+, right radial 4+, left radial 1+, and both femorals 3+. Chest x-ray film revealed a right aortic arch. An aortic root injection showed a right aortic arch, with left common carotid, right common carotid and right subclavian branches in that order (Fig 1). The left subclavian artery did not visualize immediately but filled late and retrograde from the left vertebral artery (Fig 2). A thoracotomy was performed through a midstemal splitting incision. The left pleural space was opened and' the junction of the left subclavian and left vertebral arteries was located. There was a fibrous cord 1.5 em in length from the aortic arch to the subclavian-vertebral junction (Fig 3). The subclavian artery distal to the vertebral artery was normal. An 8 mm knitted Dacron graft was anastamosed end-to-side distal to the junction of the cord to the left subclavian artery with 5-0 silky Polydek suture. The graft was anastamosed end-to-side to the ascending aorta with 4-0 silky Polydek (Fig 3). Postoperatively, the patient has been asymptomatic. The postoperative aortogram shows the graft to be patent between the ascending aorta and the second part of the subclavian artery with a normal amount of blood flow to the left arm (Fig 4). The subclavian steal is no longer present.
•
PlFARRE, ROUSE
FIGURE 3. The 8 mm knitted Dacron graft anastamosed from the ascending aorta to the junction of the fibrous cord and left subclavian artery at the origin of the left vertebral artery. RSA, right subclavian artery; RCCA, right common carotid artery; LVA, left vertebral artery; LCCA, left common carotid artery; LSA, left subclavian· artery; PA, main pulmonary artery. DISCUSSION
In 1971, Becker and colleagues 21 presented a comprehensive review of the congenital anatomic potentials for subclavian steal. With this classification, it is possible to describe all cases of congenital subclavian steal based on: 1) position of the aortic arch, whether right or left, 2) presence or absence of posterior esophageal compression, and 3) the subclavian system which bears a low
FIGURE 4. Postoperative aortogram showing the Dacron graft to be patent and filling the left subclavian and left vertebral arteries from the ascending aorta. The subclavian steal is no longer present.
CHEST, 66: 3, SEPTEMBER, 1974
Table l---€ompariwn 01 Sur«ieal Correction 01 Coqeniaal Subelm1ian Steal Syndrome
Levine, et al4
Loven
History
33 yr old white woman. Spots before eyes and light-headedness since child; numbness and pain left arm; absent pulses for 14 years; left arm 1.5 cm shorter than right arm.
30 yr old white woman. Dizziness with exercise of left arm; intermittent numbness and easy fatigability left arm for several weeks.
with strenuous work and use of left hand; weakness of left hand with exercise for 5 years.
Physical
Right arm 110/70; left arm 80/70 left axillary, brachial, and radial pulses absent; decreased L. Carotid pulse; right arch with RSA and RCC both off RINA; RINA the only branch off the aorta; retrograde fill of LCC and LVA with distal LSA filling from LVA and/or LCC retrograde.
Right arm 136/84; left arm 104/82; right arch; left desc aorta; RSA, RCC, LCC, but no LSA off arch; retrograde filling LVA into distal LSA.
Right arm 104/58; left arm 70/50 legs 140/80; decreased pulses left arm; right arch; LCC, RCC, RSA; but no LSA off arch; retrograde filling LVA into distal LSA.
Operation and Results
Median sternotomy; atretic cord from arch to junct LCC and LSA containing atretic LCC and LSA; ligamentum art. from main P.A. to LSA: 10 mm knitted Dacron graft from junct. LCC and LSA to ascending aorta; P08top: no pulses palpable in left arm or LCC; no pain or numbness in left arm; LCC and LSA visualized simultaneously with aorta; graft not visualized, but assumed patent; blood pressure, left arm 90/60, amelioration symptoms of lightheadedness and spots before eyes.
Left posterolateral thoracotomy resect 4th rib; coart. LSA from origin to LSA-LVA junct; distal LSA normal; ligamentum arterioswn compressing esophagus; 6 mm knitted Dacron graft replaced proximal LSA with the coarctation; P08top: aortogr&m at three months shows graft patent; free of symptoms blood pressure, right arm, 120/80, left arm, 110/92
Midsternotomy; fibrous cord from descending thoracic aorta to LSALVA junction; no ligamentum arterioswn; distal LSA normal; 8 mm knitted Dacron graft ascending aorta to junction of cord-LSA: cord intact for support; P08top: aortogr&m; graft patent, normal blood flow to LSA; no steal present; patient asymptomatic; blood pressures are equal in both arms.
RSA, right subclavian artery; RCC, right common carotid artery. LSA, left subclavian artery; LCC, left common carotid artery. pressure. If the congenital subclavian steal is of such a magnitude that it causes transient cerebral ischemic attacks, the condition is designated as congenital subclavian steal syndrome. The cerebral symptoms of this syndrome are vertigo, dizziness, blurred vision, and headaches. The arm symptoms of this syndrome are pain, cramps, numbness, weak pulses, supraclavicular bruit and blood pressure diHerences between the two arms. 23 The occurrence of the syndrome in the congenital form of subclavian steal has been reported only in adults. 4 •12 This is most likely because of the development of adequate collateral circulation before birth. 13•21 The patient may take years to develop the syndrome from the gradual development of atherosclerotic changes which compromise the cerebral vessels in addition to the underlying congenital subclavian steal. s Therefore, it might be suggested that congenital subclavian steal need not be corrected unless it is associated with other congenital cardiovascular malformations or until the syndrome develops, which may take years. As far as we are aware, this is the 28th reported case of a subclavian steal on a congenital basis. 3-20 It is the 11 th reported case of hypoplastic malformation of the left subclavian artery proximal to the ostium of the vertebral artery.12-20 This is only the third reported case (Table 1) of a surgical correction of a congenital subclavian steal syndrome. 4 - 12 Although congenital
CHEST, 66: 3, SEPTEMBER, 1974
Present Case Report 22 yr old white man. Dizziness
P.A. pulmonary artery; RINA, right innominate artery. LVA, left vertebral artery. subclavian steal is associated with an increased incidence of cardiac malformation,22 cardiac catheterization studies were not performed in this patient because he was asymptomatic.
fu:FEBENCES 1 Reivich M, Holling HE, Roberts B, et al: Reversal of blood flow through the vertebral artery and its effect on cerebral circulation. New Eng J Moo 265:878, 1961 2 Editorial: A new vascular syndrome-"the subclavian steal." N Eng! J Moo 265:912, 1961 3 Massumi RA: The congenital variety of the "subclavian steal" syndrome. Circulation 28:1149,1963 4 Levine S, Serfas LS, Rusinko A: Right aortic arch with subclavian steal syndrome (atresia of left common carotid and left subclavian arteries). Am J Surg 111:632, 1966 5 Lansing AM, Murphy J: Origin of the left subclavian artery from the pulmonary artery with congenital subclavian steal. Surgical implications in cyanotic patients. Ann Thorac Surg 5:146,1968 6 Clagett OT, Kirklin }W, Edwards IE: Anatomic variations and pathologic changes in coarctation of the aorta. A study of 124 cases. Surg Gynec Obstet 98: 103, 1954 7 Bosniak MA: Cervical arterial pathways associated with brachiocephalic occlusive disease. Am J Roentgenol 91: 1232, 1964 8 Gerber N: Congenital atresia of the subclavian artery, producing the "subclavian steal syndrome." Am J Dis Child 113:709, 1967 9 Maranhao V, Gooch AS, Ablaza sec, et a1: Congenital
CONGENITAL SUBCLAVIAN STEAL SYNDROME 301
10 11 12 13 14
15 16
17
18 19 20 21 22 23
subclavian steal syndrome associated with right aortic arch. Br Heart J 30:875, 1968 Bradley WG: Congenital aortic arch abnormalities with the "subclavian steal" pattern of blood flow. Br Heart J 28:718, 1966 Masswni R, Wiener L, Charif P: The syndrome of cervical aorta. Report of a case and review of the previous cases. Am J Cardiolll:678, 1963 Love JW: Varieties of congenital subclavian steal with and without the syndrome. J Cardiovac Surg 9:358, 1968 Shuford WH, Sybers RG, Schlant RC: Subclavian steal syndrome in right aortic arch with isolation of the left subclavian artery. Am Heart J 82:98, 1971 Skalpe 10, Semb GS: Collateral circluation in subclavian atresia. Report of a case with right aortic arch and tetralogy of Fallot. Scand J Thorac Cardiovasc Surg 3:207,1969 Grossman M, Jacoby WJ Jr: Right aortic arch and coarctation of the aorta. Dis Chest 56: 158, 1969 Antia AJ, Ottensen OE: Collateral circulation in subclavian stenosis or atresia. Angiographic demonstration of retrograde vertebral-subclavian flow in two cases with right aortic arch. Am J Cardiol 18:599, 1966 Oi Giacomo V, Neri A, Carmenini G, et al: Fenomena della succlavia ladra. Basi 6siopatologiche e rassegna dei case da anomalie vascolari congenite. Boll Soc ltal Cardiol 14:169,1969 Victorica BE, Van Mierop LHS, Elliott LP: Right aortic arch associated with contralateral congenital subclavian steal syndrome. Radiology 108:582, 1970 Ghon A: Ueber eine seltene Entwicklungstorung des Gefassystems. Verhandl D Deutsch Path Gesellsch 12: 242, 1908. Quoted by Shuford, WH, et al20 Shuford WH, Sybers RG, Schlant RC: Right aortic arch with isolation of the left subclavian. artery. Am J Roentgenoll09:75, 1970 Becker AE, Becker AJ, Edwards JE: Congenital anatomic potentials for subclavian steal. Chest 60:4, 1971 Patel A, Toole JF: Subclavian steal syndrome-reversal of cephalic blood flow. Medicine 44:289,1965 Skalpe 10, Semb GS: Congenital bilateral "subclavian steal" blood flow pattern. Scand J Thorac Cardiovasc Surg 4:153, 1970: reviewing Gasul Arcilla and Lev, 19662.: and LeRoux and Williams. 1968.28
atrial myxomas, while rare may present in a L eftvariety of fonns including fever of unknown origin, symptoms related to peripheral embolization, acute congestive heart failure, symptoms indistinguishable from collagen-vascular diseases, or symptoms referable to the mechanical movements of the mass within the left atrium and left ventricle. We report a 12-year-old girl in whom left atrial myxoma occurred with clinical findings suggestive of acute rheumatic fever and mitral insufficiency. CASE REpORT
A 12-year-old Spanish-American girl was first seen at age 11 years for evaluation of frequent, severe headaches of five years' duration. A throat culture obtained two weelcs prior to her initial visit was positive for Lancefield group A-beta hemolytic Streptococcus, and oral penicillin therapy was provided. She had previously been in good health and her family history was noncontributory. Initial physical examination revealed a chronically-ill appearing girl with a 2/6 apical pansystolic murmur interPreted as that of mitral insufficiency. She was anemic, and had an erythrocyte sedimentation rate ( ESR) of 52 mm/hr, a "positive" C-reactive protein (CRP), and an anti-streptolysin-O (ASO) titer of 166 Todd units. A preswnptive diagnosis of rhewnatic fever with carditis was made and she was placed on daily penicillin prophylaxis.
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Left Atrial Myxoma Presenting as Rheumatic Fever*
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Randall H. Lortscher, M.D.;" Warren H. Toews, M.D.;" lames I. Nora, M.D.;-- Robert R .Wolfe, M.D.;t and Richard D. Spangler, M.D.,: F.C.C.P.
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A 12-year-01d girl, with a diagnosis of previous rheumatic fever and murmur of mitral insufficiency, is reported. Echocardiognphic examination performed to assess mitnl valve function. Documentation of left atrial myxoma end its subsequent growth over a threemonth period recorded.
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-From the Departments of Medicine and Pediatrics, University of Colorado Medical Center, Denver. IIFellow, Pediatric Cardiology. "Professor of Pediatrics. t Assistant Professor of Pediatrics. :j:Assistant Clinical Professor. Reprint requests: Tk. Nora, 4200 East Ninth, Denver 80220
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FIGURE 1. ECG and cardiac catheterization data. The cineangiogram shows the myxoma outlined within the left atriwn. Catheterization results demonstrate the obstructive quality of the twnor and corresponding increased pulmonary artery wedge pressure.
CHEST, 66: 3, SEPTEMBER, 1974