Coarctation of the Aorta Proximal to the Left Subclavian Artery ...

ANNALS OF SURGERYAugust 1957 No. 2

Coarctation of the Aorta Proximal to the Left SubclavianArtery: Experience with Six Surgical Cases *

F. HENRY ELLIS, JR., M.D., 0. THERON CLAGETT, M.D.

Section of Surgery,Mayo Clinic and Mayo Foundation,**

Rochester, Minnesota

RESECTION of the narrowed segment withend-to-end anastomosis of the divided aortais now a well standardized procedure forthe treatment of coarctation of the aorta.Experience with large numbers of cases hasshown that this procedure is attended byan acceptably low mortality rate.5 9 For-tunately in the great majority of cases thesite of coarctation is distal to the subclavianartery in a position that is amenable toready surgical attack.6

In certain instances, however, the coarcta-tion may involve the left subclavian arteryor lie proximal to it. Although the clinicalrecognition of this type of lesion has beenappreciated for some time," 2, 4, 13 little hasappeared in the surgical literature concern-ing the management of such cases, particu-larly those in which the coarcted segmentlies between the left common carotid andleft subclavian arteries. Bing-and co-work-ers have described a case in which surgicaltreatment was successful. More recentlyEfskind and Sanderud described an un-usual case of coarctation in two sites, oneproximal and one distal to the left sub-clavian artery. This patient was treated suc-

* Submitted for publication October 22, 1956.* * The Mayo Foundation, Rochester, Minnesota,

is a part of the Graduate School of the Universityof Minnesota.

145

cessfully by surgical means. In a recent dis-cussion of coarctation of the aorta, O'Sul-livan and Glenn included cases in whichthe site of coarctation was proximal to theleft subclavian artery in the group of in-operable conditions.The purpose of this report is to summa-

rize briefly our experience in the surgicalmanagement of six patients with coarcta-tion of the aorta proximal to the left sub-clavian artery, all but one of whom sur-vived operative correction of their defect(Table 1). Of 223 patients operated on forcoarctation at the Mayo Clinic betweenJanuary, 1946, and June 15, 1956, only thesesix had coarctation at this site. In six othersthe coarctation involved the left subclavianartery but they are not included herein.

REPORT OF CASES

Case 1. (Previously reported in detatil byKirklin and associates). A child, 8 weeks old, wasbrought to the clinic because of enlargement of theheart. On physical examination there was evidenceof a greatly enlarged heart. No murmurs wereheard. Femoral pulsations could not be felt. Aroentgenogram of the thorax gave evidence thatthe lung fields were normal, and the heart wasenlarged significantly. No rib notching was seen.A retrograde aortogram demonstrated coarctationof the aorta proximal to the left subclavian arteryand stenosis of the left subclavian artery at itsorigin.

Vol. 146

ELLIS AND CLAGETT Annals of SurgeryAugust 1957

TABLE 1. Coarctation of Aorta Proximal to Left Subclavian Artery

Blood Pressure Findings When Coarctation Was Excisedin Arms Result andAge, Yr. Intercostal Associated Operative Blood PressureCase and Sex Right Left Roentgenogram Arteries Conditions Procedure in Right Arm

1 8 vk. F Markedly enlarged Normal Stenosis of L. subclavian artery 4 years laterheart; coarctation origin of L. and ligamentum chest x-raysproximal to L. subclavian arteriosum divided; neg. B.P. 98/58subclavian artery artery end-to-end anasto-in aortogram mosis of aorta

2 4, M 1 16'56 84/70 Heart enlarged; Dilated Patent ductus Ductus and L. sub- Died suddenlypulmonary vascular on right arteriosus clavian artery 1 hour aftercongestion divided; end-to- operation

end anastomosisof aorta

3 25 F 145/110 100/84 Aortic knob absenit; Dilated Superior vena L. subclavian artery Well 16 mo.notching of ribs on right cava on left; preserved; end-to- later. B.P.on right 2 aortic end anastomosis 128/100

aneurysms of aorta

4 25 F 180/98 125/95 Small aortic knob; Dilated 2 coarctations L. subclavian artery Well 4 yearsnotching of ribs on right proximal and and ligamentum later. B.P.on right distal to divided; end-to- 138/78

small L. sub- end anastomosisclavian artery of aorta

5 10° F 140,/80 90,/? Markedly enlarged Dilated Turner's L. subclavian artery Well 2 yearsheart on right syndrome; and ligamentum later; heart

superior vena divided; end-to- still enlarged.cava on left end anastomosis B.P. 130/70

of aorta6 34 NI 195/100 125/100 Prominent L. Dilated L. subclavian artery Well 1 year

ventricle and on right and ligamentum later. B.P.notching of ribs divided; end-to- 165/88on right end anastomosis

of aorta

FIG. 1 (case 1). Findings and operation. L. C.C. means left common carotid artery; L. S. A.,left subclavian artery, and Lig. A., ligamentumarteriosum in this and the following illustrations.(Modified from Kirklin, J. W., H. B. Burchell,D. G. Pugh, E. C. Burke and S. D. Mills: SurgicalTreatment of Coarctation of the Aorta in a TenWeek Old Infant: Report of a Case. Circulation,6: 411, 1952.)

Operation was performed when the child was10 weeks old. A typical coarctation of the aortawas located just proximal to the left subclavianartery, and the origin of the left subclavian arterywas narrowed (Fig. 1). The ligamentum arteriosumwas not patent and was attached to the aorta inthe region of the coarctation. The intercostal ar-

teries were not enlarged.The stenosed segment of the aorta was excised.

The ligamentum arteriosum and left subclavianartery were ligated and divided and an end-to-end

.uoarctatironFiG. 2 (case 2). Findings and operation.

anastomosis of the aorta was effected by means ofinterrupted mattress stitches of 5-0 silk.

Convalescence was uneventful. When re-ex-amined at 4 years of age, the child was asymp-tomatic. Good femoral pulses were palpable anda thoracic roentgenogram showed a normal cardiacsilhouette.

Case 2. A boy, 4 years and 9 months old, wasadmitted for treatment of a congenital heart lesion.At the age of 1 week an enlarged heart had beennoted and in the ensuing years, episodes of cyanosishad occurred. The blood pressure was 116/56 inthe right arm and 84/70 in the left. Physicalexamination revealed an overactive and enlargedheart. A loud systolic murmur was heard over thewhole precordium. In addition, a diastolic murmurwas audible in the left third intercostal space. The

146

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and thoraeie knob 1S absent.

second pulmonic sound was accentuated. Pulsationswere easily palpable in the femoral artery. Nocyanosis was noted.

A thoracic roentgenogram showed evidence ofcardiac enlargement with prominence of the pul-monary vascular shadows. There was no notchingof the ribs. Cardiac catheterization demonstratedcoarctation of the aorta proximal to a patent ductusarteriosus. While the patient was breathing air, theleft-to-right shunt through the ductus was meas-

ured as 54 per cent of systemic flow, while theright-to-left shunt was 61 per cent of pulmonaryblood flow.

The findings at operation were as follows (Fig.2): The patent ductus arteriosus entered the aortajust opposite the origin of the left subclavian ar-

tery. Coarctation of the aorta was present justproximal to the origin of the left subclavian artery.The intercostal arteries on the right were somewhatdilated.

The patent ductus arteriosus was divided andthe pulmonic end was closed with interrupted silksutures. The left subclavian artery was ligated anddivided and the area of coractation was excised.An end-to-end anastomosis of the cut ends of theaorta was effected with interrupted everting mat-tress sutures of 5-0 silk. When the procedure wascompleted, cardiac arrest occurred but the heartaction was re-established by cardiac massage andthe intracardiac administration of 2 cc. of 1 : 100,-000 epinephrine and 4 cc. of 10 per cent solutionof calcium gluconate. The child's condition re-

mained satisfactory until he suddenly died whenbeing returned to his room.

Case 3. A woman, 25 years old, was admittedwith a chief complaint of shortness of breath onexertion. She had had rheumatic fever at the ageof 12, and again at 17 years. Symptoms of dyspneahad begun during a pregnancy 4 years before ad-mission and had progressed. Physical examination

Volume 146Number 2 147

148 ELL

L 0.c.

FIG. 4 (case 3). Findings and operation

revealed a systolic murmur over the wholecordium, most marked in the aortic region.was an associated thrill. Femoral pulses wersent. The blood pressure in the right arm145/110 and in the left 100/84. A thoracic i

genogram showed absence of the aortic knolnotching of the ribs on the right (Fig. 3).

The findings at operation were as follows4): There was a left superior vena cava. Thsubelavian artery was enlarged. There weraneurysmal dilatations of the aortic arch prcto the left subelavian artery. The coractatiorjust proximal to these. No ligamentum arter:was identified, but the recurrent nerve paround the arch of the aorta. Included in tIsected portion of the aorta were the stricturthe two aneurysms. The right intercostal arwere dilated. None was sacrificed. The leftclavian artery was preserved and an end-tanastomosis of the aorta was done with contii5-0 silk sutures reinforced with a few interrmattress sutures of silk.

The postoperative course was uneventfulpatient was seen again 16 months later at N

time she was asymptomatic. The blood pressithe right arm was 128/100 and in the left 10

Case 4. A woman, 25 years old, was adnto the clinic because of high blood pressure2 years she had not been feeling well andbeen short of breath on exertion. She complalso of cold feet and mild aching in thePhysical examination revealed a blood pressu180/98 in the right arm and of 125/95 in theA systolic murmur, grade 1 (on a grading ba1 to 4), was audible over the base of the INo femoral pulses were palpable. A thoracic r

genogram indicated a heart of normal size, aaortic knob and notching of the ribs on the

At operation (Fig. 5) the coarctation seembe proximal to the left subclavian artery, vwas small. The ligamentum arteriosum entere(aorta at the site of the coarctation and anguit medially. The right intercostal arteriesdilated. The ligamentum and the left subcl

IS AND CLAGETT Annals of SurgeryAugust 1957

artery were ligated and divided, the strictured por-L.S A. tion of the aorta was excised, and an end-to-end

anastomosis was made with running sutures of 5-0silk reinforced with interrupted mattress suturesof the same silk. When examined after removal,the specimen showed not one but two strictures.The aortic lumen was completely occluded prox-imal to the left subelavian artery and partially oc-cluded distal to this vessel. A somewhat similar

ies case has been reported by Efskind and Sanderud.The postoperative course was uneventful and

the patient was dismissed on the eleventh day afterl. operation. When last heard from 4 years after op-

eration she -was well. The blood pressure in the

pre- right arm was 138/78.Case 5. A girl, 10 and one-half years old, wasThere first seen at the clinic because of webbing of there ab- neck. Physical examination showed the typical find-onwts ings of Turner's ovarian agenesis including web-

b and bing of the neck, shield-shaped chest, increasedcarrying angle of the arms and a high, arched

(Fig. palate. Blood pressure on the right was 140/80, onLe left the left 90/?. A systolic murmur could be heard

over the base of the heart. Pulsations were absentximwol from the femoral artery. Roentgenograms of then was thorax showed marked enlargement of the heartiosum but no notching of the ribs.assed At operation the coarctation of the aorta was

found proximal to the left subclavian artery (Fig.e and 6). A superior vena cava was present on the leftteries side. The aorta distal to the site of the coarctationrteresu was dilated. The ligamentum arteriosum entered.osub- the aorta in the region of the coarctation. The righto-end

intercostal arteries were enlarged; those on the lefttuous were normal in size. The ligamentum arteriosum,the left subclavian artery and three intercostal ar-

The teries, two on the left and one on the right, werewhich divided and the strictured portion of the aortaare in excised. An end-to-end anastomosis of the aorta)8/78. was done with interrupted mattress sutures of 5-0nitted silk.

.For The postoperative course was uneventful. TheI had patient was seen again 2 years later. The bloodlainedlegs.

ire ofe left.Lsis ofheart.roent-smallright.ed tovhichd thelatedwere.avian FIG. 5 (case 4). Findings and operation.

Volume 146 COARCTATIONNumber 2

LCC.~~~~~~.CCLC. L.S.A. L.C

Coarctaton- Lig._ a

Lig. a

> I-st. intercostal a.

FIG. 6 (case 5). Findings and operation.

pressure in the right arm then was 130/70. Athoracic roentgenogram showed cardiac enlarge-ment. There were, however, no symptoms referableto the heart.

Case 6. A man, 34 years old, was first seen be-cause of hypertension since youth. The physicianin his home locality had noticed a difference in theblood presure in his two arms. He was asymp-tomatic. The blood pressure in the right arm was195/100, and in the left was 125/100. A systolicmurmur, grade 1, was heard over the aortic regionand there was also a faint aortic diastolic murmur.The femoral arterial pulsations were diminished. Athoracic roentgenogram showed prominence of theleft ventricle and notching of the ribs on the right.

At the time of operation the findings were asfollows (Fig. 7): There was a coarctation of theaorta proximal to the left subclavian artery. Theligamentum arteriosum entered the aorta at thelevel of the coractation. Some poststenotic dilata-tion and angulation of the aorta were present distalto the coarctation. The intercostal vessels on theright were dilated; those on the left were normal.The ligamentum and left subclavian artery weredivided, the contracted portion of the aorta wasexcised, and an end-to-end anastomosis was ef-fected with running stitches of 5-0 silk reinforcedwith interrupted mattress sutures of the samematerial.

The postoperative course was uneventful.Hoarseness due to trauma to the recurrent nervedeveloped but cleared within 3 months. Whenthe patient was seen a year later, he was asymp-tomatic. The blood pressure in the right arm was165/58, in the left 150/90.

CLASSIFICATION

Various classifications have been pro-posed for coractation of the aorta. In theone most commonly employed coarctationis divided into two types: the so-called

0F THE AORTA 149

adult and the infantile forms. This clas-sification is not comprehensive enough toinclude all the anatomic variations thatmay be encountered clinically. Edwardssuggested a classification based on the rela-tionship of the coarctation to the ductusarteriosus which takes into considerationwhether or not the ductus is open or closed.Thus the coarctation may lie proximal ordistal to a closed ductus or proximal ordistal to an open ductus. The presence ofany anomalies of the great vessels can bereflected clinically by a difference in ar-terial blood pressure between the two arms.The various possibilities are depicted inFigure 8. The condition with which we areconcerned in this paper is depicted in Fig-ure 8a in which the coarctation lies prox-imal to the left subclavian artery. It hasbeen postulated that coarctation lying prox-imal to the left subclavian artery arises as aresult of faulty cranial migration of the leftsubclavian artery during developmentallife. It comes, therefore, to lie at a moredistal position in the aortic arch or even inthe first part of the descending aorta. A lesspronounced variant of this deformity iscoarctation at the origin of the left sub-clavian artery.A review of the operative findings and

the pathologic specimens in the six cases inthis series indicates that the coarctation hadthe same gross and histologic appearanceas coarctation appearing in the more com-

L.c.c.

FIG. 7 (case 6). Findings and operation.

ELLIS AND CLAGETT Annals of SurgeryAugust 1957

ioartatonproximal to Atretic Stenotic Stenotic Anomalousit, 5ubcl. a. It. zubcl. a. It. subcl. a. rt. zubcl. a. origin of

rt. subel. a.

FIG. 8 a, b and c. Variations of coarctation of the aorta resulting in differences in bloodpressure in the two arms. (Reprinted from Clagett, 0. T., J. W. Kirklin and J. E. Edwards:Anatomic Variations and Pathologic Changes in Coarctation of the Aorta: A Study of 124Cases. Surg., Gynec. & Obst., 98: 103, 1954. By permission of Surgery, Gynecology andObstetrics. )

mon location beyond the left subclavianartery.' The superior aspect of the aortashowed a concavity corresponding with thesite of the aortic obstruction. Histologically,this concavity conformed to the position ofan overgrowth of media which formed acurtainlike infolding into the lumen fromthe anterior, superior and posterior aspectsof the aorta. This created a narrow ec-centric lumen. In some patients there wasalso a secondary intimal fibrous reaction ofthe curtainlike infolding and the oppositewall of the aorta. This process accentuated,to some degree, the basic narrowing causedby the medial deformity. In some instancesthe fibrous overgrowth at the narrowedaortic orifice where it was closely related tothe origin of the subclavian artery causedadditionally some narrowing of the left sub-clavian artery at its origin. The ligamentumarteriosum entered the aorta either at orslightly distal to the coarctation. In the pa-tient with patent ductus arteriosus (case2), the opening straddled the site of coarcta-tion. In one patient (case 3) no ligamentumarteriosum was found. The presence ofaneurysms of the aorta in this case where aligamentum arteriosum should have beenattached suggests the possibility of trau-matic rupture of the ligamentum. Therewas, however, no history of trauma.

CLINICAL FINDINGS

A vascular malformation of this typegives rise to a rather definite clinical picturewhich has previously been emphasized. Thefinding of unusual pressures in the tw6 armswith a lower pressure in the lefarm is thefirst clue to the diagnosis. There may behypertension in the right arm, as was truein three of these cases. Roentgenograms ofthe thorax may show rib notching on theright (Fig. 3). In three of our patients,none of whom were adults, rib notchinghad not occurred.The use of angiocardiograms or retro-

grade aortograms has been proposed byMuller and Sloan as a valuable diagnosticaid in delineating the exact anatomic loca-tion of the coarcted segment in relation tothe vessels of the aortic arch. We have notemployed this method sufficiently often tomake a statement concerning its value. Itwas employed in one of these six cases andan accurate definition of the location of thecoarctation was obtained. Since location ofthe stricture in this region does not signifythat the lesion is inoperable and since in-operability for any reasor, is exceedinglyrare in patients with coarctation of the aorta,the routine use of angiocardiography oraortography does not seem justifiable to usalthough the procedure may be useful incertain particularly unusual situations. Ineach of the six cases under review a correct

150

* The help of Dr. Jesse E. Edwards of the Sec-tion of Pathologic Anatomy who reviewed the grossspecimens is greatly appreciated.

Volume 146 COARCTATION OF THE AORTA 151Number 2 5diagnosis as to the location of the coarcta-tion was made before operation.

SURGICAL MANAGEMENT

Surgical procedures for coarctation prox-imal to the left subclavian artery, althoughmore complicated than in the usual case ofcoarctation of the aorta, are certainly notcontraindicated. None of the 223 patientsoperated on for coarctation of the aorta atthe Mayo Clinic have been denied resectionand anastomosis because of this unusuallocation.No routine technic for management can

be outlined since each patient must behandled 4 ividually. The use of a curvedrig-angle clamp such as the Satinskyclamp or Glover auricular clamp is of con-siderable assistance in controlling the prox-imal end of the aorta without totally oc-cluding the left common carotid artery. Theposterior sutures of the anastomosis mayconsist of a continuous row of suturesplaced within the lumen of the vessel tofacilitate the anastomosis. This method ofsuturing has not seemed to impair the resultin any way. The anterior row may then bemade with interrupted mattress sutures inthe usual fashion.Although it was not necessary to employ

a vascular graft in any of these cases, onemay be necessary occasionally. For this rea-son it is advisable to have a homologousaortic graft available when patients of thistype undergo operation.

SUMMARY

Attention is again drawn to the clinicalsyndrome of coarctation of the aorta occur-ring at a point proximal to the origin of theleft subclavian artery. This lesion is cor-rectable by operation and six cases are re-ported to substantiate this opinion. Onepatient with associated patent ductus ar-teriosus and a predominant right-to-leftshunt succumbed shortly after operation.The remaining five survived and have re-mained well.

REFERENCES

1. Bahn, R. C., S. Sanes, B. W. Juvelier and S. T.Urban: Presubclavian Coarctation of theAorta; Report of Case with Aneurysm ofDescending Thoracic Aorta and BicuspidPulmonary Valve Complicated by Pregnancy.Am. Heart J., 47: 444, 1954.

2. Bayley, R. H. and J. E. Holoubek: Coarcta-tion of Aorta at or Above Origin of LeftSubclavian Artery. Brit. Heart J., 2: 208,1940.

3. Bing, R. J., J. C. Handelsman, J. A. Campbell,H. E. Griswold and Alfred Blalock: The Sur-gical Treatment and the Physiopathology ofCoarctation of the Aorta. Ann. Surg., 128:803, 1948.

4. Burchell, H. B., B. E. Taylor, J. R. B. Knutsonand K. G. Waldm: Coarctation of the Aortawith Hypotension in the Left Arm: Physio-logic Observations on Direct Intra-arterialPressures and Flow of Blood. S. Clin. NorthAmerica, July, 1950, p. 1177.

5. Clagett, 0. T. and R. W. Jampolis: Coarcta-tion of the Aorta: A Study of Seventy Casesin Which Surgical Exploration Was Per-formed. A. M. A. Arch. Surg., 63: 337, 1951.

6. Clagett, 0. T., J. W. Kirklin and J. E. Ed-wards: Anatomic Variations and PathologicChanges in Coarctation of the Aorta: AStudy of 124 Cases. Surg., Gynec. & Obst.,98: 103, 1954.

7. Edwards, J. E.: Pathology of Anomalies ofThoracic Aorta. Am. J. Clin. Path., 23: 1240,1953.

8. Efskind, Leif and Axel Sanderud: An UnusualCase of Coarctation of the Aorta. J. ThoracicSurg., Z9: 665, 1955.

9. Gross, R. E.: Coarctation of Aorta. Circulation,7: 757, 1953.

10. Kirklin, J. W., H. B. Burchell, D. G. Pugh,E. C. Burke and S. D. Mills: Surgical Treat-ment of Coarctation of the Aorta in a TenWeek Old Infant: Report of a Case. Circula-tion, 6: 411, 1952.

11. Muller,. W. H., Jr. and R. H. Sloan: Experi-ences with the Use of Direct Aortography inthe Diagnosis of Coarctation of the Aorta. J.Thoracic Surg., 20: 136, 1950.

12. O'Sullivan, W. D. and Frank Glenn: Contra-indications to the Surgical Therapy of Co-arctation of the Aorta. Ann. Surg., 142: 909,1955.

13. Parker, R. L. and T. J. Dry: Coarctation of theAorta at an Unusual Site, Associated with aCongenitally Bicuspid Aortic Valve: Reportof Case. Am. Heart J., 15: 739, 1938.