Atresia of the Aortic Arch13y R. CviEE PILLSBURX, M.D., RICHARD R. LOWEJR, N.D.,

\N1) NoRMAN E. S1iMWAYX, M.D.

ATRESIA of the aortic arch is arare anmaly. Evais ' reported three

cases of aortic arch interruption in 19,217 aui-topsies at the London Hospital. Roberts2 re-cenitly described three patients evaluiated atthe National Institutes of Health and 55 pre-viously reported patients. Blake 3 reported anadditionial 18 cases registered in the ArmedForces Iistittute of Patlology. In all the re-ported series,'1; aortic arch atresia wx as neverseen as an isolated cardiovascular anoomaly,was never distal to a ligamentum arteriosum,and xvas always associated with a patentduietuis arteriosuas. Only st-ven of the 75 cases

Froin the Department of Snrgery, Stanford Uni-ersity School of Miedicine, Palo Alto, Califorinia.Supported in part by Graiit 11-4658, U. S. Public

1-lealth Service.

ill Rob)erts'2 anid Blake'sl colrlilled seriCeslived loniger thani 1 year, and the oldest re-ported case was 14 years old.7,

This paper presents a 16-year-old girl vithinterruiiption of the aortic arch betxveen thein1nomin1ate anld left common carotid arteries,wx ithout associated initracardiac anomalies,and without a patent ductns arteriosu-.s. Suc-cessful suirgical restoration of the conitinuiitybetween. the ascending and descendinig aortaxxwas accomplished with the uise of a prostheticgraft.

Case ReportL. \I. (nio. 11-60-94) is a 16-vear-old white

girl, the pr-oduict of a niormiiial pregnanicy an-d de-livery. No heart ni-iurnmtitir xas heaird at birth. Thepatients iiifancv was uiiremarkable, and she grexvand (leveloped normally. At age 6 a vigorous piul-sation xas inoted in the sulprasterial notch and a

Figure 1

Chlest x-ray showinlg the normal cardiac silhotuette a(l rib niotchlinrg oil the right side.Ci;e.?laition, oJlumn XNY, November 19674

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heart murmur was heard at that time, but nodiagnosis was made. At age 14 a clinical diag-nosis of coarctation of the aorta was made.

She has always noted fatigue and shortness ofbreath with exercise that prevented her fromparticipating in strenuous physical activities. Inthe past few months she has noted increasingsor.eness in her calves and tingling in her toesafter walking more than one block or afterclimbing one flight of stairs. The patient has hadfrequent epistaxes that have been stopped eas-ily with pressure. There was no history ofparoxysmal nocturnal dyspnea, hemoptysis, se-vere headaches, diplopia, edema, syncope, cyano-sis, or palpitations.

Physical examination revealed a well-devel-oped. well-nourished girl in no discomfort. Theblood pressure in the right arm was 148/100,and no blood pressure could be obtained in theleft arm or in either leg. The heart rate was 90per minute and regular. The skin did not showcyanosis. There was a massively dilated, tortu-ous, and pulsatile vessel palpable in the supra-sternal notch and in the right side of the neck.

_,~~~ TV..i, 7j- .4IL-C - I'

A faint pulse was felt in the left common carotidartery. Faint arterial pulses were palpable in bothgroins and over the right scapular region. Noarterial pulses were palpable in the left arm orin the feet. The lungs were clear to percussionand auscultation. The heart was not enlarged.No thrills or heaves were present over the pie-cordium. There was a grade-IV/VI systolic mur-mur heard loudest over the suprasternal niotchand right side of the neck, which diminishedin intensity down the left sternal border andcould barely be heard at the apex. A systolicbruit was heard over the right scapular regionand in the right axilla. The second sound at thepulmonic area was louder than at the aortic areaand was normally split. No diastolic murmurswere heard. No bruits were heard over the abdo-men. The left hand and arm were slightly smallerthan the right. There was no cyanosis, clubbing,or edema. Neurologic examination was normal.

X-ray examination of the chest (fig. 1)showed a normal-sized heart and clear lung fields.The aortic knob could not be seen and therewas rib notching only on the right side. The

Figure 2

Electrocardiogram.Circulation, Volume XXX, November 1964

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ATRESIA OF AORTICA ARC:H

pulmiion)iarx vasculatiire wvas within) i(uno ial limllits.The electrocarc(liogrlnlli (figy. 2) wals interpretedas nirot-ial.A thoracic aoirtogramw aas (lonie in Auiguist

1963. Benografin (76 pei cent) was iiijectedt-iio-(liigli a1 catheter placedl in the riglgt felel()] s

aterxl andt11 tlireaded ilito the tho acic ai ta tothe level of the left sibLlalian artei y (fig. 3).The (lescenililig aorta was sniai] anid did notc()iiiect vitlh the aseeniniig aorta. The left snil-clavian and left comimioni carotid ta teries W crcseen to originiate fi out the descending ao ta atits pr-oximial end. A seconid inijectioni of Renio-gratfin wx its imade throuigh ia catheter- placed in theright axillary artery ani.d threaded inito the supr:a-valvilulr- aoirta (fig. 4) The aortic valve, theascendling aoita, and the coronaryx arteries ap-peared normnal. There xvas nio aortic insifficien-cy. The aseenidinig aorta xas seeni to he conitiii-otis with the innoniinate arter v, xvhich diviiledinto ainl extr-entelv large initerial ma m ar-tery, right subelavian arter y, and right common-]](]]carotid arter y. No left co()ilmmI-on carotid vesselcould be seen . A later filim (fig. 5) showxedmassive collateral circuilationi thlrouigh the neck, theright shoulder, and the right lateral thoracic ves-sels. Ni) patent ductus ariteriosus xvas present.No c(ollttei all clhannels xvere seeni ii the leftchest.

.... ..

Figure 3

Hctrogrrade ao,togriaoit slhowing, the descending" thoracieaorta (D.T.A.), and the left sohbclavian (L.S.C.), anldthe left caoamnon, carotid (L.C.C.) arteries

Figure 4

Sonaialcnlaral togao*l th1ashwing, thle aortic valves(A.V.). the internal mamnmary (I.AVt.), th7e right sihb-claviani (R.S.C.), and the right common carotid(1.C.C.) ateries.

Figure 5

Film showirg the mnassive collaterll circulation int theright lateral thioracic wall and in th2e c rericlZana.

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Figure 6Drawing of the operative findings as seen through a left lateral thoracotomy.

Left lateral thoracotomy was performed withremoval of the fourth rib on October 21, 1963(fig 6). There were no enlarged collateral vesselsin the latissimus dorsi and serratus anterior mus-cles, and the intercostal arteries were small.Dissection of the descending aorta revealed alarge left subelavian artery in the normal posi-tion and a larger vessel, the left common carotidartery, originating from the aorta just cephaladto the subelavian. None of these three vesselswas pulsatile. There was no connection betweenthe descending aorta and the pulmonary artery.Dissection at the base of the heart showed thepulmonary artery and the ascending aorta to benormal in size and position. No thrills were pal-pable at the base of the heart or over the ven-tricles. A distance of 3 cm. separated the ascend-ing aorta from the descending aorta, and nofibrous strand connected these vessels. Theascending aorta branched into three trunks: theright common carotid, the right subelavian, andthe right internal mammary arteries. The liga-mentum arteriosum connected the posteromedialaspect of the ascending aorta to the bifurcation ofthe pulmonary artery.

Continuity between the ascending and the de-scending aorta was established with a 10-mm.knitted Dacron graft. A side-biting Potts clampwas used to isolate a portion of the ascendingaorta. An end-to-side anastomosis between theascending aorta and the pre-clotted Dacrongraft was made with running 4-0 silk. A similarPotts clamp was applied across the bulbousportion of the proximal descending aorta and anend-to-side anastomosis was completed. The leftcommon carotid vessel was temporarily clampedwhile the clamps on the aorta were removed toprevent any air trapped in the graft from goingto the head.

Postoperatively there was a transient periodof hypertension with a pressure rise to 170/110that subsided in a few hours without treatment.Strong arterial pulsations were palpable in theleft radial, left common carotid, both femorals,and the posterior tibial and dorsalis pedis ves-sels as well as in the right radial and right com-mon carotid vessels. The postoperative conva-lescence was uncomplicated, and the patient wasdischarged on the tenth hospital day. On the dayof discharge the blood pressure in the left arm

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Rt. int. cora otid a,

Rt. common carotidRegression of thedorsol aorta

Innominate a.

Ligcamcntumorteriosum

Rt, subclavian a-.-

L. int. carotid a.

IL common carotid a.a Persistence of thel dorsal aJortaJ- subclovion after 7th wk.

tCephalic mirgrction|ofthe subclovian artery

/7z5 L subc\avian at thc 4fh wk.

Figure 7Schematic drawing of the possible embryology.

was 110/80 and in the right arm 120/80.The patient was seen in the surgical clinic 3

months postoperatively. The claudication andparesthesias in her legs had disappeared. Theblood pressure in the right arm was 140/90,in the left arm 120/90, and in both lower ex-tremities 130 mm. Hg systolic.

DiscussionThere is a 76-per cent mortality with in-

terruption of the aortic arch within the firstmonth after birth.2 3 This high mortality isthought to be due to associated cardiovas-cular anomalies. In Roberts'2 review of theliterature ventricular septal defects were pres-ent in 49 of 51 patients in which the in-tegrity of the ventricular septum was cited.Deformity of the aortic valve or of the sub-aortic outflow tract is seen frequently. A pa-tent foramen ovale or an atrial septal defect isnearly always present. A ductus arteriosusconnecting the pulmonary artery to the de-scending thoracic aorta has been recorded inall the previously reported cases. The largepatent ductus plus the associated intracardiaedefects lead to early congestive heart failure,pulmonary complications, and death. It hasbeen stated that complete interruption of theaortic arch occurring as an isolated anomalyis incompatible with life.2'Circulation, Volum.e XXX, November 1964

This patient's favorable clinical course canbe explained by the absence of intracardiaedefects and of a patent ductus arteriosus. Theblood flow to the descending aorta was byway of collateral vessels in the right side ofthe chest wall and through retrograde flow inthe left common carotid and left subelavianarteries. The retrograde flow of blood in theleft common carotid and left subclavian ar-teries explains the incomplete filling of thesevessels on the aortogram. Obliteration of theductus allowed the massive collateral circu-lation to develop. This collateral aided indecompressing the left side of the heart andprotected the patient against left-sided heartfailure and severe systemic hypertension. Thelack of any connection between the systemicand pulmonary circulations also preventedthe development of pulmonary hypertensionand its sequelae.The possible embryologic explanation of

this patient's defect is pictured in figure 7.Normal regression of the left-sided dorsalaorta between the left third and fourth aorticarches did not occur. This vessel persisted asthe large vessel seen connecting the upperpart of the descending thoracic aorta withthe carotid vessels and is functionally the

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left common carotid artery. There was an ab-normal regression of the left third, fourth,and sixth aortic arches, which explains theinterruption of the aortic arch between theinnominate artery and the functional leftcommon carotid artery (persistent left dorsalaorta), and the absence of the left-sidedductus arteriosus. There was a right-sidedductus arteriosus. which became obliterated.The clinical diagnosis of interruption of

the aortic arch is difficult because of thesimilarities between it and coarctation of theaorta. The associated intracardiac anomalies,usually present, tend to overshadow the in-terruption of the arch. Cardiac catheter-ization and angiograms are necessary todemonstrate the entire complex and to makethe diagnosis of arch interruption. As demon-strated by our case, the prognosis dependson the severity of the intracardiac anomaliesand the presence of a patent ductus arterio-sus rather than the arch interruption.The type of surgical correction depends on

the anatomy. An end-to-end anastomosis be-tween the ascending and the descendingaorta would be the procedure of choice;however, a prosthetic graft may be needed tobridge the interruption. Merrill8 reported asuccessful surgical correction by anastomos-ing the left subclavian artery to the descend-ing aorta in an end-to-side manner. Quie 9

reported a case in which the left commoncarotid artery was sutured to the descendingaorta, but the patient died 2 days later. RuizVillalobos 7 reported the successful correctionin a 14-year-old patient by using a Teflongraft between the left subelavian artery andthe descending aorta. In Roberts'2 case aTeflon graft was placed between the ascend-ing and the descending aorta, but the patientdied postoperatively of pulmonary complica-tions. Blake3 used a Dacron graft to bridgethe interruption between the ascending and

descending aorta. In all of these patients thepatent ductus arteriosus was divided and su-tured.

SummaryA case report of a 16-year-old white girl is

presented with the cardiovascular anomalyconsisting of interruption of the aortic archbetween the innominate artery and the leftcommon carotid artery and distal to a rightligamentum arteriosum. This case is uniquebecause the interruption of the aortic archwas not associated with intracardiac anohmalies,a patent ductus arteriosus was not present,and the patient had minimal symptoms. This isthe sixth reported attempt at surgical correc-tion of interruption of the aortic arch andrepresents the fourth successful one.

References1. EVANS, W.: Congenital stenosis (coarctation),

atresia and interruption of the aortic arch.Quart. J. Med. 26: 1, 1933.

2. ROBERTS, W. C., MORROW, A. G., AND BRAUN-WALD, E.: Complete interruption of the aor-tic arch. Circulation 26: 39, 1962.

3. BLAKE, H. A., MANION, W. C., AND SPENCER, F.C.: Atresia or absence of the aortic isthmus.J. Thoracic Surg. 43: 607, 1962.

4. KLEINERMAN, J., YANG, W., HACKEL, D. B.,AND KAUFMAN, N.: Absence of the transverseaortic arch. Arch. Path. 65: 490, 1958.

5. MEHRIZI, A., AND MoRRIsH, H. F.: Interrup-tion of the aortic arch. Bull. Johns HopkinsHosp. 111: 127, 1962.

6. NADAS, A. S.: Pediatric Cardiology. Philadel-phia, W. B. Saunders Company, 1963.

7. Ruiz VILLALOBOS, M. C. R., DE BALDERRAMA,D. P., LOPEZ Y LOPEZ, J., AND CASTELLANOS,M.: Complete interruption of the aorta. Am. J.Cardiol. 8: 664, 1961.

8. MERRILL, D. L., WEBSTER, C. A., AND SAMSON,P. C.: Congenital absence of the aortic isth-mus. J. Thoracic Surg. 33: 311, 1957.

9. QUIE, P. G., NovIcK, R., ADAMS, P., JR., ANDER-SON, R. C., AND VARCO, R. L.: Congenitalinterruption of the aortic arch. J. Pediat. 54:87, 1959.

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R. CREE PILLSBURY, RICHARD R. LOWER and NORMAN E. SHUMWAYAtresia of the Aortic Arch

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1964 American Heart Association, Inc. All rights reserved.

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