coarctation of the aorta infancy -...

Coarctation of the Aorta in Infancy

By SACHCHIDA N. SINHA, M.D., MERL L. KARDATZKE, B.A., ROGER B. COLE, M.D.,ALEXANDER J. MUSTER, M.D., HANS U. WESSEL, M.D., AND MILTON H. PAUL, M.D.

SUMMARYMaterial from 78 infants with coarctation and congestive heart failure during the first

6 months of life was- reviewed. Three fourths of these infants had combined aorticlesions, that is, coarctation, a narrow constrictive zone of stenosis, and tubular hypoplasiaof the transverse arch, a proximal, long, uniformly narrow segment.

It was the purpose of this study (1) to distinguish coarctation of the aorta from tubularhypoplasia by using pathologic and angiographic measurements, (2) to assess the rela-tionship of these two entities and additional shunting cardiac defects with the occurrenceof congestive heart failure in infancy, and (3) to evaluate the optimal therapeutic ap-proach for symptomatic infants with coarctation.

It was found that (1) coarctation of the aorta as an isolated anomaly is a relativelyuncommon cause of congestive heart failure in infancy. In contrast, coarctationassociated with tubular hypoplasia of the transverse arch or with additional cardiacdefects is frequently associated with severe congestive heart failure. It was found also(2) that surgical management is far more successful than medical management alonein symptomatic infants with coarctation of the aorta and associated significant cardiacdefects.

Additional Indexing Words:Congenital heart disease Tubular hypoplasiaCardiac surgery

COARCTATION of the aorta with conges-tive heart failure in early infancy repre-

sents a complex clinical, pathologic, physiolog-ic, and therapeutic spectrum. This report isconcerned with the interplay of the anatomyof the coarctation, the presence of associatedshunting lesions, and the response of thepulmonary vascular bed in a group of 78infants with cardiorespiratory symptoms re-quiring hospitalization before 6 months ofage.

Coarctation of the aorta was first describedby Morgagnil in 1760 as a zone of constriction

From The Children's Memorial Hospital (Willis J.Potts Children's Heart Center) and The Departmentof Pediatrics, Northwestern University MedicalSchool, Chicago, Illinois.Work was supported in part by The Helen Fay

Hunter Pediatric Cardiology Fund and Grant T12-HE-05770 from the National Institutes of Health.

Address for reprints: Milton H. Paul, M.D., TheChildren's Memorial Hospital, Chicago, Illinois 60614.

Circulation, Volume XL, September 1969

Congestive heart failure

in the aorta. In 1903, Bonnet2 classifiedcoarctation of the aorta into two types:"adult" coarctation, a sharply localized zone ofconstricf on) and "infantile" coarctation, a longuniformly narrow segment. Subsequent au-thors have attempted to reclassify this mal-formation on the basis of combined anatomicand physiologic factors. These classifications,however, encompass both localized constric-tion and diffuse hypoplasia under the defini-tion of coarctation of the aorta and lackof precise morphologic description.Edwards and associates3 advanced a more

exact anatomic description of coarctation ofthe aorta, defining it as a sharply localizedzone of stenosis characteristically found at thejunction of the transverse aortic arch and thedescending aorta. This localized stenosis isproduced by a curtain-like infolding of theaortic media into the lumen of the aorta. Along uniformly narrow segment, the infantilecoarctation of Bonnet, was described by

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Edwards and associates4 as tubular hyloplasia.It is characterized by a noimal aortic mediaand is distinguished from coarctation as aseparate pathologic entity. This pathologicdistinction allows for a more precise evalua-tion of the relative roles of coarctation of theaorta and tubular hypoplasia as causes ofcongestive heart failure in early infancy.We have reviewed material from 78 infants

with coarctation of the aorta with particularreference to the aortic arch morphology asdescribed by Edwards and co-workers.3 In allinfants congestive heart failure developedbefore 6 months of age. It was the purpose ofthis study: (1) to delineate coarctation of theaorta from tubular hypoplasia by usingpathologic and angiocardiographic measure-

Figure 1

Autopsy specimen from an infant with coarctation ofthe aorta. Note the combination of aortic pathology;a difusely hypoplastic segment involving the entirelength of the transverse aortic arch plus a narrowconstrictive diaphragm at the entry of the closingductus arteriosus. AA = ascending aorta; TA = trans-verse aortic arch; PDA = patent ductus arteriosus;DA descending aorta; PA = main pulmonary artery;CO - coarctation of the aorta.

ments; (2) to assess the relationship of thesetwo entities and additional shunting cardiacdefects to the occurrence of congestive heartfailure in infancy; and (3) to evaluate theoptimal therapeutic approach for symptomaticinfants with coarctation.

Quantitative Classification of Aortic ArchPathology (Autopsy and Angiographic

Criteria )

Criteria for tubular hypoplasia of thetransverse aortic arch were developed fromautopsy measurements of the frontal diameterof the transverse arch (TA), the ascendingaorta (AA), and the descending aorta (DA)in 26 normal infants and 37 infants withcoarctation. Measurements were made in themiddle of the ascending aorta, the middle ofthe transverse aortic arch, and in the descend-ing aorta 5 cm distal to the isthmic region inorder to avoid the region of post-stenoticdilatation in coarctation (figs. 1 and 2). It waspossible to compensate for age and otherdevelopmental variables by using the ratio of

Figure 2

Quantitative classification of aortic arch pathology.Angiocardiogram in left anterior oblique view. AA.=ascending aorta; TA = transverse arch; DA = de-scending aorta.


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OF AORTA IN INFANCY

* COARCTATION, ANGIOCARDIOGRAM

A COARCTATION, AUTOPSY

O NORMAL, AUTOPSY

00 0

0

a

0

ANO

AA o 00 0o0

A A 0U

An , 0A a AAa*~A A AU TA/AA + 0. 37 TA/DA ' 0.82A~ ~~ I

A A A_ Li

aA U

A A

I I I 1 I

.30 40 .50 .60

AA

.70 .80 .90 1.00 1.10 1.20

TA/DA

0

0

0

0O 0

O 0

0

Figure 3Quantitative classification of aortic arch pathology: The ratio of the diameter of the transverseaortic arch (TA) to that of the descending aorta (DA) on the abscissa is plotted against theratio of the diameter of the transverse aortic arch to that of the ascending aorta (AA) on theordinate. The oblique line separates the normal from hypoplastic transverse aortic arch. Thisseparation was derived by discriminant analysis of these ratio measurements. The criteria fornormal transverse aortic arch are given on the illustration.

the transverse arch diameter to that of theascending aorta and the ratio of the transverseaortic arch diameter to that of the descendingaorta. Use of the diameter ratios has the furtheradvantage of permitting recovery of directlycomparable ratio measurements from angio-cardiograms. Such in vivo measurements were

obtained on angiocardiograms from 31 infantswith coarctation of the aorta.

Reliable separation of a normal from an

abnormal aortic arch required the use of thesemultiple ratio measurements. Linear discrimi-nant analysis provided the following criteriafor a normal population (fig. 3): TA/AA +0.37 TA/DA' 0.82.By use of this discriminant function, three

types of transverse aortic arches were definedCirculation, Volume XL, September 1969

in our material: type I, normal transverseaortic arch; type II, moderate tubular hypo-plasia of the transverse aortic arch; and typeIII, severe tubular hypoplasia of the arch

(fig. 4).The anatomic malformations in our material

on 78 infants with coarctation of the aorta andcongestive heart failure in the first 6 months oflife were reviewed. In 26 infants who are

alive, the diagnosis was made by cardiaccatheterization and angiography. In the re-

maining 52 patients, the diagnosis was basedon postmortem examination. Of 78 infantswhose condition was originally diagnosed as

coarctation of the aorta, 71 had a segmentalconstrictive zone of stenosis, that is, coarcta-tion as defined by Edwards and co-workers.3

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DISCRIMINANT FUNCTION

Figure 4Quantitative classification of aortic arch pathology: Histograms showing frequency distributionbased on discriminant analysis of the ratio data from measurements of the transverse aorticarch, ascending aorta, and descending aorta. Data from autopsied infants with no aorticpathologic changes are plotted on the right (normal-autopsy); data from autopsied coarctationmaterial, on the upper left (coarctation-autopsy); and data from angiographic coarctation mate-rial, on the lower left (coarctation-angio). Three types of transverse aortic arch pathology aredefined using these measurements: type I, normal transverse aortic arch; type II, moderatelyhypoplastic transverse aortic arch; type 111, severely hypoplastic transverse aortic arch.

The other seven infants had only tubularhypoplasia of the transverse aortic arch andno coarctation of the aorta.

In the group of 71 infants with coarctationof the aorta, 16 had a normal (type I)transverse aortic arch, 44 had moderatetubular hypoplasia of the arch (type II), and11 had severe hypoplasia of the transverseaortic arch (type III). Thus, in our coarcta-tion population, 55 of 71 infants with coarcta-tion of the aorta had combined aortic lesions,that is, a narrow constrictive zone of stenosis,coarctation, and a proximal, long, uniformlynarrow segment, tubular hypoplasia of thetransverse aortic arch (fig. 1).

Of the seven infants who had only tubularhypoplasia of the transverse aortic arch, thetransverse aortic arch was moderately hypo-plastic (type II) in three and severelyhypoplastic (type III) in four.

Associated Cardiac Defects

The cardiac malformations associated withcoarctation of the aorta and their relationshipto the anatomic types of transverse aortic archin 71 infants are listed in table 1. Thecoarctation in all of these infants was locatedeither at or just proximal to the entry of theductus arteriosus.


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COARCTATION OF AORTA IN INFANCY

Table 1

Associated Cardiac Lesions in 71 Infants with Coarctation of the Aorta and with CongestiveHeart Failure in the First Six Months of Life

Aortic arch

Type II, Type III,Type I, moderately severelynormal hypoplastic hypoplastic

Infants (total) 16 44 11

Coarctation only 5 11 0Coarctation plus:

Patent ductus arteriosus 7 29 10Probe patent 6 21 8Widely patent 1 8 2

Ventricular septal defect 9 20 9Atrial septal defect 2 2 2Aortic stenosis 2 1 2Mitral stenosis 1 1 0Transposition of the great arteries 0 4 3Total anomalous pulmonary venous

return 0 1 0

In the type I transverse aortic arch group,

five infants had coarctation of the aortawithout other cardiac lesions. Seven infantshad a patent ductus arteriosus (PDA);however, in only one patient was the ductuswidely patent and hemodynamically signifi-cant. Ventricular septal defect (VSD) was

found in nine infants. Atrial septal defect(ASD) was found in two, aortic stenosis intwo, and mitral stenosis in one.

In the type II transverse aortic arch group,11 had coarctation of the aorta only. Theductus arteriosus was patent in 29; in 21 it wasprobe patent, and in eight it was widelypatent. A VSD was found in 20 patients. Fourinfants had transposition of the great arteriesand one had total anomalous pulmonaryvenous return. ASD was noted in two, aorticstenosis in one, and mitral stenosis in one.

In the infants with type III transverse aorticarch, coarctation of the aorta was alwaysassociated with additional single or multiplecardiac malformations. The ductus arteriosuswas patent in 10, although it was widelypatent in only two. VSD was found in ninepatients, transposition of the great arteries inthree, ASD in two, and aortic stenosis intwo.The different cardiac malformations present

in the seven infants who had tubular hypo-Circulation, Volume XL, September 1969

plasia of the transverse aortic arch withoutcoarctation of the aorta are listed in table 2. Inall these infants the diagnosis was verified atpostmortem examination. The ductus arterio-sus was patent in all, but widely patent in onlyone. VSD was found in two infants andtransposition of the great arteries in three,with a single ventricle in one.

Infants with particularly complex cardiacmalformations were excluded from furtherclinical analysis because of the serious inde-pendent prognostic significance of these mal-formations. Of the 11 infants with complex

Table 2

Associated Cardiac Lesions in Seven Infants withIsolated Tubular Hypoplasia

Aortic arch

Type II, Type III,moderately severelyhypoplastic hypoplastic

Infants (total) 3 4Patent ductus arteriosus 3 4Probe patent 3 3Widely patent 0 1

Ventricular septal defect 0 2Atrial septal defect 1 0Aortic stenosis 1 0Transposition of the great

arteries 1 2Single ventricle 0 1

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Table 3

Diagnosis and Aortic Arch Anatomy of 60 Infants with Coarctation of the Aorta

Associated cardiac lesion

Coarctation of the aorta onlyCoarctation of the aorta with single cardiac lesion

Patent ductus arteriosusVentricular septal defectOther intracardiac lesion

Coarctation of the aorta, patent ductus arteriosus,and associated intracardiac lesions

Table 4

Electrocardiographic Data on 60 Infants with Coarctation of the Aorta

Aortic arch anatomyand type of lesions

AnatomyType IType IIType III

Totalnumber

:15378

Axis

RAD NAD LAD

10295

250

200

XRAD

133

Ventricular pattern

RVH CVH LVH

10235

1102

330

Coarctation onlyAssociated cardiac lesions

SingleMultiple

15 13 2 0 0 12 1 1 1

20 14 2 1 3 12 5 3 025 17 3 1 4 14 7 2 2

Abbreviations: RAD = right axis deviation (QRS frontal axis between +90° and + 1800); NAD = normal axisdeviation (0° to +900); LAD = left axis deviation (0° to -900); XRAD = extreme right axis deviation (+180° to-900); RVH = right ventricular hypertrophy; CVH = combined ventricular hypertrophy; LVH = left ventricularhypertrophy; WNL = within normal limits.

cardiac malformations, 10 had transposition of Of the four infants with isolated tubularthe great arteries, and one had total anoma- hypoplasia, two had transverse aortic archeslous pulmonary venous return. Three addi- of type II and two had type III arches. In twotional infants were excluded because of of these a PDA was the only associatedincomplete clinical data. cardiac lesion. A third infant had PDA, ASD,

Further analysis of the electrocardiograms, and aortic stenosis, and the fourth infant hadphysiologic data, and the medical and surgical VSD and PDA.clinical course was directed to the remaining64 infants: 60 with coarctation of the aorta Electrocardiogramsand four with isolated tubular hypoplasia. The Electrocardiograms of 60 infants wereinfants with coarctation of the aorta are available for review. The most frequentgrouped in table 3 according to the transverse findings were right axis deviation (BAD) ofarch anatomy (type I, normal; type II, the frontal QRS vector in 44 infants (73%) andmoderate hypoplasia; type III, severe hypo- right ventricular hypertrophy (RVH) in 38plasia). Each transverse arch group is further (63%). RAD and RVH were present in all 11subdivided as having isolated coarctation, infants seen before 3 months of age withcoarctation with an additional extra or intra- isolated coarctation and a normal or moder-cardiac lesion, or coarctation with multiple ately hypoplastic transverse arch. No consistentother cardiac lesions. electrocardiographic pattem was noted in


Type I

5

5131

6

Aortic arch

Type II

10

12714

15

Type III

0

1001

6

WNL

111

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Table 5

Electrocardiographic Data on 26 Surviving Infants with Coarctation of the Aorta

Axis Ventricular pattern

RAD NAD XRAD IND RVH CVH LVH WNL

Initial 21 4 1 0 15 7 4 0Follow-up* 9 15 1 1 8 9 6 3

For abbreviations see table 4.* Follow-up period: mean, 35 mo; range 2 to 76 mo.

relation to the transverse arch anatomy or thevarious associated cardiac malformations (ta-ble 4).Only two infants showed left ventricular

hypertrophy (LVH) patterns before 3 monthsof age. One of these (L.P.) had VSD, PDA,and, surprisingly, marked pulmonary hyper-tension. The other (R.R.) had mitral insuffi-ciency in addition to coarctation of the aorta.

Four infants more than 3 months of ageshowed an LVH pattern at the initialexamination. One of these had isolatedcoarctation. One patient had an additionalaortic stenosis, the third patient had mitralinsufficiency, and the fourth infant had VSDand PDA.

Electrocardiograms were available in threeinfants with isolated tubular hypoplasia. Right

1 2 3 AVR AVL AVF V4R Vl V2 V4 V6 V7

A1/STD2

Figure 5

Electrocardiograms of three infants with isolated tubular hypoplasia and no coarctation of theaorta.


S . T .

35 DAY S

0 . E .I DAY

0. D .50 DAY S

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axis deviation was noted in all: two hadcombined ventricular hypertrophy, and onehad RVH (fig. 5).

Analysis of serial electrocardiograms in 26surviving infants (table 5) showed significantchanges with increasing age. In infants withisolated coarctation the frontal QRS axistended to return toward normal from rightaxis deviation. The RVH pattern in theprecordial leads was slowly replaced byincomplete right bundle-branch block and theleft ventricular potentials became more prom-inent in eight of 11 infants. However, in threeinfants with isolated coarctation, right ven-tricular dominance has persisted even after 3years of age. Infants with associated cardiaclesions did not show any characteristic evolv-ing diagnostic pattern in the electrocardio-gram with progression in age.

Cardiac CatheterizationRight and left heart catheterization and

cineangiocardiograms were performed on 44infants with physiologic data from 31 infantsbeing considered satisfactory for analysis.Pulmonary hypertension (pulmonary arterypeak systolic pressure greater than 50 mmHg) was present in all seven infants withseverely hypoplastic transverse arch, in 20 of27 (74%) with moderately hypoplastic arch,and in five of the 10 infants (50%) whosetransverse aortic arch was normal. All infantswith a shunting lesion had pulmonary hyper-tension, but only one third of those (four of12) with isolated coarctation of the aorta hadabnormally elevated right heart pressure.Three of these four infants with isolatedcoarctation and pulmonary hypertension hada moderately hypoplastic transverse arch(type II) and one had a transverse arch ofnormal caliber (type I). The latter infant(B.C.) had a pulmonary artery pressure of67/35 mm Hg and a very high (estimated)pulmonary vascular resistance of 17 units at 4weeks of age. Catheterization studies in thisinfant 1 year later revealed normal pulmonaryartery pressure and pulmonarv vascular resis-tance.

Six infants with coarctation of the aorta and

PDA were catheterized. Only left-to-rightshunting through the ductus arteriosus wasnoted by oxygen saturation data oY cineangio-cardiogram. Fifteen infants with PDA andVSD were catheterized. Definite evidence of abidirectional shunt across the ductus arteriosuseither by cineangiography or oxygen satura-tion data was noted in one case with type Itransverse aortic arch, in one with type II, andin two with type III aortic arch. A smallbidirectional shunt across the ductus arteriosusmay have been present in three others withcoarctation of the aorta, PDA, VSD, and typeIII transverse aortic arch, but the physiologicstudies were considered inadequate for proof.Two infants with isolated tubular hypo-

plasia and no segmental coarctation werecatheterized. One had moderate hypoplasiaand the other, severe hypoplasia of thetransverse aortic arch. In both cases theductus arteriosus could not be visualized ortraversed at the time of catheterization, butthe ductus was narrowly patent at autopsy.One of these infants had a VSD, and theother, aortic stenosis and a small ASD.Pulmonary hypertension and high estimatedpulmonary vascular resistance were noted inboth.The diagnosis and survival of the 44 infants

with physiologic studies are related to theestimated pulmonary vascular resistance infigure 6. Higher pulmonary vascular resistancewas noted in infants with coarctation of the

F- -

AORTIC ARCH CO. AORTA(01

I

RM L

MODERATELY 11AHYPOPLASTIC

SEVERELYIIIHYPOPLASTIC

ITOTAL PERCENT>5

A o o o

25%

CO- AORTA WITH CO. AORTA AITHSINGLE LESION IMULTIPLE LESIONS

(5) {M)

o0I

A o Ea InA, o

-T 1PERCENT,

PVR>I 51

40 96

nmmocl 15rA A e

0 0 AOB06 A A60%

60% 65% 52 %

DEAD ALIVE PERCENT SIJRVIV-L0 D PVR> 33%

ZA A PVR><5 36S

0 C_) PVR< 5 76%__j

Figure 6Estimated pulmonary vascular resistance in units re-lated to (1) type of coarctation (isolated or associatedlesions), (2) type of aortic arch pathology (normal orhypoplastic), and (3) survival.

Circulation, Volume XL, SePtember 1969

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............ ....

80O 5 0 *^9---+-CUMULATIVE PERCENT MORTALITY

_ - CUMULATIVE ENTRIES TOOC. / 0AJ 40 --- CUMULATIVE DEAD BY ARE OF ENTRY

40 .......... 60 C

P 30 0

B ~~~~~~~~~~~~~~~40

Z20 __ 0

I0

0 5 10 20 50 100 300

AGE OF FIRST CMH CONTACT. DAYS

Figure 7Overall mortality of infants with coarctation of theaorta and congestive heart failure as related to the ageof admission to The Children's Memorial Hospital(CMH), Chicago. No survival was noted among in-fants with congestive failure admitted to the hospitalat or before 5 days of life. Total mortality in thisgroup of 64 infants was 60%.

aorta and additional cardiac lesions, single ormultiple. Overall survival was significantlylower in the group with elevated pulmonaryvascular resistance.

Clinical Course and ManagementClinical data were available in 64 patients,

60 with coarctation of the aorta and four withisolated tubular hypoplasia. All but three ofthese infants were seen in congestive heartfailure between the age of 1 day and 180 daysof life (fig. 7). The three infants, first seenafter 180 days of life, were included becauseof a definite history of onset of symptomsduring the first 6 months of life. Of the 64patients, 16 were hospitalized by the seventhday of life, and 32 by the twentieth day of life.Mortality was high among symptomatic in-fants needing early hospitalization, and therewas no survival of infants admitted beforethey were 5 days old. The overall mortality(surgical and medical) in the entire groupwas 60% (38 of the 64).

Infants with coarctation of the aorta and anadditional single cardiac defect had a mortal-ity of 60% (11 of 18 patients). The mortalitywas 76% (21 of 27) in those with associatedmultiple cardiac lesions. Infants with isolatedcoarctation had a mortality of 27% (four of

15). Infants with coarctation and additionalcardiac lesions were hospitalized relativelyearlier in life, and no survival was notedamong those admitted before 5 days of age(fig. 8).Nonoperative management was employed

with vigorous anticongestive measures in 26 ofthe 64 infants whose clinical data wereanalyzed. Survival was 31% (eight of 26) (fig.9). Ten infants, including one with isolatedtubular hypoplasia, died following early surgi-cal intervention for noncardiac abnormalities(surgery type H, fig. 9).Operative management of the cardiac prob-

lem was elected in the remaining 28 infants.The survival in this group was 64% (18 of 28).Eight infants with isolated coarctation under-went resection of the coarctation with sixsurviving. Nine infants had resection of thecoarctation with division and closure of thePDA. Eight of these survived. Four of sixinfants with coarctation, VSD, and PDAsurvived surgical resection of the coarctation,ductus division, and pulmonary artery band-ing.

20CO AORTA ONLY

10;

0 L 77--7_ -T-

20

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O0

Uo

zLLI

LL.

m

z

CO AORTA WITH SINGLE CARDIAC LESION

, I

30 CO. AORTA WITH MULTIPLE CARDIAC LESIONS

20 - CUMULATIVE ENTRIES TO CMH 75 SNMORTALTY

---CUMULATIVE DEAD -

10

O ..,1 ,I0 5 10 20 50 100 300

AGE OF FIRST CMH CONTACT, DAYS

Figure 8

Mortality among infants with coarctation of the aortaand congestive heart failure related to the age of ad-mission to hospital (CMH) and to different diagnosticgroups. (Upper graph) Data on infants with isolatedcoarctation of the aorta; (middle graph) on infantswith coarctation associated with single cardiac lesion;(lower graph) on infants with coarctation of the aortaassociated with multiple cardiac lesions.


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zLUJ

L.L0~

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5

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TYPES OF SURGERY PERFORMED

A.

a

C.

D.

E.

F

G

H.

RESECTION OF CO, AORTA

CO. RESECTION + P. D. A

CO. RESECTION+ P D. A.

+PULMONARY ARTERY BANDING

CO. RESECTION-+ BANDING

PULMONARY ARTERY BANDING

P. 0. A.

P A. B + P D. A

OTHERS

89%75% m

m __ 67%

6 8 4

0%

0%

096 0%

m

31%

_K

A B C D E F G H NON-S.

CLASS OF SURGERY

Figure 9

Results of medical and surgical management of 64infants with coarctation of the aorta. Percentages ofsurvival are given according to mode of management.Various types of surgical approach indicated on theabscissa (A to H). Non-S = medical management;Co. aorta=coarctation of the aorta; PDA = divisionof patent ductus arteriosus; PAB = pulmonary arterybanding; Others = surgery for non-cardiac abnormali-ties.

The outcome of surgical and nonsurgicalmanagement was further analyzed (fig. 10) inrelation to the type of transverse archpathology and other additional cardiac de-fects. The infants are grouped according tothe transverse arch pathology. Each group isfurther subdivided as having isolated coarcta-tion (0), coarctation with a single cardiaclesion (S) and coarctation with multiplecardiac lesions (M).The five infants with isolated coarctation

and a normal transverse aortic arch allsurvived with either surgical or medicalmanagement. Among the 10 patients withisolated coarctation and hypoplastic aorticarch the overall survival was less (60%). Noinfant with coarctation and associated singleor multiple cardiac malformations survivednonsurgical management regardless of thetransverse arch pathology. With surgicaltreatment, however, the survival in this group

was encouraging since all infants with normaltransverse aortic arches survived and 11 of the14 infants (79%) with moderately hypoplasticarch survived.

Discussion

Our clinical material indicates that coarcta-tion of the aorta presenting with congestiveheart failure in early infancy is characterizedby a wide spectrum of anatomic and physi-ologic features. Anatomically, coarctation as

defined by Edwards and associates3 4 is a

localized, narrow, constrictive lesion whichpresents histologically as an infolding of theaortic media into the lumen. In our series, 71of 78 infants with an initial clinical diagnosisof coarctation of the aorta had this localizedconstriction. By contrast, tubular hypoplasia is

0oo

> 80

60D

*402 0

0-J

> 20

a: 4 0

z 600

80

100DIAGNOSIS

TYPE

Figure 10

Survival following medical management or surgicalmanagement as related to the diagnostic subgroupsand the transverse aortic arch pathology in infantswith coarctation of the aorta. Surgery: A=resectionof coarctation; B = resection of coarctation and divi-sion and suture of patent ductus arteriosus; C = resec-

tion of coarctation, ductus arteriosus division andpulmonary artery banding. Diagnosis: 0 = isolated co-

arctation of the aorta; S = coarctation with single car-

diac lesions; M = coarctation of the aorta with multiplecardiac lesions. (See text for comments and types ofaortic arch pathology.)


394

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fl SURGERY A, B, C

0S M 0 S M

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a diffuse narrowing involving a long segmentof the transverse aortic arch which does notinvolve the aortic media. Tubular hypoplasiaas an isolated lesion was found only in seven

infants. It is particularly significant to note,however, that 55 of the 78 infants had a

combination of both of these elements ofaortic pathology, that is, proximal tubularhypoplasia of the transverse arch ending witha localized constrictive coarctation.In an extensive review of pathologic and

surgical material,5' 6 Lev has used the term"converted adult coarctation" to describe thiscombination of lesions. The concept of con-

version implies that tubular hypoplasia, withchanging physiologic factors in some infants,may convert through a transitional stage into a

localized narrow zone of stenosis (coarcta-tion). Adequate clinical evidence is notavailable to document this longitudinal evolu-tion. Accordingly, we prefer to describe thesetwo types of aortic pathology as separateentities, occurring either alone or together as

noted in our pathologic and angiocardiograph-ic material.

Infants with coarctation of the aorta andcongestive heart failure in early infancypresent a difficult clinical problem and have a

high mortality as noted by several authors7-13(table 6). Our clinical material consisted ofinfants with coarctation of the aorta, con-

firmed by autopsy or angiography, who were

hospitalized because of congestive heart fail-ure during the first 6 months of life. Theseinfants form a heterogeneous group manageddifferently and by different surgical and

medical personnel over a considerable period.Analysis of the clinical course indicated thatinfants with coarctation of the aorta andadditional cardiac lesions became symptomat-ic earlier and had a considerably highermortality than infants with isolated coarcta-tion.

It is pertinent that 55 of the 71 infants(78%) with coarctation and early symptomsand signs of congestive heart failure showedmoderate to severe tubular hypoplasia inaddition to segmental coarctation. By contrast,only five infants of the entire group hadisolated coarctation of the aorta, a normaltransverse aortic arch, and no other additionallesion (table 1). Our material clearly demon-strates that the vast majority of infants withcoarctation of the aorta who develop conges-

tive heart failure have an additional cardiacdefect, usually a significant shunting lesion,and associated tubular hypoplasia of thetransverse aortic arch.

Coarctation of the aorta in early infancy can

be associated with varied physiologic re-

sponses. Pulmonary hypertension and in-creased pulmonary vascular resistance were

noted invariably in those infants who hadcoarctation of the aorta associated withsignificant shunting lesions. However, fourinfants with isolated coarctation of the aortaalso had elevated pulmonary artery pressures,

and in three of these the estimated pulmonaryvascular resistance was high. Such pulmonaryhypertensive responses in infants with isolatedcoarctation have also been documented byothers.14

Table 6

Mortality Among Patients with Coarctation of the Aorta in the First Year of Life as Reportedin Literature

Surgical mortality Nonsurgical mortality

No. % No. %

Glass et al.7 1960 14/34 41 37/74 50Mortensen et al.8 1959 0/8 0 4/8 50Freundlich et al.9 1961 3/3 100 12/27 44Malm et al. 10 1963 6/12 50Adam et al."1 1965 2/20 10Lindesmith et al.'2 1966 3/5 60Hartman et al.13 1967 5/15 33

Ci&culation, Volumne XL, September 1969

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The electrocardiographic findings in in- additional cardiac defects, irrespective of thefants less than 3 months of age did not transverse arch anatomy. In contrast. withcorrelate well with the different anatomic and surgical intervention, survival was 78% (18 ofphysiologic features of coarctation or the 23) in this infant group with additionalassociated cardiac lesions. Most infants less cardiac defects. The only infant with athan 3 months of age showed right ventricular severely hypoplastic arch who survived waspreponderance irrespective of the associated surgically treated. Although this study hasintracardiac lesions and the transverse arch been retrospective in nature, the strikinganatomy. At times the electrocardiogram difference between the results of medical andpresented a misleading reflection of the surgical management appears conclusive. Inphysiologic status. Thus, the left ventricular agreement with others,19-21 we would recom-preponderance in an infant with coarctation, mend that early surgical intervention isVSD, and PDA did not reflect the systemic indicated in the presence of significant asso-pressure in the pulmonary artery and the high ciated shunting cardiac lesions.estimated pulmonary vascular resistance (fig. There is a further controversy concerning11). The right ventricular hypertrophy evi- the mode of management of infants withdent in the electrocardiogram of an infant isolated coaretation of the aorta. In this groupwith isolated coarctation of the aorta was initial medical management alone is frequent-difficult to reconcile with the relatively low ly successful. If early surgery is elected, thepulmonary artery pressure and the severe possibility of residual or recurrent segmentalsystemic hypertension (fig. 12). obstruction requiring subsequent additionalManagement of the infant with coarctation surgery must be considered. Additional long-

of the aorta and congestive heart failure has term follow-up studies are needed to indicatebeen a matter of controversy.7 18 In our series, the cause and frequency of this problem. Insurvival with anticongestive measures alone our material, seven of the 18 surviving infantswas 31% (8 of 26 patients). In contrast, our treated surgically have evidence of recurrentoperative management of the cardiac problem aortic obstruction. If infants with isolatedresulted in 64% (18 of 28) survival. coarctation can be managed successfully byThere was no survival with medical man- anticongestive measures, it would seem advis-

agement alone in infants with coarctation and able to postpone surgery until a more

I I I I I I AVR AVL AVF V4R VI V2 V5 V6 V7

. ; - l .. ~~~~~t At.zt l..tT1..+..t;*7-:r:.'s

LV mm Hg

13 11l 2

MPA

130/60

PVR units

15Figure 11

Electrocardiogram and pertinent physiologic data of an infant (L.P., 30 days old) with coarcta-tion of the aorta, ventricular septal defect, and, patent ductus arteriosus. LV,= left ventricle;MPA = main pulmonary artery; PVR = estimated pulmonary vascular resistance. The electro-cardiogram does not reflect the severe pulmonary hypertension.


396 SINHA ET AL.


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I I I I I I AVR AVL AVF V4R V 1

AO mmHg

195/80

MPA

85/40

V2 V5 V6 V7

PVR units

7

Figure 12

Electrocardiogram and pertinent physiologic data of an infant (W.W., 16 days old) with coarc-

tation of the aorta. AO = ascending aorta; MPA = main pulmonary artery; PVR = estimatedpulmonary vascular resistance. The electrocardiogram does not reflect the severe systemichypertension.

satisfactory age is reached, 3 to 8 years, whenthe reconstructed lumen is more likely toprove adequate for adult life.22

AcknowledgmentWe gratefully acknowledge the surgical contribu-

tions to the care of these infants by Drs. Willis J.Potts, William Riker, Arthur DeBoer, Thomas G.Baffes and Farouk Idriss.

References1. MORGAGNI, J. B.: De Sedibus et Causis

Morborum. Epist. 18, Article 6, 1760.2. BONNET, L. M.: Sur la lesion dite stenase

congenitale de r'aorte dans la region del'isthme. Rev Med (Paris) 23: 108, 1903.

3. EDWAwDs, J. E., CHIuSTENSEN, N. A., CLAGETT,0. T., AND McDONALD, J. R.: Pathologicconsiderations in coarction of aorta. Proc MayoClin 23: 324, 1948.

4. EDWARDS, J. E., CAREY, L. S., NEUFELD, H. N.,AND LESTER, R. G.: Congenital Heart Disease,vol. 2. Philadelphia, W. B. Saunders Co., 1965,p. 677.

5. LEV, M.: Autopsy Diagnosis of CongenitallyMalformed Hearts. Springfield, Ill., Charles CThomas, Publisher, 1953, p. 58.

6. DEBoER, A., GRANA, L., PoTTs, W. J., AND LEV,M.: Coarctation of the aorta. Arch Surg 82:801, 1961.

7. GLAss, H. I., MUSTARD, W. T., AND KEruI, J. D.:Coarctation of the aorta in infants. Pediatrics26: 109, 1960.

8. MORTENSEN, JD, CUTLER, P. R., RUMEIL, W. R.,AND VEAsY, L. G.: Management of coarctationof the aorta in infancy. J Thorac Cardiovasc


Surg 37: 502, 1959.9. FREUNDLICH, E., ENGLE, M. A., GOLDBERG, H.

P.: Coarctation of the aorta in infancy.Pediatrics 27: 427, 1961.

10. MALM, J. R., BLUMENTHAL, S., JAMESON, A. G.,AND HUMPHREYs, G. H., II: Observations on

coarctation of the aorta in infants. Arch Surg86: 96, 1963.

1 1. ADAM., M., JOHNSON, A. F., JR., DAVIS, M. V.,AND MITCHELL, B. F.: Surgical management ofcoarctation of the aorta in infancy. Ann ThoracSurg 2: 188, 1966.

12. LINDESMITH, G. G., GALLAHER, M. E., ANDDURNIN, R. E.: Cardiac surgery in the firstmonth of life. Ann Thorac Surg 2: 251,1966.

13. HARTMAN, A. F., JR., GOLDRING, D., AND STAPLE,T. W.: Coarctation of the aorta in infancy. JPediat 70: 95, 1967.

14. LYNFIELD, J., KOTTMEIER, P., VICHITBANDHA,P., AND TANG, J. S.: Preductal coarctation inthe neonatal period and early infancy. (Abstr.)Program and abstracts, Section on Cardiology,American Academy of Pediatrics, Chicago,October 1966.

15. LANG, H. T., JR., AND NADAs, A. S.: Coarctationof the aorta with congestive heart failure ininfancy. Pediatrics 17: 45, 1956.

16. MUSTARD, W. T., ROWE, R. D., KEITH, J. D., AND

SIREK, A.: Coarctation of the aorta withspecial reference to the first year of life. AnnSurg 141: 429, 1955.

17. NADAS, A. S.: Pediatric Cardiology, ed. 2.Philadelphia, W. B. Saunders Co., 1963, p. 536.

18. KEITH, J. D., RowE, R. D., AND VLAD, P.: HeartDisease in infancy and Childhood, ed. 2. NewYork, Macmillan Co., 1967, p. 237.

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19. WALDHAUSEN, J. A., KING, H., NAHRWOLD, D. L.,LUIUE, P. R., AND SCHUMAKER, H. B.:Management of coarctation in infancy. JAMA187: 270, 1964.

20. DUFFIE, E. R., JR., AND WILSON, J. F.: HeartDisease in Infants, Children, and Adolescents,edited by Arthur J. Moss and Forrest H.Adams. Baltimore, Williams & Wilkins Co.,1968, p. 391.

21. MCNAMARA, D. G., AND ROSENBERG, H. S.: InPaediatric Cardiology, edited by HamishWatson. St. Louis, C. V. Mosby Co., 1968,p. 175.

22. Moss, A. J., ADAMS, F. H., O'LOUGHLIN, B. J.,AND DIXON, W. J.: The growth of the normalaorta and of the anastomotic site in infantsfollowing resection of coarctation of the aorta.Circulation 19: 338, 1959.


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ALEXANDER J. MUSTER, HANS U. WESSEL and MILTON H. PAULSACHCHIDA N. SINHA, MERL L. KARDATZKE, ROGER B. COLE,

Coarctation of the Aorta in Infancy

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1969 American Heart Association, Inc. All rights reserved.

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