Clinical and pathological findings in cases of truncus arteriosus in infancy

Clinical

and Pathological

01r

Truncus

Findings

Arteriosus

in Cases

in Infancv*

HELEN B. TAUSSIG, M.D. BALTIMORE, MARYLAND

T

years

HE exact

definition

arteriosus

has been

considerable

the condition

that

to represent

For

large

many

in order

a true

defined as a single great vessel of abnormally

of

large

caliber,

for

four semi-lunar

valves,

the blood

and the coronary

arteries

its base.

recent

During

by

aorta

of

general

velop;

that even though

is usually

fatal occurs

nary arteries

arise at its base and the pul-

arteries

are given off from this great

this anomaly,

l1 in her extensive

study of

attempt

presented

to show

dition

that cases in which

evidence

the pulmonary

failed to meet the ventricles and in which the circulation was by way of the bronchial an even earlier

arrest

indicate

arteries

arteriosus

In

most

fails to deimportance.

lived to late child-

within

the first week of

for Danelius’s4

of the normal

report

of the

hilar “coma”

found

arteriosus,

virtually

has been made to diagnose during

no

the con-

life. The

following

two cases

that in infancy

a truncus

arteriosus

causes the heart to assume a distinctive contour; indeed the contour is so unique that

or the aorta? to the lungs arteries,

to the lungs is by

in infancy;2,5v13,15,22 death

in cases of truncus

Humphreys,

sented

Except

absence

vessel.

the

arteries.

the individual

frequently

monary

the

from

or early adult life,3v14*23the condition

arteriosus,

life.

either

a few cases have been reported

as a truncus

the coro-

i.e.,

it is never of functional

in which hood”

and pumps

directly

bronchial

guarded by two, three or four semi-lunar valves, the condition may still be considered provided

arises

the ductus

Although

of the single great vessel may be

pathways,

artery the

instances

the

receives

to the body and to the lungs by

or the circulation

way

should arise from the

the base of which

arise, and which

of arterial

pulmonary

from this vessel

years

consensus1*11*15 has been the orifice

way

and the pulmonary

should arise directly

from

arteries

the blood from both ventricles

truncus

guarded

caliber,

coronary

there must be a single great vessel

of abnormally arteries

the subject

discussion.

it was maintained

arteriosus

of a truncus

the

repre-

in the forma-

clinical

relative

diagnosis

can

be made

with

ease.

tion of the great vessels than did a truncus arteriosus in which the pulmonary arteries arose directly from the aorta. She cited one case in which one pulmonary artery arose directly from the aorta and the other pulmonary artery ended blindly. The circulation to the latter lung was by way of the

half months of age because of heavy breathing, failure to gain weight and diarrhea. Physical

bronchial arteries. A truncus arteriosus

examination revealed the temperature to be 37.2%., pulse 150, respiration 50, weight 2.8

* From the Harriet

may,

therefore,

CASE REPORTS CASE I. W. T., (H. L. H. No. 99456), a colored male infant born July 5, 1936, was first seen at the Harriet Lane Home at three and one-

be

Lane Home of the Johns Hopkins Hospital and the Department University School of Medicine, Baltimore, Maryland.

26

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‘I’runcus

Arteriosus-‘~a1~s.r~~

FE. 1. ,Y-ray of hrart of Case kiloxralns. ance

56 cm. The

was that of a markedly

infant

\vho was breathing

showed The

height

slight

heart

but

definite

\vas well transmitted ‘I‘hc

limited

author

to systolc;

point differed, was

a

was a harsh

over the heart which

into the vessels of the neck

throughout

thought

the

however,

thoracic

murmur

opinions

systole

murmur

which

an d diastole.

sound at the base wxs accentuated

was

on this

and many persons thought

continuous

who

cyanosis.

and readily heard all over the posterior wall.

negro

and

persistent There

maximal

appear-

emaciated rapidly

wxs enlarged.

systolic murmur

general

there

extended

The

second

and had the

purit)- Mhich occurs when there is but one great vrssel. The 1unq-s were clear. The liver extended halfway

to thr umbilicus

but did not pulsate.

I

in antcropostrrior vir\v.

the

antrrior

ventricle

the

4.0 million l)rr cu. mm.. p
was

80/W.

of barium

aortic knob.

In the anteroposterior of In

the

curved markedly

esophagus region

In add.ition,

was displaced view the dis-

was c‘ven more

of the

left

auricle

T2 given

showed relatively

OK 2 mm.

in

congenital

heart. Therr

deflection

malformation

was no axis deviation.

‘l’he clinical congenital

impression

malformation

high

above- the iso-

electric line, and the wide ventricular common

it

to the left. (Fi,q. 3.)

The electrocardiogram P waves,

a sharp

was visible at the

backward. striking.

lrft

to be huge.

placement

the

The

(Fig.

of

the

3.)

was that of a severe of

the

heart.

of thr hrart in the anteropostcrior

The view

showed a lack of the shadow cast by

the normal

pulmonary

artcry.

a narrow

aortic

hemoglobin,

14 Gm.

shadow and a pronounced

revealed

a heart

greatly enlarged. A41though in the antcroposterior view the contour of the heart was similar

examination

a remarkable

posterior

arm

2.)

the lo\zer part of the esophagus

distinctly

in

(Fig.

of the esophagus

level of the prominent

‘I‘htz blood

data showed: red blood ccl1 count

also appeared

angulation

contour

pressure

wall.

CJpon the administration

Thrare was a strong pulse in the femoral artery. I,aboratory

chrst

contour.

auricle In

the

anrero-

view there was a sharp angulation

of

was

huge

and

aortic knob. The left both

ventricles

were

to that of a non-functioning right ventricle. in the left anterior oblique position the contour

the cardiac shadow to the left of the sternum. ‘Th~~r-c~was no visible pulmonary conus; the aorric shadow was narrow but the aortic knob

indicated that the right ventricle was huge. In short, it was a new contour which suggested

was conspicuous. (Fig. 1.) On rotation of the infant into the left anterior oblique position. the

that there was a pulmonary atresia and that the aorta over-rode both ventricles and enlargement

right ventricle appeared greatly enlarged and extcndcd abruptly outward from the aorta to

of the left auricle. The paliznt failed to qain weight.

He became

28

Truncus

Arteriosus-

~uussig

2A.

FIG.

FIN. 2. X-ray of heart in Case I of left anterior

oblique

(A)

and right

(B)

positions.

increasingly

normally

Follis.) The heart was greatly enlarged. It measured 5 cm. in transverse diameter and lay more to the right than was normal. The superior

enlarged. The foramen ovale was completely sealed. The right ventricle was a relatively small chamber but its wall was enormously hyperit measured 7 mm. in thickness. trophied;

vena cava

There

cyanotic and died of cardiac failure at four months of age. (Performed by Dr. Autopsy Report No. 15057.

and the inferior

vena

cava

opened

into

the

right

auricle,

which

was a defect in the membranous AMERICAN

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Truncus of rhe ventricular

20

Arteriosus-‘raussig

septum. The large aorta arose

al~ovr the defect. The aortic orifice was guarded by three semi-lunar werr normal.

cusps. The coronary

The pulmonary

thin-walled

arteries

artery was a small,

vessel which branched

to the lungs

in the normal fashion, but ended blindly (Fig. 4) and did not communicate aorta.

The

cord

ductus

or remnant

found: ir had

been. The

ri?hr.

It.

measured

The

artery

Icft auricle

left ventricle was

too,

The

aorta

above

the aortic

became

rower as it arched The

intercostal

arteries

orifices

the

immediately 4.5 cm. in

progressively

thcsr

\rcssels were

were greatly

were enlarged thicker

artery.

to hc caught

wall

nar-

posteriorly.

their

pulmonary

the

its

An intrrventricu-

was enlarged;

It

than

visible beneath

valve it measured

circumference.

in tht

was
hypertrophied:

9 mm. in thickness.

No was

to show whcrc

was larger

lar srptal defect was clearly aorta.

arteriosus

there was no puckering

or the pulmonary

Iarqd.

was absent.

of the ductus

moreover,

aorta

with the heart or the

arteriosus

Thr

between

and than

the

walls of

that

esophagus

arteries. (Fig. 4.) One branch of a bronchial

dilated;

the

of

the

was found

dilated

intercostal

artery was injected

and Huid emrrged

from the pulmonar),

and the pulmonary

veins at the same time, which

indicated

that there was a definite

between the bronchial artery.

The

extensive artery

of the vessels was so

injection

of the

bronchial

the differentiation

arteries from the pulmonary

.4natornical

tion

the

did not permit

bronchial

anastomosis

artery and the pulmonary

anastomosis

that

pulmonary

Congenital

Iliagnosis:

of’ 1he

artery

heart.

Displacement high

atresia,

Frr:. 3. Electrocardiogram

of the artery.

The

distortion

aorta,

due

to the large

septal

adequate

malformaof

ventricular

was caught

arteries,

arteries.

cardiac

hypertrophy,

chronic

conqcstion and lobular pneumonia. In brief. the malformation was rruncus

arteriosus

with

Comment.

topsy tht-

The

explained enlargement

narrow

aortic

condition the

that

the circulation

lungs by way of the bronchial

clinical

of

both

shadow

and

passive of a to the

at

observation ventricles, the large

CASE

February

due

auof the

aorta.

left

auricle

II.

brtween

\vits in part (for

was cvrr

to the fact the

that

which

found:)

no and

the esophagus

dilated

bronchial

Baby H. a white female infant born

0, 1943, at the Woman’s

seen in consultation

arteries. found

in part

of the esophagus

explanation

defect, premature obliteration of ductus arteriosus, closed foramen ovale, enlarged bronchial

of (:asc. 1.

Hospital

was

at ten days of age because

of a heart murmur. The infant did well for the first week of life and then began to do poorly. Physical examination revealed the pulse to be 140, respirations 30 to 40 per minute. The infant’s color was variable. At times it was

30

Truncus Arteriosus-

Kzussig

Fro. 4. Drawing of the heart of Case I; a truncus arteriosus with the circulation to the lu&s by way of the bronchial arteries. nor .mal and then it would become definitely The cya .notic. Crying lessened the cyanosis. hea trt was enlarged. The heart action was quiet; the sounds were of good quality. The second sou nd at the base was loud. There was a definite

precordial

thrill

and a harsh

systolic

murmur

was heard all over the chest. The liver extended two fingerbreadths below the costal margin and did not pulsate. There was a strong pulse in the radial artery and in the dorsalis pedis. AMERICAN

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Truncus Fluorosropic

examination

IO be greatly markedly

enlarged.

dilated.

showed

The

The

right

the heart auriclr

pulmonary

conus

not appear to be full. In the left anterior position

the right

tcntled abruptly In

Ihe

right

ventricle

oblique

chest wall. the

dt+cr

septum.

and

the liver descended follo\ving morning

to the um-

she died.

rapid failure of the circulation

indicated

~Ilalformation

heart

wrio~ts

isolarcad yross defect

of

the

The

a more than

an

in the interauricular

sep-

1un1. It was then realized that the abrupt

angu-

lation

and the great

ventricle

enlargement

of the right

seen in the left anterior

tion was consistent

oblique

posi-

with a truncus arteriosus.

‘T‘he final clinical diagnosis was: A gross defect in the

interauricular

arteriosus

wirh

septum

bronchial

nosis ivas made because

and

a

arteries.

truncus

This

diag-

of the similarity

of the

c‘ontour of the heart in the left anterior

oblique

I)osirion 10 that of the fn-st case. .-~uLo/I?J,. iPerformed haart was enormously rhc

left costal

richr

hcmithorax.

the inferior auricle

enlarged. and

normal

defect

Hellijas.) filled

into

the

There

right

was an

in the interauricular

septum;

half of the septal wall was lacking.

tricuspid

valve had only two leaflets.

venIricl<~ was rrophird.

enlarged

At rhe basr

and

its

of the

to the

vena cava and

opened fashion.

The

It extended

almost

The superior

\rrna cava

in the

c’normous nearly

margin

by Dr.

The

The right

wall

hyper-

interventricular

sei)tum was a defect which communicated

with

the left vcntriclc. The pulmonary veins opened into the left auricle which was not enlarged. The mirral valve was normal. lar septal

defect

lay beneath

The interventricuthe

base of the

aorta. A single great vessel, the aorta or truncus arteriosus,

arose above

the defect

in the inter-

ventricular st’ptum and thus received blood from both ventricles. The orifice of this vessel \+‘as quardcd by three semi-lunar valves, and th(s coronary arteries were given off in the nornA manner. The pulmonary arteries were +~:rl of directly from the aorta. The ductus

artery

or in thr .lorta

I;inni .48intomL~ffiDiagn0~i.s:‘I‘runcrus ;trrt*riosus with

the

pulmonary

art&es

arising

ditcc.tly

from the aorta, a yross defect in the inleratlricular septum and a high ventricular Comment.

diagnosis that

‘I‘hree days later rh(a infant became extremei! bilic,us. The

and there \<.>I5nc, indica

atmmt

that it had t’vcr existed.

was that of a gross

dyspnvic

LV~S

tion in the pulmonary

rsophaqram MYISnormal. The clinical impression in the interauricular

arteriostls

31

did ex-

position

‘Tmhq

was

ohliquc

was huge and

out to the anterior anterior

Arteriosus-

Autopsy

septal dcf?ct. tht> clinical

confirmed

of a truncus artcriosus

the pulmonary

artcries

but sho\ved

arose dircctl)

from the aorta. The fact that the pulmonar) artcry arose directly plained the occurrence minimal

from the aorta (axof the transitorv and

I..)-anosis. COMMENTS

The first case is one of a truncus arteriosus with bronchial

arteries.

In the second case

the pulmonary

arterirs

arose directly

the aorta

and c\‘en though

but two weeks of age, arteriosus

was found

cases differed portant

respects;

the pathway

the infant

at autops>a. ‘I’hc two

first,

other and

of in

to the lungs artcrics;

I_Ton-

only a small volume of blood the lungs for aeration ad ml). a

small volume turned

SCY~OIK~,

by which the blood I-cached the

was by way of the bronchial reached

in two im-

in the structure

septum.

lungs. In Case I. the circulation sequcntly,

was

no tract of a ductus

from each

the intcrauricular

horn

to

of oxygcnatrd

the

left

auricle

ventriclc.

Thil;

blood

relatively

large volume

blood

wa?; rc-

and

the, left

was mixed

\vith the

of blood Mhic-h M’as

directed to the systemic circulation and was returned by the superior vcna cave and the inferior vcna cava to the ri+t the right vcntriclc. It follows volume of oxygenated

auricle and that a small

blood M;C?S rnixcxd with

a large volume of unoxygenated blood and only a small volume of ox)~genatetl Mood was pumped into the systemic circulation; cyanosis was intense. In the second case the pulmonary arteries arose directly from the aorta and the circulation to the lungs ‘was far more adequate. Indeed, the c-ir-culafion

32

Truncus

FIG. 5. Drawing

of a heart,

(illustrative

arteries

Arteriosus-

of Case II); a truncus

arise directly

was such that when the oxygenated

Emssig

from the truncus

blood

returned to the left side of the heart and was mixed with the venous blood returned from the body to the right side of the heart, there was no “visible” cyanosis. These two cases differed greatly in the structure of the auricular septum. These differences were reflected in the fluoroscopic and x-ray findings. In the former, the left auricle was markedly enlarged, whereas in the latter, the right auricle was huge.

arteriosus

in which the pulmonary

arteriosus.

The structure of the ventricles was identical in the two cases. In both, the single great vessel arose directly from the two ventricles; consequently, in both instances there was a high ventricular septal defect. The structure of the heart in Case I is illustrated in Figure 4; a counterpart of Case II is shown in Figure 5, which, although drawn from a different specimen, is an exact replica, except that in Case II there was in addition a gross defect in the interauricular septum. AMERICAN JOURNAL

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Truncus It is of interest the contour

and of significance

of the ventricles

cases was identical. posterior fiillncss

view of

In Case

pulmonary

the

antc>rior oblique anterior my

two

and

of the

second

case

directly

from the aorta;

normal

conus was not as

position

abruptly

to the lungs was by way of the bronchial arteries

in the antero-

II

striking as in Case I. Nevertheless, extcndcd

that

in these

the absence

in the left

knowledge

no other malformation

causes

shelf. Such a contour

alized in the left anterior

oblique

visu-

position is

cyanosis

cyanosis

was intense.

the pulmonary

was adequate

arose there

to the luncr;s and

was absent.

In each

extended

oblique instance

out abruptly

the anterior topsy

the

artrries

circulation

the left anterior tical.

In

consequently,

In both cases the contour

the right vtmtriclc

out from the aorta to the

chrst wall as a shelf. To the best of

such an abrupt

Taussig

Arteriosus-

chest

was iden-

the right

ventricle

from

wall

the structures

of the hcaart in

position

the aorta

as a shelf.

to

:\t au-

of the vcntriclrs

and

virtually diagnostic of a truncus arteriosus. ‘l’hcrc are. in addition, two other features

their relation to the truncus arteriosus werr found to be identical. Thereforr, the author

which aid in the establishment

believes

diagnosis. aorta

The

and

prorninrnt Indrcd.

first is the abnormally

the prominent aortic

knob

normally

aortic

is rare

larg?

knob.

,A

in infancy.

the aortic knob is hidden

behind

the stc‘rnum.

always

arouse‘ suspicion

of a truncus

of the correct

A large

aorta

of thr

artcxriosus. The

should

is one \vhich I)anclius” has emphasized. namely, the ahsencc of the normal “coma“ shadolv.

In my expcricnce

diminished

hilar

shadows arc common in all malformations in which there is diminished blood flow to the lungs. It is scan in a truncus with

bronchial

pulmonary-

artcries

artery

artcriosus

but not when

arises

directly

from

the the

aorta. ‘l‘hc clinical diffcarentiates artrriosus

f?aturr thcsc

is the

which

two presence

most sharply

types or

of a truncus the ventricles

of truncus absence

of

cyanosis. When thr circulation to the lungs is through thy bronchial arteries, cyanosis is intcnsc: whereas, when the pulmonar)

SUMMARY

‘l‘wo cases of truncus arteriosus in infants arc reported. In the first case the circulation .AhlKKICAUJO”‘RNAf OF YEDICINI:

arteriosus

septum

and

is characteristic

in infancy

are normally

the aorta

over-rides

receives

in which

formed

and in

thr ventricular

blood

from

both

ventricles. REFERENCES 1. ABBOTT, M. E. Osler’s Modern Medicine. Edited by McCrae. Chap. 21, vol. 4. pp. 6121-812. Philadelphia, 1927. Lea and Fcbiger 2. BEAVER, D. C. Persistent truncus arteriosus and rongenital absence of one kidney with other drvelopmental defects. Arch. Path. 15: 51-54. 1933. 3. CARR, 1’. B., GOODALE, R. H. and ROCKWEI.I * A. E. I’. Persistent truncus arteriosus in a man aged 36 yrs. Arch. Path., 19: 833-837, 1935. 4. DANELIIJS, G. Absence of the hilar shadow. A diagnostic sign in rare congenital cardiac malformations (truncus arteriosus solitarus with hrtcrotopic pulmonary blood supply). .4rrr. ,7. Rorntgenol., 47:

870-876, 1942. 5. DAVISON, G. Case 6.

7.

arteries arise directly from the aorta. there is adcquatc circulation to the lung-s and cyanosis is minimal or absent. Nevertheless, the contour of the heart in the two conditions is identical.

this contour

which

possibilit)

second feature

rhat

8.

9. 10.

of congenital heart disrasc with single arterial trunk. 3. ,4nat., 69: 423 420, 1935. FINLEY, I(. H. .4 congenital anomaly of the hc-art (truncus arteriosus communis with subacute Icndocarditis). Am. J. Path., 6: 317, 1930. ChLTMAN, D. W. and $rERN, N. S., Congenital heart discasc. Report of a case of dextroposition, persistence of an early stage of embryonic development of the heart, prrsistrnt truncus arteriosus, abnormal systemic and pulmonic wins, and subdiaphragmatic situs inversus. :lm. ffezn;d J., 18: 176-187, 1739. GRAHAMS and MONTGOMF,‘.KY, C;. I.. Congenital malformation of heart: persistent truncus artrriosus. ,7. 7wh. Methods, 18: 97-100, 1938. GIUSTRA, F. X. and TOSTI, V. G. True car biloculart in identical twins. Am. Heart J., 17: 249-250, 1939. HARRIS, H. A. and THOMSON, G. C. Prrsistent truncus arteriosus communis with microphthalmos, orbital cyst and polydactyly. .4rrh. ni\. Chdd., 12: .5%66. 1937.

Truncus

Arteriosus-

11. HUMPHREYS, E. M. Truncus arteriosus communis persistent; criteria for identification of common arterial trunk with report of case with 4 semilunar cusps. Arch. Path., 14: 617-700, 1932. 12. HUNTER, 0. B., JR. Truncus arteriosus communis persistens. Arch. Path., 37: 328-330, 1944. 13. HUNTER, W. C. and MACKEY, H. E. Sympus apus, with associated truncus arteriosus communis. Am. J. Obst. @ Gym, 29: 726-730, 1935. 14. KLEMKE. W. Ein klassischer Fall van totaler Persist& des Truncus Arteriosus Communis. Centralbl. f. ally.Path. u. path. Anat., 36: 307-312, 1925. 15. KETTLER, L. H. Zur Frage der Persistenz des Truncus Arteriosus Communis. Virchow’s Arch. ,f. path. Anat., 304: 513-525, 1934. 16. LEV, M. and SAPHIR, 0. Truncus arteriosus communis persistens. 3. Pediat., 20: 74-88, 1942. 17. MARSHALL, R. Persistent truncus arteriosus. Bril. Heart J., 5: 194-196, 1943.

Tawtig

18. MICHELSON,R. P. Report of case of car biloculare with persistent truncus arteriosus. Am. Heart ,7., 25: 112-115, 1943. 19. MILLER, M. K. and LYONS, M. W. Persistent truncus arteriosus: cardiac hypertrophy, dysphagia, death on eleventh day. Am. Heart J., 7: 106-109, 193132. 20. ROOS, A. Persistent truncus arteriosus communis; report of case with 4 semilunar cusps and aortic arch on right side. Am. 3. Dis. Child., 50: 966978, 1935. 21. SHAPIRO, P. F. Truncus solitarus pulmonalis. A rare type of congenital cardiac anomaly. Arch. Path., 10: 671-676, 1930. 22. SZYPUIXCI, J. T. Study of congenital heart disease at Philadelphia General Hospital. 3. Tech. Methods. 17: 119-126, 1937. 23. ZIMMERMAN,H. M. Congenital anomaly of heart; truncus arteriosus communis. Am. 3. Path., 3: 617-622, 1927,

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