MITRAL INSUFFICIENCY DUE TO ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY

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MITRAL

INSUFFICIENCY

DUE

TO

ANOMALOUS

ORIGIN

OF

THE

LEFT CORONARY

ARTERY

FROM

THE

PULMONARY

ARTERY

Harry R. Foster, Jr., Jack W. C. Hagstrom, Kathryn Hawes Ehiers,

and Mary Allen Engle

Departments of Pediatrics and Pathology, The New York Hospital-Cornell University Medical College,

New York, New York

(Submitted April 7;accepted for publication June 11, 1964.)

This work was supported in part by American I-heart Grant 63 G 87.

H.R.F. is Research trainee, N. Y. Heart Association, K.E. is Research Fellow, American Heart

Asso-ciation, with support by Tompkins Co., N. Y., and Essex Co., N. J., Heart Association.

ADDRESS: (MAE.) Department of Pediatrics, New York hospital-Cornell University Medical College,

525 F. 68th St., New York 21, N. Y.

649

PEDIATRICS, November 1964 ECENT reports of survival into

child-hood15 and of associated mitral valve

involvements’ 58 have expanded the knowl-edge of the natural history of anomalous origin of the left coronary artery from the

pulmonary artery. The information has

shed additional light on the controversy concerning direction of blood flow through

the anomalous vessel,’ and on indications for surgery.1215 The 2 cases to be described bring out these points.

CASE REPORTS

Case 1 was referred in 1961, at the age of 8 years for evaluation of a heart condition which had caused much difficulty in infancy but less in

childhood. According to her mother, who is a

nurse, and her pediatrician, Dr. Charles Deich-man, she was placed in oxygen during the first

eek of life because of bouts of cyanosis and a

rapid pulse. She then did well until 3 months,

when she began to vomit frequently and again

to have episodes of cyanosis and tachycardia. The heart was found to be enlarged, and an apical

systolic murmur and gallop rhythm were heard.

By the next month her pulse rate had risen to 170

per minute, she had rapid respirations, and she had stopped gaining weight. Digitalization pro-duced improvement. This medication was

discon-tinned at 11 months, by which time the episodes

had disappeared. Thereafter, the only symptoms were easy fatigueability and difficulty in

concen-trating at school. The murmur at the apex

per-sisted.

At 8 years, her height and weight were at the 50th percentile. The pulse was 100 and the blood

pressure was 100/60 mm Hg in the arms and 130/70 in the legs. There was no cyanosis, cardiac

thrill, or unusual pulsation, but there was a

pre-cordial bulge. A holosystolic murmur was heard

at the apex and lower left sternal border. The

second sound was normally split; the aortic

com-Ponent was loud. Except for a third heart sound at the apex, diastole was clear (Fig. 1).

Cardiac fluoroscopy revealed left ventricular

en-largement in the left anterior-oblique projection.

Barium swallow disclosed moderate left atnial en-largement. The main pulmonary artery was ab-normally convex but normally pulsatile, and the over-all pulmonary vascularity was average.

Elec-trocardiogram (Fig. 2) showed left axis deviation,

kft ventricular hypertrophy, and abnormal T

waves in leads 1, a VL, and V4-6.

The clinical impression was that there was some

abnormality of the left ventricle in addition to

mitral insufficiency or a ventricular septal defect.

She was scheduled for right and left heart

catheteni-zation and aortography. However, just before

ad-mission, she died suddenly after playing in a swing. Postmortem examination was done by the

mcdi-cal examiner. Dr. I. M. Cleveland kindly sent us the report and the specimen for study. The fixed heart weighed 152 gm (expected 115 gm). The left coronary artery originated from the posterior sinus of the pulmonary artery and soon bifurcated

to form the anterior descending and circumflex

branches. The right coronary artery originated and

coursed normally. Its ostium was 9 mm in diam-eter, 2 mm greater than the left.

The leaflets of the mitral valve were uniformly thickened by dense, white fibrous tissue and were attached to the papillary muscles by coarse, con-tracted chordae tendineae. The anterior papillary muscle was strikingly deformed; it was rigid and covered by multiple calcific excrescences that gave the thick endocardium a warty appearance (Fig.

3).

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Fic. 1. Case 1. Phonocardiogram shows holosystohic murmur of plateau configuration maximal at apex. It is also recorded at fourth left interspace (4 US). The second sound is narrowly split. Third heart

sound present. (Time lines 0.04 sec. Frequency 120/500 c.p.s.)

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650 MITRAL

INSUFFICIENCY

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-FIG. 2. Case 1. Electrocardiogram shows left axis deviation, horizontal heart, left

ventricular hypertrophy, and diphasic T waves in leads V4 through V6 and lead

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Foe.. .3. Photograph of a section of the wall of the left ventricle and anterior papillary muscle

show-ing o1)acified endocardium, multiple irregular,

cal-cifil excrescences on the white surface of the

Papillary muscle, and thickening of the chordae tendineae and mitral valve leaflet.

and atrium was thick and white. The wall of

the dilated and hypertrophied left ventricle meas-tired 15 nim. The mvocardium was irregularly

re-placed by fibrous tissue that was especially dense

beneath the endocardium. Histologic sections (Fig. 4) showed that large amounts of fibrous tissue

en-cased or replaced myofibers and often surrounded

blood vessels. In the outer half, myofibers were

moderately hypertrophied and scars were focal.

The endocardiunl of the left ventricle and atrium

was niarkedly thickened by proliferated collagen

and elastic fibers. In the anterior papillary muscle the endomvocardium was replaced by acellular fibrous tissue containing calcific deposits. The

mitral valve leaflet, chordae tendineae and

endo-cardium were thickened by dense hypocellular

fibrous tissue in which lay small deposits of

cal-cium (Fig. 4).

Case 2 was born March 3, 1963, to a 20-year-old

Illother after an unconiplicated term )regnancy.

Al-though normal at birth, she became irritable, fed

poorly, and gained slowly. She slept more than

ha(l her older sil)ling. Her respirations i)ecame in-creasinglv rapid and labored. When 3 months of

age, her pediatrician detected a large heart,

sys-tolic murmur, and congestive failure. She was

hos-pitahized for digitalization and oxygen. Initial

im-provement was not maintained, and she was re-ferred for evaluation and treatment.

On physical examination, her weight was below the 3rd percentile, although the length was at the 50th percentile. She was an anxious baby in ob-vious respiratory distress but without cyanosis. Slightly distended neck veins showed prominent

l)tllsatiOns. There was a bulge of the left lateral chest wall overlying the apex impulse, which was

in the anterior axillary line. There were no thrills.

A soft, high-pitched holosystolic murmur was heard

at the apex (Fig. 5); it radiated toward the

ster-nurn and tile axilla but could not be heard over the back. 1-leart sounds were normal except for an S3 gallop. No rales could be heard. Tile liver

was palpable 2 cm below the costal margin and the tip of the spleen was felt. The ptes were

feeble but equal in all extremities.

The submitted electrocardiogram taken prior to

digitalis therapy and the tracing on admission

(Fig. 6) showed changes in ST and T waves

sug-gesting myocardial ischemia. The QRS was widened

to 0.09 sec due to a conduction disturbance of the left ventricle. The record also showed right axis

de-viation as well as right atrial enlargement. X-ray

and fluoroscopv of the chest revealed marked

car-diomegak, especially involving tile left atrium and

the left ventricle, which barely pulsated. There

was an infiltrate in the right upper lobe.

The impression was anomalous origin of the left

coronary artery from the pulmonary artery.

Mcdi-cal measures to control the heart failure were

in-tensified and diagnostic studies were undertaken.

Intravenous angiocardiogram (Fig. 7) and

cin(’-angiocardiogram revealed marked anterior and

superior displacement and compression of the right

atrium and right ventricle by a large and feebly

pulsatile, thin-walled left ventricle and enlarged

left atniunl. In the lateral view a vessel could be

seen to arise posteriorly from the pulmonary artery, but its opacification was transient and its course

uncertain. It was believed to be an anomalous left

coronary artery. For confirmation a retrograde

aortogram (Fig. 8) was performed vith injection though a catheter positioned above the aortic

valve. The right coronary artery filled first; then

by way of numerous tiny branches, a small anterior

descending and circuiiiflex branch of the left

cor-onary artery filled and in turn emptied into the

pulmonary artery. The angiographic findings are

being reported in greater detail by Stein et al”

With full realization not only of her precarious

condition but also of the apparent bidirectional flow of blood in this anomalous coronary and of the extensive myocardial damage evident, it was

believed that surgery should be undertaken to

ligate the vessel near its origin. The operation was

performed on July 31, 196-3, with immediate

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-FIG. 4. Photornicrograpll of the left ventricular endocardium and mvocarclium. There arc subendocardial

calcific (1e1)OSitS and replacellient of the mvocardium b’ bands of fibrous tissue that extend from the endocardium.

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FIG. 6. Case 2. Electrocardiogram shows right axis deviation. ‘ihere is nlarked ST

(lepression ill lea(ls 2, :3, and aVF \vitil reciprocal elevation ill leads 1, aVL, and

\‘5-6. The conduction disturbance over the left ventricle (rsR’ in \‘O) combined

with the striking elevation of ST segment in V6 suggests anterolateral mvocardial

infarction. V2-6 arc at half standardization.

left ventricle and no change in the

electrocardio-gram. However, on transfer to the recovery room

nodal tachycardia and then ventricular fibrillation

developed. Normal rhythm could not be restored.

At postmortem examination the heart and lungs weighed 162 gm (expected 97 gin). The left atrium

and left ventricle were dilated and lined with thick, white endocardium. The myocardium of the left ventricle was partially replaced by fibrous

tis-sue and there were focal areas of calcification in

the myocardium and anterior papillary muscle.

Though the posterior papillary muscle appeared

1)ink, the anterior muscle was atrophic, pale, and

shortened (Fig. 9). The anterior leaflet of the

mitral valve was thus incompetent. A ligature

en-circled the anterior descending branch of the left

coronary artery 1 cm below its origin from the

i)ulmonary artery. The terminal branches

anasto-mosed with those of the right coronary through a

tortuous array of small blood vessels that formed

fine plexus over the surface of the heart. The

right coronary artery originated and coursed

nor-mally.

Microscopic study confirmed the extensive

fi-brosis with circumferential hypertrophy of the

unaffected myocardium. The anterior papillary

muscle was particularly scarred with focal

cal-cification of the junction betveen endocardium

and myocardium. Embryonal sinusoids were not

present.

COMMENT

Mitral insufficiency as a presenting sign

has been reported in four cases of

anomo-bus origin of the left coronary from the

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ele-654 MITRAL INSUFFICIENCY

and l)I1h1llonaY artery. Arrow i s the anoma- FIG. 8. Retrograde aortogram shows a large right

bus left coronary artery, which can be seen arising coronary arising normally and filling the left

cor-from the base of the main pulmonary artery. onary through a network of fine anastornatic

chan-nels. Arrow indicates puff of contrast medium

en-tering pulmonary artery from the retrogradely

filled left coronary artery.

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Fic. 7B. The left ventricular phase of the intra-venous angiocardiogram in frontal projection shows a large left atrium and very large, thin-walled left

ventricle which emptied poorly

and atrophy of tile anterior papillary muscle which

appears white in contrast to the normal posterior

papillary muscle. The mitral valve leaflet is thick

and retracted.

vated pulmonary artery pressures might lead one to suspect that significant mitral

insufficiency was present, though

unrecog-nized, in other cases.

Of the two additional cases we are

re-porting, one died suddenly in childhood r surviving heart failure and ischemie

episodes in infancy, while the second, an

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liga-tion of the anomalous vessel. Both had

aus-cultatory and radiologic as well as patho-logic evidence of mitral insufficiency.

The mitral insufficiency results from scarring of the anterior papillary muscle,

which is the most distal point of blood

sup-ply of the anterior descending coronary

artery. It is, therefore, the area most

vul-nerable to ischemia from inadequate cor-onary perfusion. The resulting fibrosis with

shortening and retraction of the chordae

tendineae leads to an incompetent valve. The marked dilatation of the left ventricle

and the presence of endocardial fibroelas-tosis may also contribute to distortion of

the mitral ring. An analagous situation in adults has recently been noted by Phillips,

Burch, and De Pasquale.18 They described papillary muscle dysfunction and mitral

in-sufficiency following anterior myocardial

infarction.

It seems reasonable to believe that mitral insufficiency under these circumstances

im-poses an added burden on an already in-jured left ventricle. However, since the

four patients presented in previous papers and our Case 1 lived to an age beyond that

anticipated with this anomaly, it may be

that mitral insufficiency led to some

benefi-cial adjustment by causing pulmonary hy-pertension. An elevation of pulmonary

ar-tery pressure might allow better perfusion

of the damaged myocardium from the

pul-monary artery than would occur if the pres-sure in that vessel were low and large inter-coronary communications permitted a

run-off of blood from right to left coronary ar-tery and into the pulmonary artery, thus

bypassing large portions of heart muscle.

(The oxygen content of blood flowing

through the coronary artery is apparently less important as regards myocardial

is-chemia than is the volume of the coronary

perfusate, since patients who are deeply

anoxemic because of cyanotic congenital heart disease do not suffer from coronary

insufficiency.)

The concept that pulmonary

hyperten-sion resulting from mitral insufficiency

con-tributes to perfusion of the heart by way of

the anomalous left coronary artery is

sub-stantiated by a study of McNamara and

Latson.’9 They found that the pressure in the pulmonary artery of a 6-year-old boy

with

severe mitral insufficiency was ele-vated to equal that in the systemic circula-tion. “Pulmonary capillary” pressure curves gave unequivocal evidence of the presence

of mitral insufficiency. Injection of contrast medium into the pulmonary artery showed

the unexpected finding of an anomalous left coronary artery with flow from the pul-monary artery into the coronary artery. Dye

dilution

curves

showed

no

evidence

of

a

left-to-right shunt. The patient died when

the anomalous vessel was ligated near the

pulmonary artery.

In

the

situation

of

anomalous

origin

of

the left coronary artery with or without

ac-companying mitral insufficiency, it is

ap-parent that blood flow in that coronary would be either forward through the left

coronary artery, or retrograde through the right coronary artery via collateral chan-nels into the left coronary artery and pul-monary artery or, as was seen in our second case, bidirectional. Survival of the patient,

as well as surgical treatment, must then be

governed by the interplay of several fac-tors : the direction of flow, the adequacy of

intercoronary anastomoses, and the severity

of the coexistent valvular, myocardial, and

endocardial damage.

The

rationale

for

ligation

of the

left

cor-onary artery is that it acts as an arterio-venous fistula, permitting runoff of oxy-genated blood from the right coronary

artery through collateral channels to the

left coronary artery and pulmonary

cir-culation. Such an operation might be

detrimental to myocardial perfusion when pulmonary artery pressure is elevated and

intercoronary collateral circulation is not rich.14 15, 19

If only

the mitral

insufficiency

were

diag-nosed and surgically corrected and the

anomalous coronary were unrecognized, it could be postulated that with falling

pul-monary artery pressure, myocardial

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656

MITRAL INSUFFICIENCY

might become inadequate so that

addi-tional ischemic changes could result. The

patient reported by Usman et al. may be

such an example. If the correct total diag-nosis of anomalous origin of the left cor-onary artery with mitral insufficiency were

made pre-operatively, it would seem

desir-able to attach the left coronary to the

sys-temic circulation before repairing the

mitral valve. Armer

and

Schumacker2o sug-gested a method of accomplishing this shift. Such an anastomosis has been performed on a child at Mt. Sinai Hospital in New York.2’ Regardless of the type of surgery

considered, the final prognosis will depend on the severity of the myocardial damage that has taken place before attempted

ther-apy.

Even when this condition does function

as an arteriovenous fistula, the effects of inadequate myocardial perfusion are far more extensive than in a simple coronary arteriovenous fistula where still larger

de-grees of aortico-pulmonary shunting may

occur, yet myocardial damage is trivial or

non-existent. The ischemic changes

prob-ably occur during the transitional phase, postulated by Edwards,14 after the pul-monary artery pressure drops below that of

fetal life but before rich intercoronary col-lateral channels develop.

When the anomalous coronary is ligated

near the pulmonary artery, the chief hope

is that the patient will survive the

opera-lion and live long enough to establish ade-quate myocardial perfusion for the

remain-ing unscarred myocardium. With growth he may adjust to the endocardial

fibro-elastosis which is so commonly associated.

Tolerance of the mitral insufficiency would

depend on its severity as well as on the functional capacity of the left ventricle. Considering these factors, prognosis for the patient surviving operation must be

guarded. Like our first patient with

spon-taneous improvement, he might recover

from heart failure in infancy and progress

to an apparently healthy childhood, only

to die suddenly on the playground.

SUMMARY

An infant and a child with anomalous

origin of the left coronary artery from the pulmonary artery have been presented.

Each showed evidence of mitral insuffi-ciency as a consequence of ischemic dam-age to the anterior papillary muscle and sup-porting myocardium. Changes in projected surgical approach necessitated by this com-bination have been discussed. Survival of

the patient and his suitability for surgery are determined not only by the direction

of blood flow in the coronary artery, which

is influenced by the pressure in the

pul-monary artery as well as by adequacy of

collateral circulation, but also by the de-gree of endomyocardial and mitral valvular damage.

REFERENCES

1. Agustsson, M. H., Casul, B. M., Fell, E. H.,

Graetinger, J. S., Bicoff, J. P., and

Water-man, D. F. : Anomalous origin of left

cor-onarv artery from pulnlonary artery.

J.A.M.A., 180:15, 1962.

2. Cumming, C. R., and Ferguson, C. C. :

Anom-alous origin of the left coronary artery from the pulmonary artery functioning as a coronary arteriovenous fistula. Amer. Heart

J., 64:690, 1962.

3. Liebman, J., Hellerstein, H. K., Ankeney, J. L.,

and Tucker, A. : The problem of the

anoma-bus left coronary artery arising from the

pul-monary artery in older children : report of

three cases. New Engi. J. Med., 269:486,

1963.

4. Jameson, A. G., Ellis, K., and Levine, 0. R.:

Anomalous left coronary artery arising from

pulmonary artery. Brit. Heart J., 25:251,

1963.

5. Bookstein, J. J.: Aberrant left coronary

ar-tery. Amer. J. Roentgenol., 91:515, 1964.

6. George, J. M., and Knowlan, D. M. :

Anoma-bus origin of the left coronary artery from

the pulmonary artery in an adult. New Engl.

J. Med., 261:993, 1959.

7. Usman, A., Fernandez, B., Unicchio, J. F., and

Nichols, H. T. : Aberrant origin of left

coronary artery combined with mitral

regur-gitation in an adult. Amer. J. Cardiol., 8:

130, 1961.

8. Burchell, H. B., and Brown, A. L., Jr. :

Anom-alous origin of coronary artery

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9. Brooks, H. St.

J.

: Two cases of an abnormal

coronary artery of the heart arising from

the pulmonary artery with some remarks

up-on the effect of this anomaly in producing cirsoid dilatation of vessels.

J.

Anat. Physiol., 20:26, 1886.

10. Edwards,

J.

E. : Symposium on cardiovascular

disease : functional pathology of congenital

cardiac disease. Pediat. Clin. N. Amer., 1:

13, 1954.

1 1. Edwards,

J.

E. : Anomalous coronary arteries with special reference to arterio-venous-like communications. Circulation, 17: 1001, 1958.

12. Case, R. B., Morrow, A. C., Stainsby, W., and Nestor,

J.

0.: Anomalous origin of the left coronary artery. Circulation, 17: 1062, 1958.

13. Sabiston, D. C., Jr., Neill, C. A., and Taussig,

H. B. : The direction of blood flow in anom-alous left coronary artery arising from the pulmonary artery. Circulation, 22:591, 1960. 14. Edwards, J. E.: The direction of blood flow in

coronary arteries arising from the pul-monary trunk. Circulation, 29: 163, 1964. 15. Nadas, A. S., Gamboa, R., and Hugenholtz,

P. C.: Anomalous left coronary artery orig-inating from the pulmonary artery. Report

of two surgically treated cases with a

pro-posal of hemodynamic and therapeutic clas-sification. Circulation, 29: 167, 1964. 16. Stein, H. L., Hagstrom,

J.

W. C., Halpern, M.,

Ehlers, K. H., and Steinberg, I. : Anomalous

origin of the left coronary artery from the

pulmonary artery: angiographic and

patho-logic study of a case. Amer. J. Roentgenol.,

in press.

17. Bland, E. F., White, P. D., and Garland,

J.:

Congenital anomalies of the coronary

an-tenies; report of an unusual case associated

with cardiac hypertrophy. Amer. Heart

J.,

8:787, 1933.

18. Phillips,

J.

H., Burch, C. E., and De Pasquale,

N. P. : Papillary muscle dysfunction. Ann.

In-tern. Med., 59:508, 1963.

19. McNamara, D., and Latson, J.: Personal

com-munication.

20. Armer, R. M., Shumacker, H. B., Jr., Lunie,

P. R., and Fisch, C. : Origin of the left coronary artery from the pulmonary artery

without collateral circulation. Report of a

case with a suggested surgical correction.

PEDwnucs, 32:588, 1963.

21. Jacobson, J., II, and Steinfeld, L. : In

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1964;34;649

Pediatrics

Harry R. Foster, Jr., Jack W. C. Hagstrom, Kathryn Hawes Ehlers and Mary Allen Engle

CORONARY ARTERY FROM THE PULMONARY ARTERY

MITRAL INSUFFICIENCY DUE TO ANOMALOUS ORIGIN OF THE LEFT

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