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Case reports In CardIology
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had complete transposition of the great vessels with associated pulmonic stenosis
and would, therefore, benet from a subclavian-pulmonary artery anastomosis.
At operation the pulmonary artery, which was approximately the same size as
the aorta, was observed to arise from the left ventricle and in a position directly
posterior to the aorta. Pressures measured simultaneously in the pulmonary artery
and aorta were equal. Alarge number of dilated bronchial arteries and veins were
present in the hili of the lungs, in the interlobar ssures and along the subpleural
surface of the lungs. Because of the high pressure in the pulmonary artery, an anastomosis was not performed. Postoperatively, the patient became progressively more
cyanotic, and his condition gradually deteriorated until he died eighteen hours after
operation.
PATHOLOGIC FINDINGS
Necropsy conrmed the operative nding of complete transposition of the great vessels. The aorta arose exclusively from the right ventricle (Figure 3) and the pulmonary trunk from the left ventricle. The ascending aorta lay directly anterior to the
pulmonary trunk. Both ventricles were hypertrophied; the left measured 0.1cm.
and the right, 0.9cm. in greatest thickness. Aventricular septal defect was present
immediately inferior to the supraventricularis muscle (Figure 3). Both atria were
dilated, the right more than the left. The atrial appendages were normally situated.
Avalvular-competent foramen ovale was present. The venae cavae entered the right
atrium, and the pulmonary veins entered the left atrium in a normal manner. The
atrioventricular valves were normally developed. The pulmonic valve lay posteriorly
and joined the anterior mitral leaet in a manner similar to the connections between
the mitral and aortic valves in a normal heart. The cusps of the pulmonic and aortic valves were delicate and pliable and there was no evidence of stenosis. Three
large bronchial arteries arose directly from the descending thoracic aorta (Figure 4)
and coursed along the posterior aspect of the lower trachea and main bronchi before
entering the hili of the lungs (Figure 5). No atherosclerotic changes were present in
the pulmonary artery or aorta. The ostia of the coronary arteries arose from the two
posterior sinuses of Valsalva (Figure 3).
Microscopically, in the sections from the hili of the lungs many dilated, thickwalled bronchial arteries surrounding the bronchi were seen (Figure 6A). Also,
there was proliferation of the intima of some bronchial arteries (Figure 6B), a nding
which has not received attention in the past. Many dilated bronchial veins were seen
Figure 2 Selective right ventricular angiocardiogram demonstrating complete
transposition of the great vessels and large bronchial arteries arising from the
descending thoracic aorta. A, posteroanterior view: B. lateral view.
22
Case 3 InCreased BronChIal Collateral CIrCulatIon
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Figure 3 The right ventricle, aortic valve and aorta are opened. The aorta lies
anteriorly and arises exclusively from the right ventricle. The coronary arteries arise
from the aorta (narrow white pointers). Aventricular septal defect (thick white
arrow) is located immediately inferior to the supraventricular muscle. Note the large
size of the coronary arteries.
Figure 4 Descending thoracic aorta demonstrating the markedly dilated ostia
(arrows) of the bronchial arteries.
in the subpleural areas of the lungs, particularly the hilar regions. Sections from the
main pulmonary arteries showed the elastic bers arranged in an orderly fashion
similar to those found in a fetal pulmonary artery (Figure 7). This picture is noted
in the lungs of patients in whom pulmonary hypertension has been present from
birth and in whom the normal transition to the adult type of pulmonary artery has
not taken place.
4
The elastic and muscular pulmonary arteries were dilated, and the
latter showed medial hypertrophy and occasional intimal thickening. The lumina of
the pulmonary arterioles were narrowed by intimal proliferation (Figure 8).
23
Case reports In CardIology
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Figure 5 Diagram (traced from the original photograph of this area) illustrating
the large bronchial arteries coursing along the posterior aspect of the lower trachea
and main bronchi before entering the hili of the lungs.
Figure 6 A, photomicrograph of lung in hilar region demonstrating thick-walled,
dilated bronchial arteries (black arrows) surrounding a bronchus. There is intimal
thickening in addition to medial hypertrophy of the bronchial artery in the uppermost portion of the gure. Alarge elastic pulmonary artery (white arrow) of the
fetal type is present in the left upper corner of the gure. (Elastic van Giesson stain,
original magnication × 17.) B, close-up view of the large bronchial artery appearing
in A. Note the marked intimal proliferation and medial hypertrophy. (Elastic van
Giesson stain, original magnication × 120.)
COMMENTS
It was demonstrated early in experiments on dogs by Mathes and associates
bronchial collateral circulation to the lungs will develop following obstruction of
the pulmonary artery. Liebow et al.
lateral vessels from the bronchial artery to the pulmonary artery to the pulmonary
capillaries, with drainage directly into the pulmonary veins or into the azygos system by way of the bronchial veins.
24
5
that
6
have traced the pathway of development of col-
Case 3 InCreased BronChIal Collateral CIrCulatIon
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Figure 7 Photomicrograph of main pulmonary artery demonstrating the aortalike fetal conguration of the elastic lamellae. (Elastic van Giesson stain, original
magnication × 450.)
Figure 8 Photomicrograph of pulmonary artery demonstrating marked intimal
proliferation and narrowing at the junction of an arteriolar branch. (Elastic van
Giesson stain, original magnication × 155.)
There are few documented reports in the literature of augmentation of the
bronchial circulation in association with transposition of the great vessels. The
combination of complete transposition of the great vessels with pulmonary hypertension and enlarged bronchial arteries in particular, has not been well described
previously. The case of transposition of the great vessels which Cockle
in 1863 had enlarged bronchial vessels and probably a normal or increased pulmonary artery pressure, since there was no stenosis of the pulmonic valve and the
pulmonary artery was larger than the aorta. Also, no obstruction of the pulmonary
7
described
25
Case reports In CardIology
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valve was noted in pathologic ndings of the patient with complete transposition in
whom Cudkowicz et al.
8
demonstrated extensive bronchial collateral circulation by
injection technics. The pulmonary arteries were dilated and communicated freely
with the vasa vasorum supplied by the bronchial arteries.
Bronchopulmonary arterial communications may be found in patients with
single ventricle and pulmonary hypertension as in the case reported by Heath,
9
in
which bronchial collaterals were prominent in the adventitia of large arteries, surrounding the bronchi and along the visceral pleura. In a similar case illustrated by
Liebow,
10
proliferated bronchial arteries were demonstrated to communicate with
tortuous pulmonary vessels. In the lungs of patients with Eisenmenger’s complex,
collateral channels in the form of angiomatoid lesions may connect the pulmonary
and bronchial circulations.
patent ductus arteriosus with pulmonary hypertension
septal defect and pulmonary hypertension
11
Similar lesions have been reported in patients with
14
; these vessels may divert blood to the
12, 13
and in those with atrial
alveolar capillaries.
It appears, therefore, that there is a wide spectrum of malformations in which
collateral channels to the lungs may develop. The presence of reduced pulmonary
blood ow or the absence of elevated pulmonary vascular resistance should not be
suspected in a patient with cyanotic congenital heart disease merely because of the
existence of enlarged bronchial vessels. Catheterization of the pulmonary artery
is difcult or impossible in patients with transposition of the great vessels, single
ventricle, truncus arteriosus and other complicated forms of cyanotic heart disease;
therefore, the use of right- and left-sided angiocardiography would be valuable in
assessing the size of the pulmonary artery, the relative amount of pulmonary blood
ow and in estimating the pulmonary artery pressure.
In the patient reported herein a right ventricular angiocardiogram was performed, but because of the presence of complete transposition of the great vessels
only the aorta was opacied; the pulmonary artery could not be visualized. As this
case illustrates, the clinical picture associated with cyanotic heart disease and pulmonary hypertension may be confused with that associated with obstruction to pulmonary ow secondary to obstruction in the right ventricular outow tract or at the
pulmonary valve. The reduced pulmonary vascularity on the roentgenogram in this
patient was suggestive of a reduced pulmonary artery pressure. The closely split
second heart sound heard at the base resulted from the presence of equal pressures
in the great vessels rather than from an obstruction to right ventricular outow. The
very soft systolic murmur probably indicated that only a small amount of blood
passed across the ventricular septal defect, due to the equal ventricular pressures.
Accordingly, it is essential that a thorough angiographic and hemodynamic evaluation of the pulmonary vascular bed precede any operative procedure designed to
increase pulmonary blood ow in patients with transposition of the great vessels.
SUMMARY
The clinical, diagnostic and pathologic ndings in a fourteen month old infant with
complete transposition of the great vessels, ventricular septal defect and pulmonary hypertension with increased bronchial collateral circulation are presented. It
is emphasized that the presence of increased bronchial collateral blood ow is not
necessarily associated with decreased pulmonary blood ow due to obstruction to
right ventricular outow, but may also be associated with pulmonary hypertension
and elevation of pulmonary vascular resistance. Thus, the presence of enlarged
bronchial vessels should not indicate that the patient will benet from an operation designed to increase the pulmonary blood ow. The importance of right and
left ventricular angiocardiograms in the preoperative assessment of the pulmonary
vascular bed is discussed.
26
Case 3 InCreased BronChIal Collateral CIrCulatIon
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REFERENCES
1. RICH, A. R. Ahitherto unrecognized tendency to the development of widespread
vascular obstruction in patients with congenital pulmonary stenosis (tetralogy of
Fallot). Bull. Johns Hopkins Hosp., 82: 389, 1958.
2. TOBIN, C. E. The bronchial arteries and their connections with other vessels in
the human lung. Surg. Gynec.& Obst., 95: 741, 1957.
3. TABAKIN, B. S., HANSON, J. S., ADHIKARI, P. K. and MILLER, D. B. Physiologic
studies in congenital absence of the left main pulmonary artery. Circulation, 22:
1107, 1960.
4. HEATH, D., DUSHANE, J. W., WOOD, E. H. and EDWARDS, J. E. The structure
of the pulmonary trunk at different ages and in cases of pulmonary hypertension
and pulmonary stenosis. J. Path. Bact., 77: 443, 1959.
5. MATHES, M. E., HOLMAN, E. and REICHERT, F. L. Astudy of the bronchial,
pulmonary, and lymphatic circulations of the lung under various pathologic conditions experimentally produced. J. Thoracic Surg., 1: 339, 1932.
6. LIEBOW, A. A., HALES, M. R., HARRISON, W., BLOOMER, W. E. and LINDSKOG,
G. F. The genesis and functional implications of collateral circulation to the lungs.
Yale J. Biol.& Med., 22: 637, 1950.
7. COCKLE, J. Case of transposition of the great vessels of the heart. Tr. Med.-Chir.
Soc. London, 46: 193, 1863.
8. CUDKOWICZ, L. and ARMSTRONG, J. B. Injection of the bronchial circulation
in a case of transposition. Brit. Heart J., 14: 374, 1952.
9. HEATH, D. Cor triloculare biatriatum. Circulation, 15: 701, 1957.
10
. LIEBOW, A. A. Pathology of the Heart, Chap. 15. Edited by GOULD, S. E.
Springeld, IL, Charles C. Thomas, 1960.
11. BREWER, D. B. and HEATH, D. Pulmonary vascular changes in Eisenmenger’s
complex. J. Path. Bact., 77: 141, 1959.
12. BREWER, D. B. Fibrous occlusion and anastomosis of the pulmonary vessels in a
case of pulmonary hypertension associated with patent ductus arteriosus. J. Path.
Bact., 70: 299, 1955.
13. DAMMANN, J. F., JR., BERTHRONG, M. and BING, R. J. Reverse ductus: a pre-
sentation of the syndrome of patency of the ductus arteriosus with pulmonary
hypertension and a shunting of blood ow from pulmonary artery to aorta. Bull.
Johns Hopkins Hosp., 92: 128, 1953.
14. ROSSALL, R. E. and THOMPSON, H. Formation of new vascular channels in the
lungs of a patient with secondary pulmonary hypertension. J. Path. Bact., 76: 593,
1958.
27
CASE REPORTS IN CARDIOLOGY
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Case 12 Anomalous Origin of Both Coronary
Arteries from the Pulmonary Artery*
William C. Roberts, MD
†
Bethesda, Maryland
ANOMALOUS origin of either one or both coronary arteries from the pulmonary artery is rare. Less than 70 cases involving the left coronary artery have been
reported. About 15 per cent of the patients lived to adulthood and had no recognizable clinical abnormalities;
graphic features characteristic of this condition (Bland-White-Garland syndrome
and died in infancy. Recognition of this anomaly is important because surgical
treatment appears benecial.
1
the other 85 per cent had clinical and electrocardio-
3, 4
Anomalous origin of the right coronary is even
2
less common, about 20 patients having been reported. These patients characteristically have no clinical symptoms referable to the anomaly, which is usually only an
incidental nding at autopsy. Origin of both coronary arteries from the pulmonary
artery is exceedingly rare, only seven patients having previously been reported.
5–11
This report describes another patient who died of this anomaly.
CASE REPORT
N. M., a male Indian born of a normal pregnancy and delivery, died on his seventh
day of life in the Turtle Mountain Indian Hospital, Belcourt, North Dakota. He had
cyanosis and labored respirations at birth, and these symptoms progressively worsened until his death. No heart murmur was ever heard. The heart was normal in size
by chest roentgenogram. He was treated with oxygen and digitalis without benet.
Autopsy was performed at the Indian Hospital, and subsequently the heart and
other tissues were submitted to the National Institutes of Health for examination.
The heart (Figure 1) weighed 27 gm., and the right atrium, right ventricle and left
ventricle were dilated and hypertrophied. The wall of each ventricle measured up
to 0.4cm. in thickness. The endocardium of the outow tract of the left ventricle was
mildly but uniformly opaque. The four cardiac valves were normal. The foramen
ovale was closed. Both coronary arteries originated from the pulmonary artery. The
ostium of the right coronary artery was located in the right posterior sinus, and
the left one in the left posterior pulmonic sinus. No coronary arteries arose from
the aortic valve sinuses (Figure 2); the course of distribution of the coronary arteries,
which were of equal size, from there on was normal. The lungs were dark purple,
virtually solid and without crepitation.
Histologically, the coronary arteries had the structure of arterial channels
(Figure3A), although they carried only venous blood. Sections through both ventricles (Figure 3B) disclosed minimal interstitial myocardial edema but no myocardial
brosis nor inammation. Myocardial bers were fragmented, and cross-striations
(phosphotungstic acid-hematoxylin stain) were poorly preserved. Some of the myocardial nuclei appeared enlarged, but most were normal. There was no periodic
)
* From the Pathologic Anatomy Department, Clinical Center, National Institutes of Health,
Bethesda, Maryland.
†
Present address: Department of Medicine, The Johns Hopkins Hospital, Baltimore 5,
Maryland.
28 DOI: 10.1201/9781003409342-3
Case 12 anoMalous orIgIn of Both Coronary arterIes
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Figure 1 Drawing of heart. Left, the right ventricle, pulmonary valve and pulmo-
nary trunk are opened. Both coronary arteries arise from the base of the main pulmonary artery. Right, the left ventricle, aortic valve and ascending aorta are opened.
No coronary arterial ostia are present.
Figure 2 Sketch of heart showing both coronary arteries arising from the pulmonic valve sinuses. The left coronary artery arises from the left posterior sinus and
is composed of a circumex branch which courses anterior to the aortic valve to lie in
the left atrioventricular sulcus, and an anterior descending branch which descends
in the anterior interventricular sulcus. The right main coronary artery arises in the
right posterior pulmonic sinus and lies in the right atrioventricular sulcus, giving
off several small branches, and at the base of the posterior interventricular sulcus
becomes the posterior descending coronary artery.
29
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Figure 3 Histologic sections. A, photomicrograph of the anterior descending branch of
the left coronary artery 1.5cm. from its ostium. This vessel has the histologic features
of a muscular artery. Verhoeff-Van Gieson elastic tissue stain, original magnication, ×155. B, photomicrograph of left ventricle. The epicardial surface is at the top of
the gure. Hematoxylin and eosin stain, original magnication, ×30. C, fat stain of
left ventricular myocardium. Cross striations are apparent. Oil red O stain on frozen
section, original magnication, ×480.
acid-Schiff-positive material in the myocardium. Fat stain (Oil red O) on the frozensectioned myocardium was negative (Figure 3C). Cross-striations in the myocardial
bers were easily discernible in the frozen section. Section of the main pulmonary
artery stained for elastic bers (Verhoeff-Van Giesen method) disclosed a fetal, i.e.,
aortic-like, conguration of the elastica of this vessel. In the sections from the lungs
there were extensive atelectasis and congestion.
COMMENT
Clinical Features: In contrast to the diagnosis of anomalous origin of the left coronary
artery from the pulmonary artery, the antemortem diagnosis of anomalous origin
of both coronary arteries from the pulmonary artery has not been reported. The
probable reason is that this latter condition has not been considered clinically, and
thus remains only a postmortem curiosity. However, the clinical features in both
conditions are similar (Table 1) and sufciently characteristic so that the diagnosis
might be suggested before death. The newborn is cyanotic and dyspneic from birth
or shortly thereafter. On the other hand, patients with only the left coronary artery
originating from the pulmonary artery are normal at birth and usually remain so
for one to three months when evidence of heart failure, irritability or discomfort,
and respiratory infection appears. The heart in children with two anomalous coronary arteries is usually enlarged (as it is in those with anomalous left coronary
artery), sometimes reaching huge proportions, as in the patient reported by Swann
and Werthammer.
9
No precordial murmurs are heard unless there is an associated
defect of the heart or great vessels. Frank signs of heart failure are usually apparent,
and these progressively worsen. Feedings are poorly tolerated. The electrocardiogram is virtually pathognomonic in the symptomatic infant with anomalous left
coronary artery,
2, 12
but no tracing has been made in reported cases of anomalous origin of both coronary arteries from the pulmonary artery. Findings of left ventricular
ischemia would be expected. Breathing gradually becomes more labored, and life
ends before two weeks have elapsed.
Pathologic Features: At autopsy, the ventricles, particularly the left one, are typically dilated and hypertrophied, and the endocardium of the left ventricle may be
thickened. The coronary arteries, which arise from the right and left sinuses of the
pulmonic valve (the anterior sinus being the noncoronary one), are normally distributed. Histologically, they may have the same morphologic features herein described;
30
Table 1: Anomalous origin of both coronary arteries from the pulmonary artery
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Author Year Sex Age at
1. Grayzel&
Tennant5
1934
2. Limbourg6
1937
3. Williams,
Johnson,
Boulware
1951
5. Tedeschi&
Helpern3 1954
4. Swann&
Werthammer9
(Case 3) 1955
6. Alexander&
Grifth10
1956
7. Schulze&
Rodin11 1961
8. Present
Author 1961
* Average normal heart weight for full-term newborn=17 gms.
VSD=ventricular septal defect; PV=pulmonic valve; RV=right ventricle; CS=coronary sinus; LA=left atrium; RA=right atrium; PDA=patent ductus arteriosus; PS=pulmonic stenosis; PFO=patent foramen ovale;
PA=pulmonary artery; As.Ao.=ascending aorta.
F 9hours Cyanosis 19
M 10 days Cyanosis
F 4 days “Normal”
7
F 13 days Cyanosis Dyspnea 39 PDA Normal Thickening of walls
M 2 days Cyanosis Dyspnea 56 PDA, PFO Hypertrophy of
M 2 days Cyanosis PDA, PFO Atelectasis, lungs
F 8hours Cyanosis 18 PDA Normal Vein-like Nuclear pyknosis, loss of
M 7 days Cyanosis Dyspnea 27 0 Normal Arterial Normal Atelectasis, lungs.
Death
Condition at
Birth
Unconsciousness
Cyanosis on 2nd
day, Systolic
murmur
Weight of
Heart
(gm.)
*
15 PDA Normal Fat droplets, focal, in
36 VSD, PS, PDA, PFO Normal Atelectasis, lungs
Associated
Cardiovascular
Anomalies
Atresia, tricuspid valve;
VSD (2). Origin rt. PA
from As.Ao; CS → LA&
RA; PDA; PFO;
hypoplasia, RV, PV, PA
Distribution of
Coronary
Arteries
Normal Atelectasis, lungs.
Histologic
Structure of
Coronary Arteries
due to intimal
brous
proliferation& focal
edema of media&
adventitia
Histologic Structure
of Myocardium
myocardial bers
Norma 0
myocardial
bers of both RV& LV.
Fat positive vacuoles
in myocardial bers
cross-striations, &
interstitial edema with
inammatory cell
inltration
Other Findings
Hemorrhage,
cerebellum
Hemorrhage,
subarachnoid,
from tentorial tear
Congestive heart
failure
Congestive heart
failure.
Hyaline membrane
disease, lungs
Congestive heart
failure
Case 12 anoMalous orIgIn of Both Coronary arterIes
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