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Case reports In CardIology
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the vein-like structure reported by Schulze and Rodin11 or the intimal brous proliferation observed by Tedeschi and Helpern.
8
Although there may be some focal
degenerative changes of the myocardial bers suggestive of ischemia, there is no
brous scarring nor other evidence of myocardial infarction. Major associated congenital cardiac defects have been recorded twice. The patient reported by Grayzel
and Tennant
5
had tricuspid atresia, two ventricular septal defects, anomalous drainage of the coronary sinus into the left atrium, and origin of the right pulmonary
artery from the ascending aorta. An anatomic tetralogy of Fallot was present in the
patient described by Williams et al.
7
The lungs are usually collapsed and congested.
Mechanism for Heart Failure: The clinical manifestations of double anomalous
coronary arteries appear to be attributable to left ventricular failure. It is now well
established
3, 4, 13
that when only the left coronary artery arises anomalously from
the pulmonary artery, the blood contained in the aberrant vessel is fully saturated
and ows away from the left ventricle in a retrograde fashion toward the pulmonary artery. When both coronary arteries arise from the pulmonary artery, however,
blood in the anomalous vessels is poorly saturated and ows from the pulmonary
artery toward the myocardium.
The lowered oxygen content of the blood in the anomalous coronary arteries is
certainly not the cause of the left ventricular failure. Some patients with congenital
cyanotic heart disease and normally arising coronary arteries have an arterial oxygen saturation below that of venous blood and live for a number of years. The heart
failure appears to be the result of low perfusion pressure in the two anomalous
coronary arteries. At birth, the pressures in the right ventricle and in the pulmonary trunk are at or near normal systemic levels. Within a matter of hours or days
after birth, however, the pressure in the pulmonary artery falls, and consequently,
in patients with both coronary arteries arising from the pulmonary artery, the
coronary arterial perfusion pressure diminishes. Finally, about two weeks following birth, the perfusion pressure apparently is completely inadequate to supply the
nutritional requirements of the left ventricle, and life ends.
Surgical therapy in this condition would necessarily be directed toward increasing the coronary arterial perfusion pressure. Theoretically, this could be done by
either transplanting or connecting the ostia of the coronary arteries with a systemic
artery, or by constricting the pulmonary trunk above the anomalous coronary arterial ostia. Obviously, neither of these procedures has been performed in a patient
with double anomalous coronary arteries. However, each procedure has been carried out in patients with an aberrant left coronary artery arising from the pulmonary artery. Mustard
14
ligated the anomalous left coronary artery in his patient and
anastomosed it to a systemic artery. He demonstrated that this procedure was technically feasible, but his patient died eight hours following operation. An operating
dissecting microscope may be of real use in this procedure. Morrow
3
constricted the
pulmonary trunk in a patient with an aberrant left coronary artery, but ventricular
brillation occurred after completion of the supravalvular pulmonic stenosis, and
the patient died.
Embryology: The embryologic basis for anomalous development of both coronary arteries from the pulmonary artery has recently been thoroughly reviewed
by Schulze and Rodin.
sistence, have received considerable attention. Abrikossoff
11
Briey, two theories, septal deviation and involution-per-
14
suggested that either
the truncus arteriosus is abnormally divided so that the two coronary anlagen
are included with the pulmonary artery (Figure 4), or that a coronary bud arises
anomalously from a part of the truncus destined to become the pulmonary artery.
The involution-persistence theory suggested recently by Hackensellner
15
provides
explanations for several anomalies of the coronary arteries which the former theories failed to do, and it appears more acceptable. He described the occurrence of
32
Case 12 anoMalous orIgIn of Both Coronary arterIes
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Figure 4 Illustration to demonstrate Abrikossoff’s theory of normal and abnormal development of the coronary arteries. Aand B, normal division of the truncus arteriosus by
a septum (dotted line) so that two coronary anlagen are included with the aorta. C
and D, abnormal division of the truncus arteriosus by a deviated septum so that the
two coronary anlagen are included with the pulmonary artery. T= truncus arteriosus; A=aorta; P=pulmonary artery; 1a=left aortic valve cusp; 3a=right aortic
valve cusp; 1p=left pulmonic valve cusp; 3p=right pulmonic valve cusp; 2=anterior pulmonic valve cusp; 4=posterior aortic valve cusp.
an anlage of a coronary artery in each of the six regions of the semilunar valves of
the aorta and pulmonary trunk. Normally, permanent coronary arteries arise from
anlagen in two aortic sinuses, and the anlagen in the other aortic and in all three
pulmonic valvular sinuses either are not formed or are rapidly involuted (Figure 5).
Thus, the combination of persistence of two normally involuted pulmonary coronary buds and involution of two normally persistent aortic coronary buds would
result in anomalous origin of both coronary arteries.
SUMMARY
The clinical and pathologic features of a 7 day old infant in whom both coronary
arteries arose from the pulmonary artery is presented, and information derived
from the 7 previously reported patients with this anomaly is summarized.
Characteristically, these infants have cyanosis and dyspnea at birth or shortly thereafter, cardiac enlargement and no heart murmur, and rapidly progress to heart
failure. All reported patients with this condition have died within two weeks after
birth. None of the reported patients with double anomalous coronary arteries has
been diagnosed ante mortem.
ACKNOWLEDGMENTS
I wish to thank Dr. Benjamin Highman, Chief, Section of Pathology Anatomy,
Laboratory of Experimental Pathology, National Institute of Arthritis and Metabolic
33
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Figure 5 Illustration to demonstrate Hackensellner’s theory of normal and abnormal
development of the coronary arteries. A, normal division of the truncus arteriosus
before formation of coronary anlagen. B, normal involution of all coronary anlagen
(dotted lines) except the two that arise from the right and left aortic valve sinuses.
C, involution of the two normally persisting aortic coronary anlagen (at 3a and 1a),
with persistence of two normally involuted pulmonary coronary anlagen (at 3p and
1p). The result is anomalous origin of both coronary arteries from the pulmonary
artery.
Diseases, for his kindness in referring this case and for allowing me to publish it.
Also, Iam grateful to Dr. Louis B. Thomas, Chief of the Surgical and Postmortem
Service, National Cancer Institute, and to Dr. Ross C. MacCardle, Laboratory of
Pathology, National Cancer Institute, for reviewing the manuscript.
REFERENCES
1. KEITH, J. D. The anomalous origin of the left coronary artery from the pulmo-
nary artery. Brit. Heart J., 21: 149, 1959.
2. BLAND, E. F., WHITE, P. D. and GARLAND, J. Congenital anomalies of coro-
nary arteries. Report of an unusual case associated with cardiac hypertrophy.
Am. Heart J., 8: 787, 1933.
3. CASE, R. B., MORROW, A. G., STAINSBY, W. and NESTOR, J. O. Anomalous
origin of the left coronary artery. The physiologic defect and suggested surgical
treatment. Circulation, 17: 1062, 1958.
4. SABISTON, D. C., JR., NEILL, C. A. and TAUSSIG, H. B. The direction of blood
ow in anomalous left coronary artery arising from the pulmonary artery.
Circulation, 22: 591, 1960.
5. GRAYZEL, D. and TENNANT, R. Congenital atresia of tricuspid orice and
anomalous origin of coronary arteries from pulmonary artery. Am. J. Path., 10:
791, 1934.
34
Case 12 anoMalous orIgIn of Both Coronary arterIes
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6. LIMBOURG, M. Uber den Ursprung der Kranzarterien des Herzens aus der
Arteria pulmonalis. Beitr. Path. Anat., 100: 191, 1937.
7. WILLIAMS, J. W., JOHNSON, W. S. and BOULWARE, J. R. Acase of tetralogy of
Fallot with both coronary arteries arising from the pulmonary artery. J. Florida M.
A., 37: 561, 1951.
8. TEDESCHI, C. G. and HELPERN, M. M. Heterotopic origin of both coronary
arteries from the pulmonary artery. Review of literature and report of a case not
complicated by associated defects. Pediatrics, 14: 53, 1954.
9. SWANN, W. C. and WERTHAMMER, S. Aberrant coronary arteries. Experiences
in diagnosis with report of three cases. Ann. Int. Med., 42: 873, 1955.
10
. ALEXANDER, R. W. and GRIFFITH, G. C. Anomalies of the coronary arteries
and their clinical signicance. Circulation, 14: 800, 1956.
11
. SCHULZE, W. B. and RODIN, A. E. Anomalous origin of both coronary arteries.
Report of a case with discussion of teratogenic theories. Arch. Path., 72: 36, 1961.
12
. TAUSSIG, H. B. Congenital Malformations of the Heart, p. 324. New York, The
Commonwealth Fund, 1947.
13
. EDWARDS, J. E. Symposium on cardiovascular diseases. Functional pathology
of congenital cardiac disease. Pediat. Clin. North America, 1: 13, 1954.
14
. ABRIKOSSOFF, A. Aneurysma des linken Herzventrikels mit abnormer
Abgangsstelle der linken Koronararterie von der Pulmonalis bei einem fünfmonatlichen Kinde. Arch. Path. Anat., 203: 413, 1911.
15
. HACKENSELLNER, H. A. Akzessorsiche Kranzgefässanlagen der Arteria pul-
monalis under 63 menschlichen Embryonenserien mit einer grössten Länge von
12 bis 36mm. Ztschr. Mikroscopischanat. Forsch., 62: 153, 1956.
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Case 15 Survival to Adulthood in a Patient with
Complete Transposition of the Great Vessels*
Including a Note on the Association of
Endocrine Tumors with Heart Disease
William C. Roberts, MD, Dean T. Mason, MD, and Eugene Braunwald, MD
Bethesda, Maryland
THE MAJORITY OF PATIENTS with complete transposition of the great vessels die
in infancy. The subject of this paper is a man who lived for 21years with this malformation, which was associated with several other cardiac defects. ABlalock-Taussig
procedure had been performed at the age of 10years. An adrenal cortical tumor was
discovered at autopsy, and this nding provided the stimulus to review the association of endocrine tumors with heart disease.
REPORT OF PATIENT
A 21-year-old white man, a furniture maker, was admitted to the National Heart
Institute on September 20, 1961. Aheart murmur and cyanosis had been discovered in infancy. Growth and development had been subnormal, and fatigue, dyspnea, and squatting on exertion had been noted frequently. At the age of 10years,
a diagnosis of tetralogy of Fallot was made at another hospital following an angiogram, and a left subclavian-left pulmonary arterial anastomosis was performed.
Following surgery, cardiac symptoms and cyanosis decreased, and the patient was
able to nish high school and subsequently to maintain a good employment record.
In September1960, cyanosis began to increase progressively and in June 1961, the
patient suddenly developed right hemiparesis and was hospitalized. Hematocrit
reading at the time was 70%. It was believed that he had had a cerebral thrombosis,
and he was treated with serial phlebotomies, anticoagulants, and physical therapy.
On admission to the Clinical Center, he was critically ill, and had marked generalized cyanosis and digital clubbing. Blood pressure was 120/80mm Hg, and pulse
rate was 110/min. There were slight bilateral papilledema and retinal venous congestion. The neck veins were at, the lungs, clear, and the extremities and sacrum,
free of edema. There were a prominent right ventricular lift, a loud, single, palpable,
basal second sound, and a coarse grade 4/6 systolic ejection murmur in the third left
intercostal space. The patient had an expressive aphasia, right facial paralysis, right
spastic hemiparesis, and bilateral extensor plantar responses.
Hematocrit reading was 55%; the white blood cell count, platelets, and serum
electrolytes were normal except for a sodium value of 129 mEq/ liter; blood cultures
were sterile; cerebrospinal uid, skull, and sinus roentgenograms were normal. An
Received May11, 1962; accepted for publication May29, 1962.
* From the Pathologic Anatomy Department, Clinical Center, and Cardiology Branch,
National Heart Institute, National Institutes of Health, Bethesda, Maryland.
Requests for reprints should be addressed to William C. Roberts, M.D., Department of
Medicine, The Johns Hopkins Hospital, Baltimore 5, Maryland.
36
DOI: 10.1201/9781003409342-4
Case 15 survIval to adulthood In a patIent wIth CoMplete transposItIon
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electrocardiogram (Figure1) disclosed a vertical upright electrical axis, rst degree
atrioventricular block, and right ventricular hypertrophy. Achest roentgenogram
(Figure2) showed a slightly enlarged heart, diminished pulmonary vascularity, and
a concavity in the region of the pulmonary artery. Aphonocardiogram (Figure3)
revealed a complex rst sound at the base, which contained an atrial sound, an atrioventricular valve closure sound, and an aortic ejection sound. The second sound at
Figure 1 Electrocardiogram.
Figure 2 Anteroposterior roentgenogram.
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Figure 3 Phonocardiogram recorded in the second right intercostal space and
simultaneous indirect carotid pulse tracing. S
tion sound; Sm, systolic murmur; S
, second heart sound.
2
, rst heart sound; SE, systolic ejec-
1
Figure 4 Diagram depicting the multiple cardiac anomalies in this patient. There
is complete transposition of the great vessels, with the aorta arising entirely from
the right ventricle, and the pulmonary trunk exclusively from the left ventricle.
Several defects are present in the atrial septum, and one large defect is present in
the basal portion of the ventricular septum. The pulmonic valve is stenotic, and
the pulmonary trunk, relatively hypoplastic. All cardiac chambers are dilated. The
patent, although narrowed, left subclavian-pulmonary arterial anastomosis and the
bronchial arterial collateral circulation are not shown in this diagram.
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Case 15 survIval to adulthood In a patIent wIth CoMplete transposItIon
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Figure 5 Anterior view of the heart (550 g). The ascending aorta (Ao) arises to the
right of, and anterior to, the pulmonary trunk (P.T.). The left subclavian (L.S.)—left
pulmonary arterial anastomosis is intact. Both atrial appendages lay to the left of the
pulmonary trunk and adjacent to one another. The right appendage (R.A.A.) is the
larger one and lies to the right of the left atrial appendage (L.A.A.). Both ventricles
(R.V. and L.V.) are dilated and hypertrophied.
the base was single in all phases of respiration, and appeared to be produced by
aortic valve closure. Along systolic murmur which reached aortic valve closure was
recorded at the base, and a continuous murmur was recorded over the left sternal
border.
The life-threatening neurological complications prevented denitive evaluation
of the patient’s cyanotic congenital heart disease. His condition progressively deteriorated, and on the ninth hospital day he had a generalized seizure and became
semicomatose. During the next 3 days additional seizures occurred, and, despite
large doses of anticonvulsants, he became comatose and died.
PATHOLOGIC FINDINGS
The pathologico-anatomic features of the heart and great vessels are described in
Figures4–8. There was complete transposition of the great vessels; also, there were
atrial and ventricular septal defects, valvular pulmonic stenosis, juxtaposition of the
atrial appendages, and collateral bronchial arterial circulation. Vascular thrombi
were widespread. There were both old and recent thrombi occluding the superior sagittal sinus, the left middle cerebral and mesenteric arteries, and right iliac,
femoral, and splenic veins. Multiple infarcts were present in the liver, spleen, and
left lung. The lungs and viscera were acutely and chronically congested. The right
adrenal gland was normal, but the left one contained a large, well-circumscribed
39
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Figure 6 Photographs showing the interior of the cardiac chambers. A. The right
atrium and right ventricle (R.V.) are opened. Defects (A.S.D.) are present in both the
mid and lowermost portions of the atrial septum. The opening into the right atrial
appendage (R.A.A.) is located immediately anterior to the largest atrial septal defect.
The ventricular septal defect (V.S.D.) is located behind the junction of the anterior
and septal tricuspid valve leaets. The superior vena cava (S.V.C.) is designated. B.
The left atrium (L.A.) and left ventricle (L.V.) are opened. The multiple defects in
the atrial septum are apparent. The mitral valve is normal. Many recent ante-mortem thrombi are interspersed among the trabeculae carneae muscles in the apex of
the left ventricle. C. The ascending aorta (Ao.) and right ventricle (R.V.) are opened.
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Figure 6 (Continued) Probes are placed in each of the ostia of the coronary arteries. Note that there is no connection between the tricuspid and aortic valves. The ventricular septal defect (D), which measures 2.5 × 3cm in size, is located immediately
below the aortic valve cusps and is bordered anteriorly, posteriorly, and caudally by
ventricular myocardium. The right ventricular wall is greatly hypertrophied. D. The
pulmonary trunk (P.T.), pulmonic valve (P.V.), and left ventricle (L.V.) are opened. The
stenotic, dome-shaped pulmonic valve is continuous with the anterior mitral leaet
(A.M.L.). The pulmonary trunk (P.T.) is small and the ascending aorta, dilated. There
is a rim of myocardium between the pulmonic valve and the cephalic margin of
the ventricular septal defect (D). Both atrial appendages are visible to the left of the
pulmonary trunk.
Figure 7 Photographs demonstrating the pulmonic valve before it was opened. A.
The domeshaped pulmonic valve, which is brotic and partially calcied, as viewed
from above. B. The stenotic pulmonic valve (P.V.) as seen from the left ventricular
(L.V.) aspect. The ventricular septal defect (V.S.D.) and the anterior mitral leaet
(A.M.L.) are apparent.
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