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The ndings on physical examination, chest roentgenogram and electrocardiogram
were unchanged from March1968. The administration of long-acting vasodilators
afforded some improvement. The patient died during sleep at home three months
later.
At autopsy (A69–61), the heart weighed 800 gm. All chambers were dilated,
hypertrophied, and the subepicardial adipose tissue was excessive. Focal deposits
of calcium were present in the left and noncoronary cusps of the aortic valve, but
the cusps were freely movable, competent and not stenotic. The other valves were
normal. No foci of myocardial necrosis were present histologically, but focal brous
scars were present in the anterolateral wall and papillary muscles of the left ventricle (Figure3).
The altered anatomy of the coronary arteries is depicted in Figure4. Atiny dimple, approximately 0.1cm in diameter, was the only remnant of the left coronary
arterial orice in the left coronary sinus of the aorta. The right coronary orice was
normal. In the position of the normal left coronary artery a cord, 0.2cm thick and
1.5cm in length and composed of smooth muscle, was found (Figure4). There was
no lumen in the cord but ve minute vessels containing erythrocytes were present.
Distal to the atretic left main coronary artery, the circumex and anterior descending branches arose and pursued normal courses. The circumex branch terminated
at the obtuse margin of the heart by dividing into three marginal branches. The
anterior descending artery traversed the anterior interventricular sulcus terminating at the cardiac apex. The right coronary artery was a large vessel which also
pursued a normal course in the atrioventricular groove, terminating just short of
the obtuse margin of the inferior left ventricular surface. The anterior descending
and circumex branches were supplied by two large collaterals of the right coronary
artery. The rst, the conus branch (Figure4), arose from the proximal right coronary
artery and joined the atretic left main coronary artery, giving rise at this point to
Figure 3 Transverse sections of cardiac ventricles showing multiple brous scars,
severe left ventricular hypertrophy but no left ventricular dilatation.
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Case 122 Congenital atresia of the left main Coronary artery
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Figure 4 The coronary arterial anomaly. a, showing the atretic left main coronary
artery and the large collaterals from the right coronary artery. L.C.=left circumex; A.D.= anterior descending. b, heart from anterior view. c, coronary arteries
and aorta from above. Focal calcic deposits are present in the aortic valve cusps.
C.A.=coronary artery. d, photomicrograph of cross-section of congenitally atretic
left main coronary artery. Hematoxylin and eosin stain, original magnication × 56.
both anterior descending and circumex arteries. At the acute margin of the heart a
marginal branch arose from the right coronary artery and connected with the anterior descending artery at the cardiac apex.
All extramural coronary arteries were rigid, tortuous and calcied (Figures 5
and 6). There were focal luminal narrowings throughout the coronary arterial tree
by calcic and brous plaques. The lumen of the rst portion of the conus artery was
severely narrowed by hemorrhage into an old atherosclerotic plaque.
An attempt was made to determine the cause of the left bundle branch block in
this patient. Alarge block of tissue which included the penetrating and bifurcating
portions of the atrioventricular (A-V) bundle and the proximal right and left bundle
branches was processed, embedded in parafn and serially sectioned at 6 µ intervals. Of the 3,020 sections cut, each tenth was stained and examined histologically
(odd numbered slides were stained by hematoxylin and eosin and even numbered
slides by the elastic Van Gieson method). No anatomic interruption of the left bundle
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Figure 5 Roentgenogram of the heart at autopsy showing calcic deposits in the
coronary arteries and in the aortic valve (A.V.).
branches proximally was found. The A-V bundle and both right and left bundle
branches were intact proximally. The arteries supplying the A-V bundle, however,
showed severe (up to >75 per cent) luminal narrowing by brous tissue proliferation.
COMMENTS
Congenital anomalies of the coronary arteries have been classied by Edwards
those of minor and those of major functional signicance. Included in the former
are anomalies in which the coronary arteries arise from the aorta and in which no
abnormal arteriovenous communication is present. Single coronary artery is an
example of this type. This is a rare anomaly, only seventy-nine such cases having
been reported through 1966.
major congenital cardiovascular abnormalities, with which there is a high association, single coronary artery is compatible with a normal life expectancy.
because major collateral channels develop which connect the single artery with the
vascular bed lacking an aortic orice, or the “missing” coronary artery is merely
displaced and arises as a branch of the single coronary artery.
two situations are similar, but it is likely that they represent different abnormalities
of embryogenesis.
In patients who survive to adulthood, atherosclerosis may complicate single
coronary artery.
5, 6
One would expect that the consequences would be more severe
in this situation. Allen and Snider,
in nine recorded cases of single coronary artery with myocardial infarction was
identical with the average age at death of all patients with myocardial infarction.
In our patient the course of ischemic heart disease was slow. The rst manifestation
124
1
into
2
It has long been appreciated that in the absence of other
3
This is so
4
Functionally, these
5
however, found that the average age at death
5
Case 122 Congenital atresia of the left main Coronary artery
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Figure 6 Photomicrographs of cross-sections of the major extramural coronary
arteries, all taken at the same magnication (× 11.5). a, right coronary artery in rst
1cm. b, congenitally atretic left main coronary artery; c, rst 1cm of the conus artery
showing a large hemorrhage into an old atherosclerotic plaque. The lumen is narrowed by the extravasated blood. d, proximal 1cm of anterior descending vessel. e,
proximal 1cm of left marginal branch. f, proximal 1cm of left circumex branch. All
sections stained with hematoxylin and eosin.
appeared twenty years before death, with the onset of atrial brillation. Angina
pectoris occurred ten years later and remained of mild severity for the ensuing ten
years. The development of cardiomegaly without signs or symptoms of congestive
heart failure and the development of left bundle branch block on electrocardiogram
preceded death by one year. Sudden death in this patient was heralded three months
earlier by a change in the pattern of his previously stable angina pectoris. It is apparent that the branches of the left coronary artery, which were normally positioned,
were adequately supplied by the two large collateral branches of the right coronary
artery. Diffuse coronary atherosclerosis involving his entire coronary arterial tree
was responsible for the manifestations of ischemic disease. The presence of a single coronary arterial orice probably had little effect on his course until the conus
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artery, the main supplier of the left anterior descending and circumex branches,
was nearly occluded by hemorrhage into an old plaque.
A dimple at the site of the “absent” aortic coronary arterial orice has been
described in several recorded cases of single coronary artery,
structure which occupied the position of the left main coronary artery in our case
has been reported only twice previously. Maddox and Isbister
4, 6, 7
but the cordlike
8
briey mentioned a
similar structure replacing the main left coronary artery, but the histologic features
were not described and the presence or absence of an aortic dimple was not noted.
Murphy
2
described a patient similar to ours in whom a dimple was present in the
aortic sinus, and a cord composed of cardiac muscle and brous tissue was the only
remnant of the left main coronary artery. The distal branches were normally positioned. He believed that the structure most likely represented an atretic remnant of
the left main coronary artery. The histologic ndings of the cord in our case resembled those described by Murphy.
9
considered two embryologic mechanisms for the development of a single
Smith
coronary artery: (1) congenital absence of a coronary arterial anlage, and (2) displacement of an anlage so that it fused with the normal vessel which remained. The
ndings in our patient and in the two similar patients previously described suggest
a third mechanism, which was proposed by Roberts and Loube
of a proximal coronary artery. Rarely the atretic segment may remain, as in our
patient, or only an aortic dimple may remain, or perhaps neither may persist.
6
: congenital atresia
10
ACKNOWLEDGMENT
We thank Dr. Charles M. Gillikan and his associates at the Outpatient Clinic of
the U.S. Public Health Service, South Building, Washington, D. C., for making this
patient’s records available to us. Also, the help provided by Dr. Deborah Carpenter
is gratefully acknowledged.
REFERENCES
1. Edwards JE: Anomalous coronary arteries with special reference to arteriove-
nous-like communications. Circulation 17: 1001, 1958.
2. Murphy ML: Single coronary artery. Amer Heart J 74: 557, 1967.
3. Edwards JE: Pathology of the Heart (Gould SE, ed), Springeld, IL, Charles C.
Thomas, 1960, p.426.
4. Blake HA, Manion WC, Mattingly TW, Barotch G: Coronary artery anomalies.
Circulation 30: 927, 1964.
5. Allen GL, Snider TH: Myocardial infarction with a single coronary artery. Arch
Intern Med (Chicago) 117: 261, 1966.
6. Roberts JT, Loube SD: Congenital single coronary artery in man. Amer Heart J 34:
188, 1947.
7. Sanes S: Anomalous origin and course of the left coronary artery in a child. Amer
Heart J 14: 219, 1937.
8. Maddox K, Isbister J: Acase of single coronary artery, quadricuspid pulmonary
valve and anomalous right subclavian artery: death from acute rheumatic carditis. Med J Aust 1: 50, 1940.
9. Smith JC: Review of single coronary artery with report of 2 cases. Circulation 1:
1168, 1950.
10
. Ogden JA: Congenital Variations of the Coronary Arteries. Thesis, Yale University
School of Medicine, 1968, p.48.
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Case 138 aneurysm of the nonPatent duCtus arteriosus
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Case 138 Aneurysm of the Nonpatent
Ductus Arteriosus
M. Wayne Falcone, MD*, Joseph K. Perloff, MD, FACC†
and William C. Roberts, MD, FACC
Bethesda, Maryland
Clinical and necropsy ndings are described in a 6 week old infant with
aneurysm of the ductus arteriosus. It was obliterated at the pulmonary arterial
end and patent at the aortic end. Observations in this case and in 60 previously
described cases of ductal aneurysm disclosed that 46 of the aneurysms occurred
in infants less than 2 months old, 4 in children, and 11 in adults. The aortic end
of the ductal aneurysm is always patent. The pulmonary arterial end may or
may not be patent. Since complications (rupture, embolism or infection) tend
to develop in nearly half of the ductal aneurysms, operative resection appears
indicated.
Persistent patency of the ductus arteriosus is a common congenital cardiovascular
malformation. Aneurysmal dilatation is an occasional complication of patent ductus
arteriosus in adults. Aneurysm of the ductus arteriosus in infancy—in contrast to
simple patency—is rare. However, such was the case in an infant we recently studied. Clinical and necropsy observations in this patient with ductal aneurysm and in
those previously reported by others are described in this communication.
REPORT OF PATIENT
A 52 day old white boy, the rst-born of twins, delivered at a referring hospital,
weighed 2.2 kg after a term gestation. At birth he showed accidity, cyanosis and
regurgitation; Apgar scores were 2 and 8 at 1 and 5 minutes, respectively. Positive
pressure respirator and intubation were required initially with spontaneous onset
of respirations at 5 minutes. He had multiple congenital abnormalities including an
elongated neck, cloudy cornea, low-set and posteriorly rotated ears, complete cleft
palate, long thumbs, fetal hypospadius and undescended testes. No precordial murmur was ever heard. Chest roentgenogram (Figure 1) showed cardiomegaly. Alarge
convex shadow at the left base was initially thought to be thymus.
On day 1, the hematocrit was 33 vol percent; leukocyte and platelet counts and
serum electrolytes, including calcium and phosphorus, were normal. Chromosome
pattern was 46 XY without any structural abnormality.
From the Departments of Medicine and Pediatrics, Georgetown University School of
Medicine, Division of Cardiology, Georgetown University Hospital, Washington, D. C., and
the Section of Pathology, National Heart and Lung Institute, National Institutes of Health,
Bethesda, Md. Manuscript received January8, 1971, accepted March25, 1971.
Address for reprints: William C. Roberts, MD, Section of Pathology, National Heart and
Lung Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, Md. 20014.
* Clinical fellow of the Washington Heart Association, Washington, D. C.
†
Holder of Research Career Program Award HE 14,009 from the U. S. Public Health Service,
Bethesda, Md.
127DOI: 10.1201/9781003409342-17
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Initially the infant was hypotonic, fed poorly and had respiratory difculties.
Barium swallow on day 12 ruled out tracheoesophageal stula. The cardiac silhouette was smaller (Figure 1). The shadow in the angle between the aortic knob and
pulmonary trunk was still interpreted as representing thymus.
The baby continued to lose weight and by day 22 he began to vomit almost all
feedings, necessitating parenteral uids to maintain electrolyte and caloric balance. The next day a mass was palpated in the right upper quadrant of the abdomen, and upper gastrointestinal series demonstrated pyloric stenosis (Figure 1).
On the same day, he was transferred to Georgetown University Hospital, and pyloromyotomy under local anesthesia was performed immediately. Postoperatively,
he had several apneic spells and continued to feed poorly; poor Moro and suck
reexes and generalized hypotonicity persisted. He died without an obvious precipitating cause.
At necropsy, a 1.5 by 2 by 1cm ductal aneurysm containing thrombus was
found (Figure 2). The pulmonary arterial end of the ductus was closed, and
the aortic end was open. The wall of the aneurysm was composed primarily
of smooth muscle and elastic brils; young connective tissue with focal calcic
deposits was present at the junction of intraaneurysmal thrombus and ductal
wall (Figure 3). The right atrium was dilated, and the valve over the fossa ovale
was incompetent. No evidence of rupture, embolism or infection of the aneurysm was present.
Figure 1 Chest roentgenograms. The arrows designate the ductal aneurysm. The
cardiac size decreased from day 1 to day 23. Roentgenogram on the right demonstrates, in addition, pyloric stenosis with complete retention of contrast material 45
minutes after instillation of barium into the esophagus.
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Figure 2 Unopened and opened views of the ductal aneurysm demonstrating
its relation to surrounding structures. D.A.=ductal aneurysm; Inn.=innominate;
L.P.A.=left main pulmonary artery; L.V.=left ventricle; P.T.=pulmonary trunk;
R.V.=right ventricle; T.=trachea.
Figure 3 Photomicrographs of ductal aneurysm (a), and of portion of wall of aorta
(b), ductal aneurysm (c) and left main pulmonary artery (L.P.A.) (d). The sites at which
photomicrographs b, c and d were taken are shown by small blocks in a. The congu-
ration of the elastic brils in the aorta (b) and left main pulmonary artery (d) are
virtually identical. In contrast, fewer elastic brils are present in the wall of the ductal
aneurysm (c), and the brils at this site have various spacial orientations. Movat stain,
× 6 (a); elastic Van Gieson stains, each × 250 (b to d), all reduced by 43percent.
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Table 1: Aneurysm of the ductus arteriosus
I. Patent aortic and pulmonary arterial ends
A. Fusiform* (with or without aneurysmal thrombus)
1. Infected
2. Noninfected
B. Tubular dilatation
C. Dissection
II. Nonpatent pulmonary arterial end
A. Fusiform* (with or without aneurysmal thrombus)
1. Infected
2. Noninfected
III. Postoperative
* Both aortic and pulmonary arterial openings are small in comparison to the diameter of the
aneurysm.
†
Openings into aorta and pulmonary artery are of similar diameter to that of the aneurysm.
‡
After operative ligation of a patent ductus arteriosus.
4,13,19,20,23,26
3,6–8,22,24,25,27,31,39,40
†16,18,28
10,12,21,27
33,34
5,14,27,29,35–37,42,43
‡52,53
DISCUSSION
1
Ductal aneurysm was described originally by Martin
1828. Thore
3
in 1850 reported 8 cases of ductal aneurysm, and Hammerschlag4 in
1925 reviewed the literature and excluded cases of aortic aneurysms involving the
site of ductal insertion. Cruickshank and Marquis
ous literature and added the tenth adult case.
At least 60 cases of ductal aneurysm have been reported.
are summarized in Table 1and Figures4 and 5. Exact anatomic classication is not
possible in 19 cases
3, 8, 9, 11, 14, 15, 17, 27, 29, 32
because of insufcient information regard-
ing the status of the pulmonary arterial end of the ductus. Six cases
in 1827 and by Billard2 in
5
in 1958 summarized the previ-
1–43
The anatomic types
44–49
previously
considered ductal aneurysm were excluded because of inadequate anatomic detail.
Observations in the remaining 42 cases are summarized in Table 2. Ductal aneurysm also may be classied by age— infantile, childhood and adult. Complications
of the aneurysm in the 61 patients grouped by age are summarized in Table 3 and
Figure4.
Infantile ductal aneurysm: Nonpatent ductal aneurysm has been reported in
13 patients, including the infant described herein. The pulmonary arterial end was
closed and the aortic end open in all 13. The pathogenesis of nonpatent ductal aneurysm is uncertain. Normally, the ductus arteriosus closes physiologically shortly
after birth, but anatomic closure requires several days. By intimal proliferation
the ductus becomes obliterated rst at the pulmonary arterial end, then the aortic end.
21, 51
The walls of the ductus undergo involutional changes by a decrease
50
in elastica in comparison with the pulmonary trunk and aorta, and by increase
of brous connective tissue, giving rise to the ligamentum arteriosum. If closure
of the aortic end is delayed, the ductus in effect becomes an aortic diverticulum,
subject to systemic pressure at a time when its walls are undergoing involution.
This circumstance favors dilatation of the patent portion of the ductus with subsequent aneurysm formation. This mechanism is the most attractive explanation
of nonpatent ductal aneurysm in the infants described, all of whom were less than
2 months old. Necrosis of the walls of the ductus was thought to have been etiologically important in several cases
23, 26, 27
but was not present in our patient. In those
cases in which the histologic features were presented, decrease in elastic tissue in
the wall of the aneurysm was observed.
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Table 2: Spontaneous* aneurysm of the ductus arteriosus
Anatomic Classication no. Age Range Complications Assoc
Mal
Rupture Embolism Infection
Patent ductal aneurysm
Fusiform 19 2 days–51 yr 2 5 5 3
Tubular dilatation 3 3 days–27 days 0 1 0 0
Dissection 7 12 hr–1 mo 3 0 0 1
Nonpatent ductal
aneurysm
Fusiform 13 13 days–66 yr 4 2 2 6
Totals 42¶12 hr–66 yr 9 8 7 10
Excludes postoperative patients.
*
†
Extrophie vesicale,8 valvular incompetent patent foramen ovale,31 aortic coarctation.
‡
Hydrocele.
§
Exomphalos,27 double pelvis and ureter,33 atresia of left pulmonary artery, stenosis of right
pulmonary artery and aneurysm of pulmonary trunk,
aorta.
¶
Autopsy in 39.
Assoc Mal=associated congenital malformations
27
36
42
right aortic arch,39 coarctation of
†
‡
§
41
Adult ductal aneurysm: The origin of nonpatent ductal aneurysms in adults is
less certain. Documented persistence into adulthood of an infantile ductal aneurysm
is lacking. However, the anatomic similarities between adult and infantile ductal
aneurysms suggest that the adult ductal aneurysm may represent persistence of the
infantile variety. Other mechanisms have been proposed to explain adult nonpatent
ductal aneurysm including trauma to a persistently patent ductus,
ritis in a patient with subsequent inammatory closure of the pulmonary arterial
33
and delayed spontaneous closure with arrest at the stage of obliteration of the
end,
38–40
infective arte-
pulmonary arterial end.
Figure 4 Diagram of aneurysm of the nonpatent ductus arteriosus and its complications. LPA=left main pulmonary artery; PT=pulmonary trunk; RPA=right
main pulmonary artery.
131
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