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CASE REPORTS IN CARDIOLOGY
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Case 673 Sudden Death in Infancy Associated
with Origin of Both Left Main and Right
Coronary Arteries from a Common Ostium
Above the Left Sinus of Valsalva
Charles W. Barth III, MD, Michael Bray,
MD and William C. Roberts, MD
Anomalous origin of the left main coronary artery from the right sinus of Valsalva
(SV) with subsequent coursing of the left main artery between the aorta and pulmonary trunk can cause myocardial ischemia, sometimes resulting in sudden
unexpected death at a young age.
artery (RCA) from the left SV with coursing of the RCA between aorta and pulmonary trunk also may cause nonfatal and fatal myocardial ischemia.
necropsy patients dying suddenly with 3-cuspid aortic valves and anomalous origin
of the RCA from the left SV, 5 were aged 17 to 49years and 1 was a 9-month-old
2–5
infant.
Herein we report an infant who died suddenly with the latter anomaly.
A 4-month-old girl, who previously had been healthy, was found dead in her crib. Anonidentical twin was healthy. At necropsy, both the left main coronary artery and the RCA
arose from a common, shallow, oblong ostium situated in the aortic wall just above an imaginary line separating the left SV from the tubular portion of aorta (Figures 1 and 2). The
ostium was oriented with its longest dimension in a right-to-left direction. The RCA, which
had a round ostium, coursed at an acute angle from the aorta directly to the right between
aorta and pulmonary trunk to reach the right side of the heart. The left main coronary artery,
which had an oval ostium, arose just to the left of the RCA, and it coursed sharply downward
from the ostium to the surface of the heart where it divided into the left circumex and left
anterior descending coronary arteries. The myocardium was normal.
1
Similarly, anomalous origin of the right coronary
2–6
Of 6 reported
The mechanism by which anomalous origin of the RCA from the left SV with
coursing of the RCA between the pulmonary trunk and aorta causes myocardial
ischemia is believed to be similar to that when the left main coronary artery arises
from the right SV and courses between aorta and pulmonary trunk. The acute angle
at which the anomalous RCA arises from the aortic lumen produces a narrowed slitlike orice at the point it traverses the aortic wall, and it probably is further narrowed
by dilatation of the aortic root during exertion, to the extent that impairment of coronary blood ow may result in myocardial ischemia and fatal arrythmia.
described systolic compression of the origin of the anomalous RCA during coronary
angiography in a 25-year-old man with acute myocardial infarction and complete
heart block. The course of the anomalous RCA between the great arteries also may
result in compression of the anomalous artery and myocardial ischemia.
Our infant differs from the 6 previously reported patients who died unexpectedly in that both the left main artery and RCA arose from the aorta just above the
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes
of Health, Bethesda, Maryland 20205, and The Medical Examiner’s Ofce, Washington, D.C.
Manuscript received and accepted July5, 1985.
242 DOI: 10.1201/9781003409342-40
1
Benge et al6
Case 673 sudden deaTh in inFanCy
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Figure 1 Diagram showing the anomaly in the infant described. AV=aortic valve;
L=left aortic valve cusp; LAD=left anterior descending coronary artery; LC=left
circumex artery; LM=left main coronary artery; NC=non-coronary aortic valve
cusp; PV=pulmonary valve; R=right aortic valve cusp or right coronary artery.
Figure 2 Opened left ventricular outow tract, aortic valve and ascending aorta
in the infant described showing the common ostium of the coronary artery which
quickly divides into the left (LCA) and right (RCA) coronary arteries. AML= anterior
mitral leaet; L, R and P=left, right and posterior aortic valve cusps, respectively;
LV=left ventricle.
243
Case rePorTs in Cardiology
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left SV rather than from the aortic wall behind the SV. Although all 6 patients were
described as having “slitlike” ostia as a result of the acute angle of takeoff of the RCA
from the left SV, the shallow common ostium in our child allowed the acute takeoff
of the RCA from the aorta without producing a slit-like orice. Nevertheless, the
RCA could have been compressed between aorta and pulmonary trunk and it could
have dilated during a crying spell.
REFERENCES
1. Cheitlin MD, DeCastro CM, McCallister HA. Sudden death as a complication of
anomalous left coronary origin from the anterior sinus of Valsalva. Anot so minor
congenital anomaly. Circulation 1974;50:780–787.
2. Roberts WC, Siegel RJ, Zipes DP. Origin of the right coronary artery from the
left sinus of Valsalva and its functional consequences. Analysis of 10 necropsy
patients. Am J Cardiol 1982;48:863–868.
3. Liberthson RR, Gang DL, Custer J. Sudden death in an infant with aberrant origin
of the right coronary artery from the left sinus of Valsalva of the aorta: case report
and review of the literature. Pediatr Cardiol 1983;4:45–48.
4. Isner JM, Shen EM, Martin ET, Fortin RV. Sudden unexpected death as a result of
anomalous origin of the right coronary artery from the left sinus of Valsalva. Am J
Med 1984;76:155–158.
5. Topaz O, Edwards JE. Pathologic features of sudden death in children, adoles-
cents, and young adults. Chest 1985;87:476–482.
6. Benge W, Martins JB, Funk DC. Morbidity associated with anomalous origin of
the right coronary artery from the left sinus of Valsalva. Am Heart J 1980;99:96–100.
244
Case 725 aneurysm oF The Pulmonary Trunk unassoCiaTed wiTh shunTing
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Case 725 Aneurysm of the Pulmonary
Trunk Unassociated with Intracardiac or
Great Vessel Left-to-Right Shunting
Deborah J. Barbour, MD and William C. Roberts, MD
In contrast to aneurysm of the aorta, which is fairly common, aneurysmal dilation
of the pulmonary trunk (PT) or of the right or left main pulmonary arteries is rare
except in congenital heart disease with an intracardiac or great artery shunt. During
the past 27years we have studied at necropsy 3 patients with aneurysms (diameter
more than 4cm) of the PT and of 1 or both of its 2 major branches unassociated with
congenital heart disease. This report describes ndings in these 3 patients and summarizes ndings in 3 previously reported necropsy patients with PT aneurysms
unassociated with congenital heart disease.
Certain ndings in our 3 patients are summarized in Table 1. All were women aged
40, 58 and 63years. Patient 1 had hypertrophic cardiomyopathy (Figure 1) and a pulmonary arterial systolic pressure of 54, 51 and 35mm Hg on 3 occasions in the last 2years of
life. Patient 2 had systemic sarcoidosis and scleroderma with interstitial pulmonary brosis
(Figure 2) and a pulmonary arterial systolic pressure of 85mm Hg. In patient 3, the cause
of the PT aneurysm, which contained a large intraaneurysmal thrombus, could not be determined (Figure 3). The PT aneurysms were 5, 7 and 7cm in maximal diameter. Histologic
study of the wall of the aneurysm disclosed it to be abnormal in patients 2 and 3 and normal in patient 1. The numbers of elastic bers in the media in patients 2 and 3 clearly were
decreased compared with normal (Figure 3). Histologic study of 34 sections of lung in these
3 patients disclosed the pulmonary arterial intima and media to be thickened in all, but none
had plexiform lesions (indicative of irreversible pulmonary hypertension).
1–3
Three previously reported patients with PT aneurysms unassociated with shunts
included a 57-year-old man and 45- and 64-year-old women. The man had chronic
lung disease and was an opium smoker; 1 woman was presumed to have primary
pulmonary hypertension with pulmonary plexiform lesions and a pulmonary arterial mean pressure of 66mm Hg; the other woman had no apparent explanation for
the PT aneurysm which, like our patient 3, had an intraaneurysmal thrombus. All
3 had decreased numbers (compared with normal) of elastic bers in the media of
the PT.
Aneurysms of the PT have been reported by other investigators in patients with
primary pulmonary hypertension,
syphilis.
4–8
Since 1959, approximately 8,000 hearts have been examined in our laboratory and only the 3 patients included herein had aneurysms of the PT unassociated with left-to-right shunts. Of our 435 patients studied at necropsy with mitral
stenosis, of 25 with primary pulmonary hypertension, and of 26 with the Marfan
syndrome, none had an aneurysm of the PT.
The mechanism of development of PT aneurysm unassociated with left-to-right
shunting is unclear. Pulmonary hypertension is usually present but it does not have
From the Pathology Branch, National Heart, Lung, Blood Institute, National Institutes of
Health, Bethesda, Maryland 20892. Manuscript received and accepted June18, 1986.
2
mitral stenosis,4 the Marfan syndrome
5–7
and
245DOI: 10.1201/9781003409342-41
246
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Table 1: Clinical ndings in three patients
Case
1 A63–77 40 35/17 35/5 4 130/85 125/12 7 0
2 A86–14 58 85/32 85/9 ... 140/60 ... 7 ++
3 A85–29 63 ... ... ... ... 5 +
EF=elastic bers; LV=left ventricle; m=mean; PA=pulmonary artery; PT=pulmonary trunk; RA=right atrium; RV=right ventricle; SA=systemic artery; s/d=systolic/diastolic.
Necropsy
Number Age (yr)
Pressures (mm Hg)
Case rePorTs in Cardiology
Diameter (cm)
PTAneurysm ↓EF in PTPA (s/d) RV (s/d) RA (m) SA (s/d) LV (s/d)
Case 725 aneurysm oF The Pulmonary Trunk unassoCiaTed wiTh shunTing
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Figure 1 Patient 1 (Table 1). Heart and radiograph. a, posteroanterior chest radio-
gram showing marked dilatation of the pulmonary trunk. b, posteroanterior angiogram with injection of contrast material into the huge pulmonary trunk (PT). c,
lateral view of the same angiogram demonstrating aneurysm. d, opened left ventricular (LV) outow tract showing a mural endocardial plaque in opposition to
the anterior mitral leaet (AMI) and a normal aortic valve. The mural endocardial
plaque is typical of hypertrophic cardiomyopathy. Ao=ascending aorta; RV=right
ventricle.
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Case rePorTs in Cardiology
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Figure 2 Heart and lung. a and b, exterior view of the anterior surface of the heart
at necropsy showing the severely dilated pulmonary trunk (PT). The right ventricular (RV) cavity is much larger than the left ventricular (LV) cavity. c, transverse
section through the ventricles at the level of the papillary muscles showing severe
right ventricular (RV) wall thickening. d, longitudinal section through the lung near
the hilum showing atherosclerotic plaques in the pulmonary arteries and enlarged
perihilar lymph nodes containing noncaseating granulomas typical of sarcoidosis.
Ao=aorta; RA=right atrium.
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Case 725 aneurysm oF The Pulmonary Trunk unassoCiaTed wiTh shunTing
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Figure 3 Patient 3. Heart (a, b and d) and pulmonary trunk (b, c and e). a, ante-
rior view of the heart at necropsy. The right ventricle (RV) is massively enlarged
and forms the cardiac apex. The pulmonary trunk (PT) and right main pulmonary
artery (RMPA) are markedly dilated. b, posterior view of heart and right pulmonary artery (PA) at the level of its bifurcation showing extension of the PT aneurysm
into the right upper and lower lobe main arterial branches, both of which are lled
with organized mural thrombus. c, cross section through the right main pulmonary
artery (RMPA) showing the aneurysmally dilated lumen to be markedly narrowed
by organized thrombus. d, transverse sections through right (RV) and left ventricular (LV) chambers extending from base (top left) to apex (bottom right). The right
ventricular cavity is markedly dilated while the left ventricular cavity is of normal
dimension. No myocardial necrosis or brosis was present. e, photomicrograph of
portion of wall of PT showing severe depletion of elastic bers.
to be severe as seen in our patient 1. The wall of the aneurysm may be abnormal as
demonstrated by loss of elastic bers in the media, but the wall may not be abnormal
as was observed in our patient 1.
REFERENCES
1. Shilkin KB, Low LP, Chen BTM. Dissecting aneurysm of the pulmonary artery. J
Pathol 1969;98:25–29.
2. Luchtrath H. Dissecting aneurysm of the pulmonary artery. Virchows Arch (Pathol
Anat) 1981;391:241–147.
3. Chiu B, Magil A. Idiopathic pulmonary arterial trunk aneurysm presenting as cor
pulmonale: Report of a case. Hum Pathol 1985;16:947–949.
249
Case rePorTs in Cardiology
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4. Deterling RA, Clagett OT. Aneurysm of the pulmonary artery: Review of the lit-
erature and report of a case. Am Heart J 1947;34:471–499.
5. Tung HL, Liebow AA. Marfan’s syndrome. Observations at necropsy: With spe-
cial reference to medionecrosis of the great vessels. Lab Invest 1952;1:382–405.
6. Headley RN, Carpenter HM, Sawyer CG. Unusual features of Marfan’s syndrome
including two postmortem studies. Am J Cardiol 1963;11:259–266.
7. Roberts WC, Honig HS. The spectrum of cardiovascular disease in the Marfan
syndrome: Aclinicomorphologic study of 18 necropsy patients and comparison
to 151 previously reported necropsy patients. Am Heart J 1982;104:115–135.
8. Boyd LJ, McGavack TH. Aneurysm of the pulmonary artery. Areview of the lit-
erature and report of two new cases. Am Heart J 1939;18:562–578.
250
Case 848 reTroaorTiC ePiCardial Course oF The leFT CirCumFlex
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Case 848 Retroaortic Epicardial Course of the
Left Circumflex Coronary Artery and Anteroaortic
Intramyocardial (Ventricular Septum) Course of
the Left Anterior Descending Coronary Artery
An Unusual Coronary Anomaly and a Proposed Classification
Based on the Number of Coronary Ostia in the Aorta
Allen L. Dollar, MD and William C. Roberts, MD
In recent years a number of articles and books have focused on various coronary
anomalies.
retroaortic epicardial course of the left circumex (LC) coronary artery and anteroaortic intramyocardial course of the left anterior descending (LAD) coronary artery.
Herein, we describe another such case and provide a classication for such cases
based on the present and previously published cases.
cardiac dysfunction, died from complications of gastrointestinal bleeding. At necropsy, the
heart weighed 405 g. None of the 4 chambers was dilated. No grossly visible foci of myocardial
brosis or necrosis were present. The 4 cardiac valves were normal. The origins and courses
of the coronary arteries are shown in Figure1.
bined retroaortic epicardial course of the LC and anteroaortic intramyocardial
course of the LAD coronary arteries (Table 1). None of the 6 previously reported
patients had symptoms of cardiac dysfunction or myocardial ischemia. One
patients, however, was found dead in bed and necropsy disclosed severe atherosclerosis of all 3 major coronary arteries and a left ventricular scar. In these 6 patients,
the LC and the LAD arose either from the right coronary artery or from the right
aortic sinus.
ber of coronary ostia in the aorta. Three possible variations of this complex exist
(Figure2). If 2 aortic ostia are present, there are 2 further variations based on whether
the LC or the LAD arises from the right coronary artery (Figure2, IIa and IIb).
1–4
One of the least frequent coronary anomalies is the combination of
A.S., an 87-year-old man, who during life never had evidence of myocardial ischemia or
5–9
There have been at least 6 previously reported necropsy patients
A useful way to classify this combination of anomalies is according to the num-
with com-
9
of the 6
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Bethesda, Maryland 20892. Manuscript received April18, 1989, and accepted June26.
251DOI: 10.1201/9781003409342-42
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