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Table 1: Cardiac catheterization data
Site Pressure
(mm. Hg)
Right atrium A5, V2, M2 17.8 7
Coronary sinus ... 10.1 ...
Right ventricle (inow) 91/3 17.1 ...
Pulmonary artery 91/58 22.4 178
Aortic root 91/56 19.7 ...
Descending aorta (via patent
ductus arteriosus)
Brachial artery 93/56 19.6 ...
Left atrium A6, V4, M3 24.8 ...
Pulmonary vein ... 24.6 ...
91/68 22.5 ...
Oxygen Content
(Vol. %)
Krypton
Index (%)
Figure 3 Biplane angiocardiograms with injection into the right ventricle (R.V.).
The aorta is in a relatively rightward and anterior position, originating entirely from
the right ventricle. The enlarged pulmonary trunk is biventricular in origin. The
two semilunar valves are in the same horizontal plane (see Figure 5). The patent
ductus arteriosus (P.D.A.) is readily identied. The coronary arteries are tortuous
and dilated (see Figure 4).
straddled a large ventricular septal defect (2.2cm. in diameter) that was situated
just beneath the pulmonary valve (Figure 5). Alarge patent ductus arteriosus was
present and there was hypoplasia of the transverse aorta (Figure 6). The foramen
ovale was probe-patent but valvular-competent. The right and left coronary arteries and their branches were aneurysmally dilated and tortuous (Figure 6 through
8). Each contained an occasional yellow streak but their lumens were widely patent
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Figure 4 Biplane angiocardiograms with injection into the aortic root. There is
enormous dilatation and tortuosity of both coronary arteries (compare with Figure 7).
In the lateral projection, the rst portion of the aortic root rises vertically and the
plane of its valve is horizontal.
(Figure 8). The right ventricular wall was severely hypertrophied (Figure 5). The
external diameters of several vessels were right coronary artery 0.7cm., ascending
aorta 2.0cm., pulmonary trunk 3.8cm., patent ductus arteriosus 1.5cm., transverse
aorta 1.0cm. and proximal descending aorta 2.5cm.
COMMENTS
The degree of coronary arterial dilatation and tortuosity in our patient was unique
(Figures 4, 6, 7 and 8). In 1955 Aitchison etal.
2
published photographs of the heart of a
thirty-four year old man with complete transposition of the great vessels. Unusually
large, tortuous extramural coronary arteries were clearly visible but no comment
was made about these vessels. In 1966 Bjork
1
reported “a rare and previously undescribed anomaly consisting of marked dilatation and tortuosity of the coronary
arteries ... in one adult and two children with tetralogy of Fallot.” There were no
abnormal communications between the coronary arteries or between them and the
cardiac chambers or other vessels. Bjork called the anomaly “ectasia of the coronary
arteries.” Dr. Owings W. Kincaid and Dr. Jack L. Titus at the Mayo Clinic reviewed
the coronary arteriograms shown in part in Figure4. They commented upon their
own experience in patients with cyanotic congenital heart disease of long duration
together with dilated coronary arteries in the absence of other anomalies of these
3
vessels
dilatation of the coronary arteries secondary to prolonged congenital cardiac cyanosis with gradual progression of the dilatation over a period of years
. They suggested that the ndings in our patient appeared to be extreme
3
. Bjork1 had
also remarked that the ectasia may become more pronounced with increasing age.
The cause of these morphologic changes in the coronary arteries is unknown,
but the following speculation is offered. Nutritional requirements of myocardium
normally demand maximal extraction of oxygen from coronary arterial blood,
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Figure 5 The exterior of the heart is shown in the upper left, and its interior (follow-
ing removal of the anterior one-half) in the upper right. The pulmonary trunk (P.T.) is
markedly dilated. The aorta (Ao.) arises entirely from the right ventricle (R.V.). The
pulmonary trunk and pulmonic valve (P.V.) are so dilated and the ventricular septal
defect (V.S.D.) so situated that this great vessel overrides both ventricles and receives
blood from each of them, but mainly from the left (L.V.). The entrances into the great
arteries are separated from one another by the septal band of the crista supraventricularis. The aortic (A.V.) and pulmonic (P.V.) valves are in the same horizontal plane.
The patent ductus arteriosus (P.D.A.) is large but noncalcied. Both right (R.C.A.)
and left (L.C.A.) coronary arteries are enormously dilated and tortuous, particularly
the right one. The anterior descending (A.D.) branch of the left coronary is indicated.
The right ventricular wall is severely hypertrophied (1.4cm.) and thicker than the
left ventricular wall (1.2cm.). R.A.=right atrial appendage. Lower left, opened right
ventricle (R.V.). Lower right, opened left ventricle (L.V.). The lower photographs show
the location of the ventricular septal defect (V.S.D.). There is continuity between elements of the anterior mitral leaet (A.) and the pulmonic valve (P.V.). P=posterior
mitral leaet.
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Figure 6 Coronary arteries, aorta and pulmonary trunk. Left, right (R.C.A.) and
left (L.C.A.) coronary arteries as they arise from the aorta. The right one particularly
is enormously dilated and tortuous. Two large tortuous arteries arise from it to supply the anterior and lateral walls of the right ventricle. P.A.= pulmonary trunk;
R.P.A.= right pulmonary artery; R. A.=right atrial appendage. Right, note again
the large size of the pulmonary trunk compared to the ascending (Asc.) aorta. The
transverse aorta, particularly that portion between the origins of the left carotid,
(L.C.) and vertebral artery (V.) is narrowed and considerably smaller than the lumen
of the patent ductus arteriosus (P.D.A.). The vertebral artery (V.) arises anomalously
directly from the aorta. The descending (Des.) aorta proximally is much larger than
the transverse aorta as is the patent ductus arteriosus. L.P.A.=left pulmonary artery;
L.A.A.=left atrial appendage.
which is high in oxygen content. When the aortic root saturation is low, as it is in the
presence of a right to left intracardiac shunt, maximal extraction may not provide
the heart with adequate oxygen. Under these circumstances, the decit may be overcome by increased ow through the coronary bed. Alarge ow acting over a protracted period of time may result in dilatation and tortuosity of the coronary arteries
found in some patients with cyanotic congenital heart disease of long-standing.
Right ventricular origin of both great vessels is a malformation in which the pulmonary trunk arises in its normal position but the aorta arises entirely from the right
ventricle.
4–6
Aventricular septal defect provides the left ventricle with its only outlet. The position of the ventricular septal defect may be above the crista supraventricularis just beneath the pulmonic valve but some distance from the aortic valve.
Blood from the left ventricle is directed principally toward the pulmonary trunk,
and from the right ventricle blood is directed toward the aorta so that cyanosis is
invariable. The peak systolic pressures are the same in the right ventricle, aorta and
pulmonary artery. The ventricular septal defect is generally large, and the anterior
mitral leaet usually lacks continuity with both aortic and pulmonic valves.
8
The
5, 7
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Figure 7 The external surface of the heart in situ showing the remarkable dilatation and tortuosity of the coronary arteries (compare with Figure4). Ao.= aorta.
P.T.=pulmonary trunk. L.C.= left circumex branch. A.D.= anterior descending
branch. R.=right coronary artery. R.M.=right marginal branch. R.A.=right atrium.
L.A.=left atrium.
high location of the ventricular septal defect permits direct communication between
the left ventricle and the pulmonary trunk, although the latter vessel originates
wholly from the right ventricle. In some patients in whom the aorta arises entirely
from the right ventricle, the pulmonary trunk straddles the ventricular septal defect
to varying degrees and is therefore biventricular in origin.
7, 8
This condition of right
ventricular origin of the aorta and biventricular origin of the pulmonary trunk is clinically
similar to right ventricular origin of both great vessels with high ventricular septal
defect. The internal architecture of the heart, however, has some features of complete
transposition of the great vessels, especially when the pulmonary trunk arises principally from the left ventricle.
elements of the mitral and pulmonary valves.
8
Under these circumstances there is continuity between
8
It has been argued that varying
degrees of biventricular origin of the pulmonary trunk are part of a continuum, with
right ventricular origin of both great vessels at one end of the spectrum and complete transposition of the great vessels at the other.
the gradations in this continuum should be separately designated or whether they
should merely be considered variations of each other.
ditions, i.e. “right ventricular origin of both great vessels”
lar origin of the aorta with biventricular origin of the pulmonary trunk”
taken to represent the “Taussig-Bing” complex.
9
It is still debated as to whether
9
Each of the two basic con-
4, 7
and “right ventricu-
7, 10–13
The clinical and hemodynamic
8
has been
manifestations are much the same whether the pulmonary trunk originates entirely
from the right ventricle or whether it straddles the ventricular septal defect and rises
partially from the left ventricle, Accordingly, if the eponym “Taussig-Bing”
10
is used
clinically, it should include both of these anatomic variations.
In the patient described in this report, the aorta arose entirely from the right ventricle, and the pulmonary trunk was biventricular in origin arising partially from
the right ventricle but principally from the left (Figures 3 and 5). The peak systolic
pressures were the same in the right ventricle and in both great arteries (Table 1).
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Figure 8 Photomicrographs of sections of right and left coronary arteries from
our patient (upper) and from a thirty-ve year old woman without cardiac disease
(lower) for comparison. Each of the four sections is at the same magnication (× 18),
and each is located 1cm. from the coronary arterial ostia. The coronary arteries in
our patient are severely dilated, particularly the right one (upper left). The coronary
arteries in the lower panel are of normal size for comparison. Lower left, right coronary artery. Lower right, left coronary artery. Elastic van Gieson stain on each.
Pulmonary hypertension had apparently been present from birth since the conguration of elastic bers in the pulmonary trunk was virtually indistinguishable from
that in the ascending aorta
trations were higher in the pulmonary artery than in the ascending aorta (Table 1)
indicating that the left ventricle communicated directly with the pulmonary trunk
which straddled the ventricular septal defect (Figures 3 and 5). The biventricular
origin of the dilated pulmonary trunk and the right ventricular origin of the aorta
were clearly shown in the angiocardiograms (Figure 3) and at necropsy (Figure 5).
14
(Figure 9). The oxygen contents and krypton85 concen-
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Figure 9 Photomicrographs of the pulmonary trunk (upper left), ascending aorta
(upper right) and hilar pulmonary vessels (lower). The transverse sections of the pul-
monary trunk and aorta were taken 1cm. above the superior margins of the semilunar valves, each is taken at the same magnication (× 135), and each is stained
for elastic bers by the van Gieson method. The conguration of the elastic bers
of the pulmonary trunk indicates that pulmonary hypertension had been present
from birth. Normally, after one year of age, the pulmonary trunk contains far less
elastic bers than the ascending aorta. Lower, section of hilar area of one lung show-
ing that the pulmonary arteries are greatly dilated and that there is an insignicant
amount of intimal proliferation. Multiple sections in the lungs themselves disclosed
the muscular pulmonary arteries and arterioles to be dilated but their lumens to be
virtually free of intimal proliferation. The media of some of the muscular arteries
was mildly hypertrophied. Thus, despite pulmonary hypertension for forty years
as evidenced by aortization of the pulmonary trunk, there were only insignicant
changes of the muscular pulmonary arteries and arterioles. Elastic tissue stain, original magnication × 5.
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Since the aorta arose wholly from the right ventricle (Figure 5), the position of its
ascending portion differed from normal
15
. The aortic root was relatively rightward and anterior (Figure 3), the two semilunar valves were at the same body level
(Figures 3 and 5), the root rose rather vertically (Figure 4) and the plane of its valve
was horizontal rather than oblique
Anomalies of the aortic arch are commonly associated with either right ventricular origin of both great arteries
lar pulmonary trunk.
8
In our patient three such anomalies were identied, namely,
15
(Figure 4).
7
or with right ventricular aorta and biventricu-
hypoplasia of the transverse aorta, patent ductus arteriosus and anomalous origin
of the vertebral artery (Figure 6). The patent ductus arteriosus, together with the
abnormal position of the great arteries, set the stage for an unusual form of differential cyanosis in which the toes were relatively pink and the ngers frankly
blue. Recognition of this distribution of cyanosis is clinically useful in suspecting the basic malformation.
16
Oxygenated blood from the left ventricle entered the
pulmonary trunk and owed via the patent ductus arteriosus into the descending
aorta. The toes, therefore, were relatively acyanotic and were clubbed little if at all.
Unoxygenated blood from the right ventricle entered the ascending aorta so the ngers were obviously cyanosed and clubbed. These circulatory pathways are illustrated in Figure5.
It is noteworthy that our patient’s condition improved toward puberty. It may be
postulated that in the early years of life, congestive cardiac failure occurred because
pulmonary blood ow was excessive. As time went on, changes in pulmonary vascular resistance may have favorably regulated the amount of pulmonary blood
ow so that even though adequate, it was not excessive. Under these circumstances
the symptoms of cardiac failure may have been relieved and longevity improved.
Patients with the Taussig-Bing complex have been known to live into the second,
third or even fourth decades,
11, 12
but our patient is the oldest subject with right ventricular origin of the aorta and biventricular origin of the pulmonary trunk thus far
reported.
The brisk bouts of hemoptysis appeared analogous to the hemoptysis occasionally observed in the pulmonary hypertension of Eisenmenger’s complex
patient described by Eisenmenger in 1897 was a thirty-two year old man who died
following a large hemoptysis.
17
Right ventricular aorta with biventricular pulmo-
17
. The
nary trunk differs clinically from Eisenmenger’s complex in two respects. (1) In
Eisenmenger’s complex, cyanosis occurs with normal or diminished pulmonary blood
ow whereas in our patient cyanosis occurred with increased pulmonary blood
11
(Figure 1). (2) In subjects with right ventricular aorta and biventricular pul-
ow
monary trunk, cyanosis is usually present from birth, but in Eisenmenger’s complex
cyanosis generally begins at a later date.
10
REFERENCES
1. BJORK, L. Ectasia of the coronary arteries. Radiology, 87: 33, 1966.
2. AITCHISON, J. D., DUTHIE, R. J. and YOUNG, J. S. Palpable venous pulsations
in a case of transposition of both arterial trunks and complete heart block. Brit.
Heart J., 17: 63, 1955.
3. KINCAID, O. W. and TITUS, J. L. Personal communication.
4. WITHAM, A. C. Double outlet right ventricle. Am. Heart J., 53: 928, 1957.
5. NEUFELD, H. N., LUCAS, R. V., LESTER, R. G., ADAMS, P., JR., ANDERSON,
R. C. and EDWARDS, J. E. Origin of both great vessels from the right venticle
without pulmonary stenosis. Brit. Heart J., 24: 393, 1962.
6. NEUFELD, H. N., DUSHANE, J. W., WOOD, E. H., KIRKLIN, J. W. and
EDWARDS, J. E. Origin of both great vessels from the right ventricle. I. Without
pulmonic stenosis. Circulation, 23: 399, 1961.
89
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7. EDWARDS, J. E., CAREY, L. S., NEUFELD, H. N. and LESTER, R. G. Congenital
Heart Disease, p.306. Philadelphia, W. B. Saunders Co., 1965.
8. ELLIOTT, L. P., ADAMS, P., JR., LEVY, M. J. and EDWARDS, J. E. Right ventricu-
lar aorta and biventricular pulmonary trunk, an uncommon form of transposition. Am. Heart J., 66: 478, 1963.
9. LEV, M., RIMOLDI, H. J. A., ECKNER, F. A. O., MELHUISH, B. P., MENG, L. and
PAUL, M. H. The Taussig-Bing heart. Qualitative and quantitative anatomy. Arch.
Path., 81: 24, 1966.
10. TAUSSIG, H. B. and BING, R. J. Complete transposition of the aorta and a levo-
position of the pulmonary artery. Am. Heart J., 37: 551, 1949.
11. CAMPBELL, M. and HUDSON, R. E. B. A case of Taussig-Bing transposition
with survival for 34years. Guy’s Hosp. Rep., 107: 14, 1958.
12. BEUREN, A. Differential diagnosis of the Taussig-Bing heart from complete
transposition of the great vessels with a posteriorly overriding pulmonary artery.
Circulation, 21: 1071, 1960.
13. MARINI, A. Clinical features in the Taussig-Bing syndrome. Ann. Paediat., 197:
19, 1961.
14. ROBERTS, W. C. Histologic structure of the pulmonary trunk in patients with
primary pulmonary hypertension. Am. Heart J., 65: 230, 1963.
15. CAREY, L. S. and EDWARDS, J. E. Roentgenographic features in cases with ori-
gin of both great vessels from the right ventricle without pulmonic stenosis. Am.
J. Roentgenol., 93: 269, 1965.
16
. TAUSSIG, H. B. Congenital Malformations of the Heart, p.162. Cambridge, Harvard
University Press, 1960.
17. WOOD, P. The Eisenmenger syndrome. Brit. M. J., 2: 701, 1958.
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Case 84 sCimitar syndrome assoCiated with Patent duCtus arteriosus
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Case 84 Scimitar Syndrome Associated with Patent
Ductus Arteriosus, Aortic Coarctation and Irreversible
Pulmonary Hypertension
D. Luke Glancy, Nina S. Braunwald, Kevin P. O’Brien and William C. Roberts
The scimitar syndrome receives its name from the scimitar-like, roentgenographic
appearance of an anomalous vein connecting most or all of the right pulmonary
veins with the inferior vena cava
with other anomalies of the right lung. Since it was rst recognized
congenital malformation has been observed in over 70 patients,
data together with detailed morphologic study at necropsy have been presented in
only one of them
1
. This report describes the clinical, hemodynamic, and necropsy
ndings in a boy with patent ductus arteriosus and preductile aortic coarctation,
a complex of malformations not previously found in association with the scimitar
syndrome.
REPORT OF PATIENT
A seven-year-old boy (C.C. #07-39-01) entered the National Heart Institute for evaluation of cardiomegaly, rst noted at age two months, and a precordial murmur,
discovered at age three years. During infancy he had gained weight slowly, had
frequent respiratory infections, and had developed dyspnea and cyanosis with exertion. At age three years he had been given digoxin, and thereafter his respiratory
infections and symptoms had steadily decreased. Examination (age seven) revealed
a bulging precordium, a prominent left parasternal lift, and a loud pulmonic closure sound. Agrade 3/6 continuous murmur beneath the left clavicle, a grade 2/6
basal systolic ejection murmur, and a grade 2/6 mid-diastolic rumble at the lower
left sternal border were heard. Femoral arterial pulsations were full, but delayed.
The electrocardiogram and chest roentgenograms are shown in Figures1 and 2,
respectively.
At cardiac catheterization (TableI) oxygen step-ups were found high in the inferior vena cava and in the pulmonary artery. Pulmonary blood ow was calculated to
be twice systemic ow, and the pulmonary resistance to be approximately one third
of systemic resistance. A13mm Hg pressure gradient was measured across the junction of an anomalous right pulmonary vein with the inferior vena cava at the level
of the diaphragm. Pressures in the pulmonary artery and descending aorta, entered
from the pulmonary artery, were identical. Cinearteriograms following an aortic
root injection showed narrowing of the aortic isthmus and lling of the pulmonary
artery from the aorta distal to the coarctation. A30mm Hg peak systolic pressure
gradient was measured across the coarctation.
On August 23, 1967, the aortic coarctation was resected; its lumen measured
1.5mm in diameter (Figure5). Apatent ductus arteriosus, which was distal to the
1
. The venous abnormality is usually associated
2
3, 4
in 1836, this
but hemodynamic
The Section of Pathology and the Surgery and Cardiology Branches, National Heart Institute,
National Institutes of Health, Bethesda, Maryland
Received for publication July29, 1968
91DOI: 10.1201/9781003409342-11
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