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Figure 1 Electrocardiograms recorded when the patient was 8years old (above),
and nine days before death when he was 21years old (below). The upright P wave in
lead I (above) indicates that the malrotation of the heart is dextroversion rather than
dextrocardia. The atrial brillation (below) began 18 months before he died.
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Case 51 CoMplex CongenItal CardIaC MalforMatIon
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Figure 2 Chest roentgenogram. The cardiac apex points to the right.
roentgenogram showed an enlarged and dextrorotated heart. At age 20 months
signs of cardiac failure appeared; he was digitalized and remained on digitalis the
rest of his life. He was followed closely in the Harriet Lane Home Cardiac Clinic
and over the years the heart murmur separated into a harsh, loud, pansystolic component best heard at the right sternal border, but widely transmitted, and a softer
diastolic component heard at the apex. The cardiac apex moved laterally to the right
anterior axillary line, and a systolic thrill was palpated widely over the active right
anterior chest. Slight cyanosis and clubbing were occasionally described. At cardiac
catheterization at age 8 (1953) the pressure in the “right ventricle” was 92/6, and in
the femoral artery, 102/64mm. Hg. The pulmonary trunk was not entered. The systemic arterial oxygen saturation was 90 per cent. The cardiac output was 3.4 L./min.
Except for slight dyspnea and cyanosis on strenuous exertion, he was asymptomatic until about one and a half years before death. He completed high school and
one year of college, played and marched with his high school band and worked in
the summers. In August, 1964 he noted a rapid pulse and decreased exercise tolerance and was found to have atrial brillation. This arrhythmia persisted until his
death despite conversion attempts with quinidine. Paroxysms of rapid tachycardia
occurred during his last year.
He was hospitalized on February 7, 1966, because of increasing fatigability, exertional dyspnea, reduced exercise tolerance, insomnia and nervousness. The liver was
enlarged, the pretibial regions edematous, the digits moderately clubbed and the nail
beds quite cyanotic. The hematocrit was 63 per cent; the electrocardiogram (Figure 1)
showed atrial brillation, and ventricular hypertrophy, and the chest roentgenogram
(Figure 2) showed marked cardiomegaly, dextrocardia, enlarged pulmonary vessels,
and a narrowed “vascular pedicle.” The hematocrit was lowered to 59 per cent by the
removal of 500 ml. of blood. On February15, he developed severe pain in his right
eye, and three hours later ventricular brillation appeared and he died.
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Figure 3 Diagram of the heart. The right (R.A.) and left atria (L.A.), vena cavae
(S.V.C. and I.V.C.) and pulmonary veins (P.V.) are normally situated. The right sided
atrioventricular valve is bicuspid, and the left sided one is tricuspid. The large ventricular cavity, with its apex pointing to the right (dextroversion), is for practical purposes a single ventricle with remnants of ventricular septum (below the ventricular
septal defect) serving as the point of separation between right and left halves of the
common ventricle. The interior lining of the right sided ventricle is smooth and is
typical of that of a normal left ventricle (L.V.). The wall of the left sided ventricle is
thicker, its interior lining is coarser, and it contains an infundibulum. The latter
two features are characteristic of an anatomic right ventricle (R.V.). Both pulmonary
trunk (P.T.) and aorta (Ao.) arise from the anatomic right ventricle, and both pulmonic and aortic valves are separated from the A-V valves and from one another
by infundibulum. Since both great arteries arise from the anatomic right ventricle,
the only outlet for blood from the anatomic left ventricle is via the huge ventricular
septal defect (V.S.D.). Both pulmonic and aortic valves are on the same horizontal
plane, and these arteries ascend parallel to one another.
The cardiac ndings at autopsy (#34437) are summarized in Figure 3, and
described in detail in Figures4 to 6. He had corrected transposition of the great vessels,
a huge ventricular septal defect (functionally single ventricle), origin of both great vessels
from the anatomic right ventricle (double outlet right ventricle), and dextroversion.
The pulmonary arteries and veins were dilated. Microscopically, the pulmonary
arteries showed medial hypertrophy and focal narrowing of the lumens by brous
intimal proliferation.
COMMENTS
Confusion may arise when the more or less contradictory terms “corrected transposition of the great arteries,” “origin of both great vessels from the right ventricle”
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Figure 4 Various views of the heart. Upper left: Radiograph of the heart specimen.
Alarge deposit of calcium is present in the left atrium (L.A.), and it extends down
one portion of the left A-V valve to the base of a papillary muscle of the anatomic
right ventricle (R.V.). Before this radiograph was taken pins (arrows) were placed laterally (right to left) across the superior margins of each of the semilunar valves. On
this radiograph the two pins arc virtually superimposed on one another, indicating
that one valve lies directly behind the other and that each is on the same horizontal
plane. The latter is a characteristic feature of the entity “origin of both great vessels
from the right ventricle.” (16) R.A.=right atrium. L.V.=anatomic left ventricle. Lower
left: Anterior view of the heart. The apex points to the right. The coronary arteries
have a mirror image of normal: the anterior descending (A.D.) branch arises from the
right circumex (R.C.) coronary artery. The left coronary artery is not shown. The
aorta (Ao.) is anterior to the pulmonary trunk (P.T.). Abbreviations as in upper left;
L.A.A.= left atrial appendage. Upper right: The ventricular cavity is opened along
its entire inferior (caudal) border. From this view the orices of both A-V valves are
seen and each of these valves is continuous with one another across the dashed line
which represents the circumference of the huge ventricular septal defect. The remnant of the ventricular septum (V.S.) is shown. Both aorta (A.) and pulmonary trunk
(P.) arise from the anatomic right ventricle. Lower right: The anterior half of the heart
has now been removed. The interior lining of both anatomic left ventricle (L.V.) and
anatomic right ventricle (R.V.) are well seen. The arrows designate the large communication (V.S.D.) between the two ventricles.
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Figure 5 The atrioventricular valves. Left: Opened right atrium (R.A.), right
A-V valve and anatomic left ventricle (L.V.). This valve has the conguration of a
mitral valve. A=anterior leaet. Right: Opened left atrium (L.A.), left A-V valve and
anatomic right ventricle (R.V.). The large calcium deposits extending from the left
atrium to the base of a papillary muscle may be seen. The solid black line designates
the mitral valve annulus, and the dashed white line, the basal attachment of the
valve leaets. The basal attachment of the valve leaets is not to the mitral annulus,
but to the left ventricular wall, and this malformation constitutes the Ebstein-type
anomaly.
Figure 6 The semilunar valves. Left: From above. The aortic valve (A.V.) is smaller
and anterior to the pulmonic valve (P.V.). The left (L) and right (R) coronary arteries arise from the aorta. Right: Opened anatomic right ventricle (R.V.) showing that
the semilunar valves (A.V. and P.V.) are separated from one another by one limb of
infundibular myocardium and from the A-V valves by another limb of infundibulum. The circumference of the ventricular septal defect is designated by the dashed
white line. LA.=left atrium.
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and “common ventricle” are employed to describe the same heart. Each term is
applicable, however, in the case of the patient presented herein. The aorta arose anterior to the pulmonary trunk (transposition of the great vessels), and the ventricles,
A-V valves, and coronary arteries were inverted in a mirror image of normal (inversion). The anatomic left (“venous”) ventricle was located on the right side of the heart
and the anatomic right (“arterial”) ventricle was located on the left side of the heart.
In contrast to the typical “corrected transposition” heart, however, the pulmonary
trunk was not attached to the venous ventricle, but both great arteries arose from the
anatomic right ventricle (double outlet right ventricle), and neither semilunar valve
was continuous with an A-V valve. The only outlet for blood from the anatomic left
ventricle was through the huge ventricular septal defect. Since only a remnant of
a ventricular septum was present, it appears best to consider this defect as absent
ventricular septum or single ventricle.
The patient described above is very similar to the 47-year-old man reported by
Rawson and Doerner.
13
Their patient also had “corrected transposition,” “origin of
both great vessels from the anatomic right ventricle,” “common ventricle,” “dextroversion,” but in addition had valvular pulmonic stenosis. Most patients with virtually absent ventricular septa die during the rst few months or years of life, and
survival to adulthood is rare. It is well recognized that obstruction to pulmonary
blood ow (by valvular or subvalvular pulmonic stenosis) increases the length of
survival of patients with common ventricle. It is remarkable that the present patient
survived so long since he also had a common ventricle, but no obstruction of ow of
blood to the lungs.
SUMMARY
The clinical and pathologic ndings are described in a 21-year-old man who died
of a complex cardiac malformation consisting of “corrected transposition,” “origin of both great vessels from the anatomic right ventricle,” single ventricle, and
dextroversion.
REFERENCES
1. SCHIEBLER, G. L., EDWARDS, J. E. BURCHELL, H. B., DUSHANE, J. W.,
ONGLEY, P. A. AND WOOD, E. H.: Congenital corrected transposition of the
great vessels: Astudy of 33 cases. Pediatrics (Suppl.), 1961, 27: 851.
2. BERRY, W. B., ROBERTS, W. C., MORROW, A. G. AND BRAUNWALD, E.:
Corrected transposition of the aorta and pulmonary trunk. Amer. J. Med., 1961,
36: 35.
3. LEV, M. AND ROWLATT, U. F.: The pathologic anatomy of mixed levocardia.
Areview of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Amer. J. Cardiol., 1961, 8: 216.
4. LEVY, M. J., LILLEHEI, C. W., ELLIOTT, L. P., CAREY, L. S., ADAMS, P., JR. AND
EDWARDS, J. E.: Accessory valvular tissue causing subpulmonary stenosis in
corrected transposition of great vessels. Circulation, 1963, 27: 494.
5. ESPINO-VELA, J.: On a variety of the “corrected” type of transposition of the
great vessels associated with dextrocardia: Astudy of two cases with autopsy
report. Amer. Heart J., 1959, 58: 250.
6. MORGAN, A. D., KROVETZ, L. J., BARTLEY, T. D., GREEN, J. R., SHANKLIN,
D. R., WHEAT, M. W. AND SCHIEBLER, G. L.: Clinical features of single ventricle with congenitally corrected transposition. Amer. J. Cardiol., 1966, 17: 379.
7. RATNER, B., ABBOTT, M. E. AND BEATTIE, W. W.: Rare cardiac anomaly:
Cortriloculare biventriculare in mirror-picture dextrocardia with persistent
omphalo-mesenteric bay, right aortic arch and pulmonary artery forming
descending aorta. Amer. J. Dis. Child., 1921, 22: 508.
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8. ANSELMI, G., MUNOZ, S., MACHADO, L., BLANCO, P. AND ESPINO-VELA,
J.: Complex cardiovascular malformations associated with the corrected type of
transposition of the great vessels. Amer. Heart J., 1963, 66: 614.
9. TODD, D. B., ANDERSON, R. C. AND EDWARDS, J. E.: Inverted malformations
in corrected transposition of the great vessels. Circulation, 1965, 32: 298.
10. LOCHTE: Ein Fall von Situs Viscerum Irregularis, nebst eincm Beitrag zur Lehre
von der Transposition der arteriellen grossen Gefässstämme des Herzens. Beitr.
path. Anat., 1898, 24: 187.
11. ROYER, B. F. AND WILSON, J. D.: Incomplete heterotaxy, with usual heart mal-
formations. Case report. Arch. Pediat., 1908, 25: 881.
12. ROSLER, H.: Beiträge zur Lehre von den angeborenen Herzfehlern. VI. Über die
angeborene isolierte Rechtslage des Herzens. Wien. Arch. Inn. Med., 1930, 19: 505.
13. RAWSON, F. L., JR. AND DOEKNER, A. A.: Functional cortriloculare. Amer.
Heart J., 1953, 46: 779.
14. 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
pulmonary stenosis. Circulation, 1961, 23: 399.
15. NEUFELD, H. N., DUSHANE, J. W. AND EDWARDS, J. E.: Origin of both great
vessels from the right ventricle. II. With pulmonary stenosis. Circulation, 1961, 23:
603.
16. NEUFELD, H. H., LUCAS, R. V., JR., LESTER, R. G., ADAMS, P., JR., ANDERSON,
R. C. AND EDWARDS, J. E.: Origin of both great vessels from the right ventricle
without pulmonary stenosis. Brit. Heart J., 1962, 24: 393.
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Case 79 aneurysMal dIlatatIon of the Coronary arterIes
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Case 79 Aneurysmal Dilatation of the Coronary
Arteries in Cyanotic Congenital Cardiac Disease*
Report of a Forty Year Old Patient with
the Taussig-Bing Complex
Joseph K. Perloff, MD, Charles W. Urschell, MD, William C.
Roberts, MD, and Walter H. Caulfield, Jr., MD
Washington, D. C.
Clinical, hemodynamic, angiocardiographic and autopsy observations are
described in a forty year old woman with right ventricular origin of the aorta
and biventricular origin of the pulmonary trunk. She lived longer than any
previously described patient with this congenital malformation. At angiography
and at autopsy the extramural coronary arteries were found to be aneurysmally
dilated and tortuous. Coronary arterial ectasia has been described once before
in patients with cyanotic congenital cardiac disease, but the remarkable degree
of dilatation and tortuosity found in our patient has not been recorded.
MARKED dilatation and tortuosity of the coronary arteries in patients with cya-
notic congenital heart disease has been commented upon only once before.
1
This
report describes the clinical, hemodynamic, angiographic and necropsy ndings in
a forty year old cyanotic woman in whom the aorta arose entirely from the right
ventricle, whereas the pulmonary trunk arose from both ventricles. She survived
longer than any known patient with this malformation. In addition, she exhibited
a degree of coronary arterial dilatation and tortuosity that is without precedent in
the literature.
CASE REPORT
The patient, a white housewife, was rst evaluated by us when she was thirtyeight years of age. She was the product of a normal pregnancy and apparently had
been cyanotic from birth. Effort dyspnea, fatigue and orthopnea had been present
from childhood. These symptoms lessened in the teens; she ultimately married
and was able to do light housework. Mild exertional chest pain began at about
twenty years of age and recurred intermittently thereafter. Between ages thirtythree and thirty-eight years, hemoptysis occurred three times. Two episodes were
sudden and severe, each lasting a day. Increased fatigability began at age thirtysix, and there was a 15 pound weight loss between the ages of thirty-six and thirtyeight years.
* From the Department of Medicine, Georgetown University School of Medicine, Division
of Cardiology, Georgetown University Hospital, Washington, D. C. and the Section of
Pathology, National Heart Institute, National Institutes of Health, Bethesda, Maryland.
This work was supported by U. S. Public Health Service Grant HE-09093, by Public Health
Service Career Program Award HE-14,009, and by the Eric T. Paglin Memorial Fund for
Cardiovascular Teaching and Research. Requests for reprints should be addressed to Joseph
K. Perloff, M.D., at the Georgetown University Hospital, Washington, D. C. Manuscript
received November8, 1967.
DOI: 10.1201/9781003409342-10
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When initially seen the patient complained of breathlessness, chest pain and
fatigue but she was still able to do much of her shopping and was moderately active.
On examination, she was a thin, small woman with distinct cyanosis and clubbing
of the ngers but with much less cyanosis and little or no clubbing of the toes. The
arterial pulses were normal, and the blood pressure was similar in the arms and legs
(92/65mm. Hg). The jugular venous pulse was normal in height and contour. The
lungs were clear. Aright ventricular impulse was readily appreciated, and a moderate left ventricular impulse was identied near the mid-clavicular line. The sound
of pulmonary valve closure was palpable in the second left interspace. Auscultatory
signs included a normal rst heart sound, a pulmonic ejection sound, a grade 3/6
mid-systolic murmur maximal in the second and third left interspaces, a loud, single
second sound in the second left interspace, and a soft, inconstant mid-diastolic murmur at the site of the left ventricular impulse. The liver was not enlarged and there
was no edema.
The hematocrit was 60 per cent. On chest roentgenogram (Figure 1) the lung
elds were plethoric, especially the middle and inner thirds. The pulmonary trunk
was dilated. The atria were normal in size, and the right and left ventricles were only
slightly enlarged. The electrocardiogram (Figure 2) showed a P-R interval of
0.20 second with P waves of right atrial and perhaps biatrial hypertrophy. Depolarization
appeared to be clockwise with a frontal plane QRS axis directed inferiorly and to the
right. Precordial leads conrmed the presence of right ventricular hypertrophy with tall
R waves in lead V
trophy was suggested by the relatively high R waves in leads V
and deep S waves in leads V5 and V6. Coexisting left ventricular hyper-
1
and V6 although Q waves
5
of volume overload were not present.
Cardiac catheterization data are summarized in Table 1. A right saphenous
venous catheter entered the right atrium, right ventricle and pulmonary artery in
normal fashion. The descending aorta was easily entered via a patent ductus arteriosus. The atrial septum was crossed through a patent foramen ovale. Retrograde femoral arterial catheterization showed that the right ventricle communicated directly
with the aorta which was in a relatively rightward position. Peak systolic pressures
were identical in right ventricle, pulmonary trunk and aorta. The concentration of
inhaled krypton
85
was greater in the pulmonary trunk than in the brachial artery
Figure 1 Anteroposterior, right oblique and left oblique chest roentgenograms.
Pulmonary blood ow is increased, especially in the middle and inner thirds of the
lung elds (anteroposterior projection). The position of the great vessels appears
normal. The pulmonary trunk is moderately enlarged. The cardiac dimensions are
relatively unimpressive.
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Figure 2 The P waves show hypertrophy of the right atrium and perhaps of the
left atrium as well. There is distinct right ventricular hypertrophy. Coexisting left
ventricular hypertrophy is suggested by the relatively tall left precordial R waves. Q
waves of volume overload are absent.
(index 178 per cent). The oxygen content of pulmonary arterial blood was 22.4 volumes per cent and of aortic root blood 19.7 volumes per cent. Indocyanine green dye
dilution curves showed the following patterns. With injection into the right atrium
and right ventricle, the appearance time was ve seconds, the primary curves large
and the early recirculation peaks (left to right shunt) small. With injection into the
left atrium, the appearance time was ve and a half seconds, the primary curve
relatively lower and the early recirculation peak (left to right shunt) larger. Right
ventricular angiocardiograms (Figure 3) showed opacication of both great arteries. The aorta arose entirely from the right ventricle and the dilated pulmonary
trunk arose from both ventricles (biventricular in origin). The aortic root was in a
relatively anterior position and the aortic and pulmonary valves were in the same
horizontal plane. The patent ductus arteriosus was readily identied. Tortuous coronary arteries were indistinctly seen on the surface of the heart following the right
ventricular angiocardiogram (Figure 3), but were strikingly evident following injection of contrast material into the aortic root (Figure 4). Both coronary arteries were
remarkably dilated and tortuous. In addition, the lateral projection showed that the
rst portion of the aortic root rose vertically and the plane of the aortic valve was
horizontal (Figure 4).
Following these studies the patient was not seen again for two years. She was
then deeply cyanosed, dyspneic and semistuporous, and she died within twentyfour hours.
At necropsy the heart weighed 350 gm. The principal morphologic features are
described in detail in Figures5 through 9. In summary, the aorta arose solely from
the right ventricle, and the pulmonary trunk arose from both ventricles but predominantly from the left (Figure 5). The pulmonary trunk was greatly dilated and
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