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Case reports In CardIology
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Figure 1 Chest roentgenogram in the patient (A59–151) presented. The prominent shadow at the left upper cardiac border is produced by the dilated, transposed
ascending aorta, and not by the pulmonary trunk, which is small and lies to the right
of the ascending aorta. The gastric air bubble on the right is designated by the arrows.
Figure 2 Angiocardiograms. The contrast material is injected into the systemic
ventricle. a. Anteroposterior projection showing that the aortic valve (A.V.) lies
directly to the left of the pulmonic valve (P.V.), and that the two semilunar valves are
on the same frontal plane. The rudimentary chamber proximal to the pulmonic valve
appears to be lled in a retrograde fashion, since no contrast material is seen entering this subvalvular outow tract from the systemic ventricle. The aorta descends
(D.A.) on the right and the great arteries arising from the arch have a mirror-image
reverse of normal. The patent ductus arteriosus is not clearly identied. b. Lateral
view. The aorta arises anteriorly, indicating transposition of the arterial trunks. In
this view the pulmonary trunk is apparent directly behind the proximal portion of
the ascending aorta.
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Case 17 the sIgnIfICanCe of asplenIa In CongenItal heart dIsease
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Figure 3 Diagrammatic representation of the heart and great vessels. Blood enters
the right atrium (R.A.) through the superior vena cava (S.V.C.) and the hepatic vein
(H.V.). The termination of the inferior vena cava was not determined at the time of the
original dissection, but it is apparent that this vessel did have an abnormal course.
The coronary sinus is absent. Blood in the right atrium either enters the left atrium
(“L.A.”) through defects in the lowermost and midportions of the atrial septum, or enters
the systemic ventricle directly through a common atrioventricular valve. No vessels are
connected to the left atrium, although a small protrusion on its surface suggests a rudimentary vascular bud. The right and left pulmonary veins drain into a common pulmonary vein (shown in gure4), which in turn terminates by dividing into two branches:
the larger one connects to the “left” gastric vein; the smaller one, to the portal vein. The
left-sided atrium is anatomically a right atrium in that its wall is composed entirely of
pectinate muscles. The systemic ventricle, which is large and thick-walled, functions as
a single ventricle. The leaets of the common AV valve are not continuous with those of
either the aortic or pulmonic valves. An intramural opening (0.3cm. in diameter) below
the aortic valve connects the systemic ventricle to a rudimentary, smooth-walled chamber below a stenotic dome-shaped, unicuspid, unicommissural pulmonic valve. The pulmonary trunk is hypoplastic. The aortic valve is located on the same plane and directly
to the left of the pulmonic valve. The aorta arises anteriorly and does not cross the pulmonary trunk in its ascent. Asmall patent ductus arteriosus, which is connected to the
right pulmonary artery, is present. The pressure in the systemic ventricle was recorded as
70/5mm. Hg and the peripheral arterial oxygen saturation was 58 per cent. In summary,
there is total anomalous pulmonary and systemic venous drainage, persistent common
atrioventricular canal, common ventricle, transposition of the great vessels, stenotic subpulmonary outow tract with pulmonic valvular stenosis, patent ductus arteriosus, right
aortic arch, absent coronary sinus, and anatomic double right atrium.
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Figure 4 This drawing demonstrates partial situs inversus, symmetrically lobed
lungs, and abnormal systemic and pulmonary venous connections in the patient
described. The largest lobe of the liver is on the left, the stomach and tail of the
pancreas, on the right. The gallbladder is in the midline, and the spleen is absent.
The colon and appendix are normally located but the mesenteric attachments of the
small intestine are abnormal.
SUMMARY AND CONCLUSION
The nding of asplenia and situs inversus in a patient with congenital heart disease
virtually precludes the presence of cardiac lesions which would be beneted by corrective or even palliative surgical procedures.
REFERENCES
1. IVEMARK, B. I.: Implications of agenesis of the spleen on the pathogenesis of
cono-truncus anomalies in childhood: An analysis of the heart malformations in
the splenic agenesis syndrome, with fourteen new cases. Acta Paediat. Suppl. 104,
44: 110, 1955.
2. PUTSCHAR, W. G. J., AND MANION, W. C.: Congenital absence of the spleen
and associated anomalies. Am. J. Clin. Path. 26: 429, 1956.
54
3
Case 17 the sIgnIfICanCe of asplenIa In CongenItal heart dIsease
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3. LYONS, W. S., HANLON, D. G., HELMHOLZ, H. F., DUSHANE, J. W., AND
EDWARDS, J. E.: Cardiac Clinics. CXLVIII. Congenital cardiac disease and asplenia: Report of seven cases. Proc. Staff Meet. Mayo Clin. 32: 277, 1957.
4. LUCAS, R. V., NEUFELD, H. N., LESTER, R. G., AND EDWARDS, J. E.: The sym-
metrical liver as a roentgen sign of asplenia. Circulation 25: 973, 1962.
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Case 18 Spontaneous Closure
ofVentricular Septal Defect*
Anatomic Proof in an Adult with Tricuspid Atresia
William C. Roberts, MD, Andrew G. Morrow, MD, Dean T.
Mason, MD, and Eugene Braunwald, MD
SPONTANEOUS CLOSURE of ventricular septal defect has been suspected on
the basis of clinical examinations by physicians caring for children with congenital cardiac disease. Conrmative clinical and hemodynamic evidence documenting spontaneous closure of such lesions also have been presented in several recent
reports.
of a ventricular septal defect been recorded.
was established and who died after operation. At autopsy, there was unequivocal
evidence that a functional ventricular septal defect had been present and had subsequently closed. The clinical and pathologic observations leading to this concluion
are summarized in this report,
CLINICAL SUMMARY
A. H. (No. 03-87-02), a 27-year-old man, had had cyanosis, clubbing, and a precordial
murmur since infancy. During childhood and adolescence, fatigue, dyspnea, and
repeated upper respiratory infections prevented him from attending school. At the
age of 17 a left subclavian-pulmonary arterial anastomosis was performed at another
hospital. The cyanosis and dyspnea, however, were only transiently improved, and
his physical activity became progressively limited.
and toes. The heart was enlarged, and a left ventricular thrust was palpable. The second sound at the base was single, and a grade II/VI ejection-type systolic murmur
and a faint continuous murmur were heard at the upper left sternal border. The electrocardiogram revealed left ventricular hypertrophy, left axis deviation, left atrial
enlargement, and abnormal initial forces indicative of an old anteroseptal myocardial infarct. Fluoroscopic and radiographic examinations disclosed enlargement of
the left ventricle and hypoplasia of the pulmonary arterial segment. The hematocrit
value was 82 per cent.
munication into the left atrium and then into a ventricular chamber, where a pressure of 116/12mm. Hg and an oxygen saturation of 83 per cent were recorded.
Simultaneously, the systemic arterial pressure was 112/66 mm. Hg and systemic
arterial oxygen saturation was 88 per cent. Neither the pulmonary artery nor the
right ventricle was entered by the catheter. Indicator-dilution curves indicated a
1–6
In only one patient, however, has anatomic proof of spontaneous closure
7
We recently studied an adult patient in whom the diagnosis of tricuspid atresia
On examination he was cyanotic, and there was marked clubbing of the ngers
At right heart catheterization the catheter passed across an interatrial com-
* From the Pathologic Anatomy Department, Clinical Center, and the Clinic of Surgery
and Cardiology Branch, National Heart Institute, National Institutes of Health, Bethesda,
Maryland.
Dr. Roberts present address is Department of Medicine, The Johns Hopkins Hospital,
Baltimore, Maryland.
56
DOI: 10.1201/9781003409342-7
Case 18 spontaneous Closure of ventrICular septal defeCt
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Figure 1 Diagram summarizing the multiple cardiac anomalies in the patient
herein described. There is atresia (agenesis) of the tricuspid valve, a large atrial septal defect (A.S.D.), a large left ventricle (L.V.) (functional single ventricle), a ventricular septal defect (V.S.D.) which has closed, and a hypoplastic right ventricle. The
pulmonic valve is bicuspid. S.V.C., superior vena cava; I.V.C., inferior vena cava; R.A.,
right atrium; L.A., left atrium; P.V., pulmonary vein; and P.T., pulmonary trunk.
large right-to-left shunt at the atrial level. Aselective angiocardiogram with right
atrial injection conrmed the clinical diagnosis of tricuspid atresia.
At operation an anastomosis was created between the distal end of the right pulmonary artery and the proximal end of the superior vena cava. The procedure was
complicated by the presence of an extensive collateral circulation between the lung
and chest wall, and the patient died in the early postoperative period of massive and
uncontrollable bleeding into the pleural space.
PATHOLOGIC FINDINGS
The pertinent patho-anatomic features of the heart are summarized in gure1 and
illustrated in gures2 through 4. Aclosed defect was present in the basal portion
of the muscular ventricular septum. The gross and microscopic appearance of this
lesion is shown in gure3.
DISCUSSION
In this patient the evidence provided by both gross and microscopic study furnishes
proof not only that a ventricular septal defect had been present but that prior to
its spontaneous closure it had been of functional signicance. This is indicated by
the prominent jet lesion still evident in the right ventricle and also by the size of
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Figure 2 Photographs showing the interior of the cardiac chambers. Upper left: The
right atrium. No remnant of the tricuspid valve is present. The ostium of the coronary
sinus is also atretic. The atrial septal defect (D.), which measures 3.5 by 2.0cm., is of the
foramen ovale type. The superior (S.V.C.) and inferior (I.V.C.) venae cavae are connected
normally to the right atrium. Upper right: The left atrium (L.A.), mitral valve and left
ventricle (L.V.). The valve guarding the foramen ovale is totally incompetent resulting in
the large atrial septal defect (D.). The dashed circle depicts the communication between
the coronary sinus (C.S.) and the left atrium. The left ventricular chamber is considerably dilated and its wall thickened. Lower Left: The ascending aorta (Ao.), aortic valve
(A.V.), and septal wall of the left ventricle (L.V.) are shown. The ventricular septal defect
(V.S.D.), which has closed, is located immediately below the aortic valve. This view also
illustrates the normal continuity between the anterior leaet of the mitral valve (A.M.L.)
and the aortic valve. The ostia of the coronary arteries are apparent. These vessels were
widely patent and normally distributed. Lower right: The anterior wall of the hypoplastic right ventricle (R.V.) has been removed, exposing the site of the former defect (V.S.D.)
in the muscular ventricular septum. Note the jet lesions on the endocardium of the
right ventricle adjacent to the site of the former opening in the ventricular septum. The
pulmonic valve (P.V.) and pulmonary trunk (P.T.) are only slightly smaller than normal.
Note that the left ventricle (L.V.) accounts for most of the mass of the heart. The inset is
the bicuspid pulmonic valve as seen from above. (R.A.) right atrium.
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Case 18 spontaneous Closure of ventrICular septal defeCt
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Figure 3 Photographs demonstrating the gross and histologic appearance of the
closed ventricular septal defect. Upper left: The closed defect from the left ventricular (L.V.) aspect. The site of the former defect (designated by the arrows) is a linear indentation 1 cm. below the aortic valve (A.V.). The endocardium adjacent to
the indentation is elevated, smooth, and pearly white. The endocardial thickening
is probably the result of turbulent ow of blood in this area. Lower left: The site
of the former defect as viewed from the right ventricular aspect. The anterior wall
of the hypoplastic right ventricle has been removed. The depression between the
muscle bands is the site of the former defect. The arrow points to the pearly white
endocardial thickening, clearly the result of a jet lesion, on the lateral and superior
walls of this chamber opposite the depression. (P.V.), pulmonic valve. Upper right:
Photomicrograph of a section through the closed defect in the muscular ventricular
septum. The entire area of the former defect was blocked, embedded in parafn, and
serially sectioned at intervals of 6 micra. In none of the sections was a residual opening apparent. Arepresentative section is shown here. Note that the actual closure of
the defect is produced by brous proliferation (jet lesion), and not by direct apposition of the myocardium. No lesions were found in the adjacent myocardium. (R.V.),
right ventricle; (L.V.), left ventricle. Verhoeff-Van Gieson elastic tissue stain: original
magnication, × 8. Lower right: Photomicrograph of the jet lesion on the laterosuperior aspect of the right ventricle. There is marked broelastic thickening of the
endocardium. Verhoeff-Van Gieson elastic tissue stain: original magnication, × 21.
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Figure 4 Photographs demonstrating the marked bronchial arterial collateral
circulation in the patient described. This patient’s relatively long life-span probably can be attributed to the enormous bronchial collateral blood ow which was
further augmented by the subclavian-pulmonary arterial anastomosis. Upper: The
descending thoracic aorta is opened. The dilated ostia of the bronchial arteries are
designated (arrows). Lower: Photomicrograph of a section of lung demonstrating
the dilated and thick-walled bronchial arteries (arrows). The bronchial cartilage is
on the right. Verhoeff-Van Gieson elastic tissue stain: original magnication, × 16.
the right ventricle. For some time prior to the terminal operation and death, pulmonary blood ow was supplied entirely by systemic collateral vessels and by the
subclavian-pulmonary arterial anastomosis. The right ventricle was functionless
and received no blood except that minute amount which may have been returned to
it from Thebesian vessels or retrograde through the pulmonic valve. Had this situation been present throughout the patient’s life, the right ventricle would probably
have been atretic. Instead, its cavity, although small, approximated the size of the
pulmonary trunk, which was essentially normal. It seems clear, therefore, that the
right ventricle attained its size as a result of ejecting blood that was shunted into it
when the defect was patent.
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Case 18 spontaneous Closure of ventrICular septal defeCt
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Spontaneous closure of a ventricular septal defect is probably a relatively
unusual occurrence, and it would appear likely that only those defects whose margins are entirely muscular can do so. Edwards
7
has suggested that the closure of
defects of this type, which occur relatively infrequently, may be related to progressive elongation of the myocardial bers bordering them. Initially the defect may be
round or oval but with growth it becomes slit-like and nally its margins approximate each other as the myocardium hypertrophies and stretches. In the elderly
patient reported by Edwards closure was apparently effected entirely by apposition
of muscle. In the present patient this process also was operative but actual closure
resulted from endocardial proliferation, probably stimulated by turbulent blood
ow through the defect. In the usual type of ventricular septal defect, involving
principally the membranous septum, muscle approximation is impossible and it
would seem unlikely that closure of a defect in this location could occur without the
superimposition of an active inammatory process, such as bacterial endocarditis.
In this regard, it should be noted that the patient described gave no history suggestive of endocarditis and there were no lesions in the myocardium bordering the
closed defect that suggested previous inammation.
Of additional interest is the prolonged survival of the present patient. Recently
Fontana and Edwards
8
reported 125 cases of tricuspid atresia conrmed at autopsy,
119 of which were collected from the literature. Two thirds of these patients died
within the rst year of life and only eight lived for more than 10years. Probably the
main factor allowing such a long survival in our patient was the extensive bronchial
collateral circulation. The left subclavian-pulmonary arterial anastomosis, which
was performed when the patient was 17years old, further augmented the collateral
blood ow to the lungs. The decrease in pulmonary blood ow during his latter
years, as evidenced by increasing cyanosis and disability, no doubt was caused by
progressive closing of the ventricular septal defect.
REFERENCES
1. AZEVEDO, A. DE C., TOLEDO, A. N., CARVALHO, A. A. DE, ZANIOLO, W.,
DOHMANN, H., AND ROUBACH, R.: Ventricular septal defect; an example of
its relative diminution. Acta Cardiol. 13: 513, 1958.
2. HARNED, H. S., AND PETERS, R. M.: Spontaneous closing of ventricular septal
defects: Two cases reported. Abstract, Circulation 22: 760, 1960.
3. EVANS, J. R., ROWE, M. B., AND KEITH, J. D.: Spontaneous closure of ventricular
septal defects. Circulation 22: 1044, 1960.
4. NADAS, A. S., SCOTT, L. P., HAUCK, A. J., AND RUDOLPH, A. M.: Spontaneous
functional closing of ventricular septal defects. New England J. Med. 264: 309, 1961.
5. AGUSTSSON, M. H., GASUL, B. M., ARCILLA, R. A., BICOFF, J. P., AND
MONCADA, R.: Spontaneous closure of ventricular septal defect in eight children
demonstrated by serial cardiac catherization and by angiocardiography. Abstract,
Circulation 24: 874, 1961.
6. BLOOMFIELD, D. K.: Spontaneous closure of ventricular septal defect: Clinical
and pathologic correlations. Abstract, Circulation 24: 890, 1961.
7
. EDWARDS, J. E.: Congenital malformations of the heart and great vessels. In
Gould, S. E.: Pathology of the Heart. Springeld, IL, Charles C. Thomas, 1953, p.266.
8
. FONTANA, R. S., AND EDWARDS, J. E.: Congenital Cardiac Disease: AReview of 357
Cases Studied Pathologically. Philadelphia, PA, W. B. Saunders Company, 1962, p.291.
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