The Pathogenesis of Tetralogy of Fallot

Kevin J. Winn, MD and Grover M. Hutchins, MD

A pathogenetic mechanism for the formation of tetralogy of Fallot is postulated.

We propose that the right ventricular ejection stream is divided into a transseptal
aortic stream and an infundibular pulmonary stream during embryonic life before
the ventricular septum is closed. The surgically unaltered hearts from 77 cases of
tetralogy of Fallot were examined, and measurements made of certain morphologic
features. A nmalformed, stenotic pulmonary valve was found in most all cases and
accounts for a divided ejection stream. Infundibular stenosis is shown to be a
postnatally acquired, progressive lesion. The detailed anatomic features of the heart
and great vessels in tetralogy of Fallot are explained as a response of the developing
heart and great vessels to the abnormal blood flow pattern of a divided right ventricular ejection stream. This pathogenetic mechanism, compared to previously proposed hypotheses, appears better able to account for the malformation complex of
tetralogy of Fallot (Am J Pathol 73:157-172, 1973).

THIS STUDY OF TETRALOGY OF FALLOT was undertaken to determine to what extent this malformation complex could be explained
as a morphologic response of the heart to the physical forces resulting from an abnormal blood flow and blood pressure pattern. The
defining features of tetralogy of Fallot (TF) used in this study are
shown in Text-figure 1, which views the base of the heart from below. These features include: a high ventricular septal defect (VSD)
through which the aortic valve protrudes to be positioned over the
ventrictular septum, obstruction in the right ventricular outflow tract
at either the infundibtulum or at the pulmonary valve, and right ventricular hypertrophy.
The classic explanation of the genesis of this malformation is the
malseptation hypothesis,' which holds that the conus arteriosus is
une(jually divided, yielding a small infundibulum, and is incompletely
divided in its inferior aspect, yielding a membranous VSD. More
recently, the infundibular hypoplasia hypothesis 2 has proposed that
the infundibulum is uinderdeveloped in all its dimensions and that
From the Department of Pathologv, The Johns Hopkins University School of Medicine

aind Hospital, Baltimore, Md.

Supported in part by Contract PN 43-67-1444 with the National Institutes of Health

US Public Health Service, the Department of Health, Education and Welfare.
Presented in part at the Seventieth Annual Meeting of the American Association of
Palthologists and Bacteriologists, Washington, DC, February 1973.
Accepted for publication June 12, 1973.
Address reprint requiests to Dr. Kevin J. Winn, Department of Pathology, The Johns
Hopkins Hospital, Baltimore, MD 21205.
157

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Normal

TEXT-FIG 1-The bases of

the normal and TF hearts
viewed from the ventricular
aspect. The TF heart shows an
intracristal VSD with an overriding aorta, right ventricular
hypertrophy, infundibular stenosis and a bicuspid pulmonary
valve.

Tetralogy
of
Fallot

failure of posteroinferior growth of the infundibulum results in a VSD

which would also be membranous in position.
The pathogenetic mechanism of the formation of tetralogy of Fallot
proposed in this presentation is as follows: During cardiac morphogenesis before the ventricular septum is closed, there is division of the
right ventricular ejection stream into a transseptal aortic stream and
an infundibular pulmonary stream (Text-figure 2). This division of the
right ventricular ejection stream is caused by obstruction to flow by a
stenotic malformed pulmonary valve in most all cases. The transseptal
aortic portion of the stream passes through the unclosed ventricular
septum, maintains the patency of this communication and expands
the VSD either behind or into the crista supraventricularis. The decreased volume of flow through right ventricular outflow tract results
in a small infundibulum which develops progressive stenosis in postnatal life.

TETRALOGY OF FALLOT

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-Right Lateral

A-P

Normal

159

|L

L
R

Tetralogy
of

Fallot

L
R

TEXT-FIG 2-Diagram of ventricular ejection pathways in the A-P and lateral planes.
normal human embryo. Bottom-proposed alteration in TF.

Top-a

Materials and Methods

All available hearts from the autopsy files of The Johns Hopkins Hospital with
either a ventricular septal defect or a right ventricular outflow tract obstruction
or both were examined. All cases in which there was absence of fibrous continuity between the aortic and mitral valve were excluded. This eliminated all
cases of transposition of the great vessels and double outlet right ventricle. Cases
with malformations of the artrioventricular canal region were eliminated. Cases
with anomalous muscle bands causing right ventricular outflow obstruction were
felt to represent a distinct entity and were eliminated. Cases with normally
formed but small tricuspid valves were included, while cases of tricuspid atresia
were eliminated. Hearts were also excluded if they had been subjected to intracardiac surgery or such extensive dissection that interpretation was impossible.
There were 138 hearts with TF as previously defined, and 77 of these were suitable for study. In addition, 18 hearts had pure pulmonary valve stenosis (pure
PS), an obstruction to right ventricular outflow with anl intact ventricular septum.
Twenty-one hearts had a high ventricular septal defect and normal semilunar
valves, an isolated VSD.

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The clinical records and autopsy findings for each patienit were reviewed. The
patient's age at surgery and at death were noted. The heart was examined with
particular attention to the structure of the aortic and pulmonary valves, the interventricular septum and the infundibular portion of the right ventricle. Measurements were made of right and left ventricular free wall thickness and the
diameters of the "os" of the infundibulum, the pulmonary valve, the main pulmonary artery, the ventricular septal defect, the aortic valve and the ascending
aorta. The degree of aortic overriding was estimated. The position of the ventricular septal defect, position of the crista supraventricularis and the relative
right and left ventricular chamber volumes were noted. Associated cardiovascular abnormalities and noncardiovascular malformations were recorded.

Results

The ages ranged from stillborn to 34 years. Of the 77 patients with

TF, 39 died at the time of systemic to pulmonary artery shunt surgery.
Eleven other patients had had shunt surgery prior to death. In the
patients with pure PS, 2 of the 18 had undergone systemic to pulmonary artery shunt surgery and 2 others died at the time of similar
procedures. Two of the 22 patients with an isolated VSD had had
pulmonary artery banding procedures, and 4 others died at the time
of surgery.
In all but one case of TF, the aortic valve was a normally formed
three-leafed valve. In that case the aortic valve was bicuspid. Examination of the pulmonary valves in cases of TF revealed a 95% inciPULMONARY
NORMAL

4(5%)

AORTIC
76 (99%)

STENOTIC
TRICUSPID

2(3%)

BICUSPID

43(56%)

UNICUSPID

9(11%)

DOMED

5(6%)

ATRETIC

12(16%)

ABSENT

2(3%)

1(1%)

TEXT-FIG 3-Incidence of semilunar valve malformations in TF. Valve malformations

are schematically indicated with spiral lines representing fused commissures.

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dence of abnormalities as shown in Text-figure 3. All malformed

valves would not open completely and were considered stenotic in
some degree. This would be especially true during early embryonic
life, when the newly formed valve leaflets are very thick. In the two
cases in which the pulmonary valve leaflets were absent, there was a
localized stenosis at the level of the valve ring atop the infundibular
muscle. The valve malformations seen in cases of pure PS are listed in
Table 1. One case with a bicuspid pulmonary valve and all cases of
pulmonary atresia had small "stenotic" but normally formed tricuspid
valves. Infundibular stenosis was conspicuously absent from this group.
There were various degrees of aortic overriding of the ventricular
septum in TF, quantitated on the basis of the number of aortic valve
leaflets that protruded into the VSD. The overriding varied between
33% (1 cusp) and 75% (21/3 cusps).
In TF the position of the VSD from the left ventricular side was
directly below the aortic valve. The position of the VSD, viewed from
the right ventricular aspect, varied from membranous to intracristal
(Text-figure 4, Figure 1). In this latter situation a band of muscle in
30% of the cases and a fibrous band in 15% of the cases was found
separating the tricuspid valve ring from the portion of the aortic valve
ring which protruded through the VSD. This band is part of the
crista supraventricularis. This means that the VSD divides the crista
supraventricularis, and therefore the VSD is intracristal in position in
45% of the cases of TF. In all cases of isolated VSD, the VSD was membranous in position.
The main portion of the crista supraventricularis, the parietal band,
was found to be displaced in an anterior, superior and leftward direction in all cases of TF. This finding was unique to these cases and was
not found in the other two conditions examined.
The shape of the right ventricular outflow tract varied in cases of
TF. In general, it was short and narrow. There were varying amounts
of fibroelastic thickening of the endocardium at the very bottom of the
infundibulum, and in 5 cases there was marked thickening with
valvuloid formation (Figures 1-5). These latter cases represent soTable 1-Pulmonary Valve Malformations in Pure Pulmonary Valve Stenosis

Bicuspid
Unicuspid
Domed
Atresia

4
1
9
4

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30% MUSCLE BAND

15% FIBROUS BAND

TEXT-FIG 4-Position of the

VSD in TF schematically represented, viewing the base of the
heart from the ventricular aspect.
The muscle or fibrous band separating the tricuspid valve from
the aortic valve in 45% of the cases
is a portion of the parietal band of
the crista supraventricularis.

55% NO SEPARATIONi

called fibrous infundibular stenosis. Many cases did not show this endocardial thickening, and these represent so-called muscular infundibular stenosis. In some cases the narrowing of the infundibulum was
localized to the opening or os of the infundibulum, while in others the
narrowing was diffuse along the entire length of the infundibulum.
In still other cases the infundibulum was only slightly narrowed, and
the obstruction to right ventricular oufflow was localized to the pulmonary valve. This was particularly noticeable in cases with domed
pulmonary valves.
There was right ventricular hypertrophy in all cases of TF. In virtuLally
all these cases the right and left ventricular free wall thicknesses were
equal. Similar findings were seen in isolated VSD. Right ventricular
hypertrophy was also present and often marked in cases of pure PS.
Significant malformations of the great vessels were frequently present (22%) in the 77 cases of TF (Table 2). The malformations included: right sided aortic arch (15 cases), vascular ring (1 case) and
absent left pulmonary artery (1 case). Other minor deviations from
normal development of the great vessels included patent ductus
arteriosus (6 cases) and aberrent origin of one subclavian artery (4
cases). Significant great vessel malformations were not present in the

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.DINf

DSMALLEST

OPV

RV OUTFLOW

NORMAL

PURE
PULMONARY
STENOSIS

163

0.9

0.7

3.9

4.9

4.6

1.3

ISOLATED VSD

1.0

TEXT-FIG 5-Schematic and quantitative comparison of the diameters of the infundibulum and pulmonary valve (Dinf/Diw) and of the aortic valve and the smallest right
ventricular outflow tract (DAv/Ds.iailest RV outflow) for the normal heart, TF, pure PS
and isolated VSD.

cases

of

aortic

arch.

pure

PS and in only

case

of isolated VSD, that a right sided

Text-figure 5 shows graphically and numerically a comparison of the

diameters of the infundibulum and pulmonary valve and of the diameter
of the aortic valve and smallest right ventricular outflow tract diameter
averaged for the normal heart and the three conditions examined. The
first ratio (Di.f/Dinv) is a measure of the degree of stenosis of the infundibulum. The second ratio DAv/D.iiiallest RN, ouitflow is a measure of the
overall right ventricular outflow tract obstruction.
For TF the first ratio indicates that there is a greater degree of infundibular stenosis than pulmonary valvular stenosis. The second ratio

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WINN AND HUTCHINS

indicates that the right ventricular outflow tract is in general smaller

than the aorta.
These ratios in pure PS indicate that, although the smallest right
ventricular oufflow diameter is small compared to the aorta, the
infundibulum is not smaller than the pulmonary valve, as in TF. Therefore although there is right ventricular outflow obstruction, it is valvular, and infundibular stenosis is not present in pure PS. The ratios for
isolated VSD are essentially the same as for the normal heart, indicating that there is no right ventricular oufflow obstruction and in particular there is no infundibular stenosis.
When DAv/Dsgtmallest RN otutflow is plotted against the patient's age in cases
of TF (Text-figure 6) it can be seen that the RV outflow is smaller than
normal in early life (normal ratio 0.9) and the relative degree of RV
outflow obstruction increases with age.
When Di,f/DPN- is plotted against the patient's age in cases of TF
(Text-figure 7) it can be seen that the ratio is in the normal range in

6-

5-

4-

i
a

QZ

3-

a
I

I,

0-1

1-2

2-3 3-4 4-5

5-10

1o-15 15-20 20+

YEARS
TEXT-FIG 6-The degree of right ventricular outflow obstruction (DAv/Drn,aII est ltV
outflow ) plotted against the patient's age at death on a logarithmic scale with SD indicated.
Normal ratio is 0.9. Right ventricular outflow tract is smaller than normal at birth and
becomes even smaller with age.

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2.0-

1.5-

1.0

0.5

0-1

1-2

2-3 3-4 4-5

5-10 10-15 15-20 20+

YEARS
TEXT-FIG 7-The degree of infundibular stenosis in the right ventricular outflow tract
(Dinf/Dt ) plotted against the patient's age at death on a logarithmic scale with SD
indicated. The normal ratio is 1.2; ratios less than 1.0 indicate infundibular stenosis,
which is not present at birth but appears in the first months of life and progresses.

early life, and that stenosis of the infundibulum develops in the first
months of life.
Discussion

The finding of malformed pulmonary valves in 95% of the cases of

TF is in agreement with other large series of cases reported in the literature. Rao, Anderson and Edwards " found malformed valves in 94%
of 80 cases, and Lev and Eckner 4 state that, in their series of 158
cases, the pulmonary valve is usually bicuspid, rarely unicuspid or
tricuspid. In the only case in this series with an abnormal (biscuspid)
aortic valve, the pulmonary valve was unicuspid and severely stenotic, much more stenotic than the aortic valve.
Neither the malseptation hypothesis nor the infundibular hypoplasia
hypothesis explain the malformed stenotic pulmonary valve. WVe consider this pulmonary valve malformation to be the primary lesion in
most all cases of TF, causing resistance to blood flow through the
pulmonary artery and division of the right ventricular ejection stream.
Histologic examination of the region of commissural fusion in the
pulmonary valve revealed nonspecific changes, and the mechanism of
valve fusion could not be determined. Any one of several teratogenic insults might be responsible for this lesion.
In this series of cases of TF, the VSD was intracristal in position in
45% of the cases. Others have noted the intracristal position of

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Pathology

the VSD in TF,5 and Lev 6 has described the muscle band which separates the aorta from the triscuspid valve as a second parietal band
of the crista supraventricularis. Both the malseptation hypothesis and
the infundibular hypoplasia hypothesis predict that the VSD would be
membranous in position. This is not so in 45% of the cases in this
series.

We explain the position of the VSD in the following manner: we

propose that pulmonary valve stenosis causes division of the right ventricular ejection stream before the ventricular septum is closed. If the
pulmonary valve stenosis develops after the ventricular septum has
closed, the resulting malformation will be isolated pulmonary stenosis.
Septation of the conotruncal region occurs during Streeter Horizon
XV and is complete with well-formed semilunar valves by Streeter
Horizon XVII. The ventricular septum does not close until Streeter
Horizon XVIII.7 The transseptal aortic portion of the right ventricular
ejection stream

passes

through the unclosed interventricular

com-

munication and maintains its patency. As blood flows through the VSD
into the aorta, the aorta would tend to undergo relative migration to
the right to a position over the ventricular septum. This relative migration of the aorta, viewed from the right ventricular aspect, might
occur behind the crista supraventricularis, which would yield a
membranous VSD as in 55% of our cases of TF. Alternatively, the migration might occur into the developing crista, separating the muscle
fibers of that structure and resulting in an intracristal VSD as shown
in Text-figure 4.
The presence of frequent great vessel malformations in cases of TF
(Table 2) supports the proposal of rearranged outflow streams during
early embryonic life. A rearrangement of the ventricular outflow
streams would tend to result in an unusual pattern of blood flow in
the developing aortic arches. This would favor persistance of unusual aortic arches and would result in great vessel malformations.
The comparison of the diameters of the infundibulum and pulmonary valve and the aortic valve and the smallest RV oufflow tract diameter in TF plotted against the patient's age reveals two important
facts. The first is that the right ventricular outflow tract is small at
Table 2-Major Great Vessel Malformations in Tetralogy of Fallot

Right sided aortic arch

Vascular ring
Absent left pulmonary artery
Total

15
1
1
17/77 (22%)

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birth when compared to the aorta. The second fact is that, although
the entire right ventricular outflow tract is smaller than normal,
localized infundibular stenosis is not present at birth, when compared
to the degree of pulmonary valve stenosis, but develops in the first
months of life.
The pathogenetic mechanism of TF we propose explains these
findings. The infundibular pulmonary portion of the divided right
ventricular ejection stream is less than normal. This decreased relative
flow through the infundibulum results, at birth, in a small infundibulum
which is not yet more stenotic than the pulmonary valve. During the
first few months of life, the infundibulum participates in the generalized right ventricular hypertrophy. In addition to a stenotic malformed
pulmonary valve, this increasing muscle mass in an already small
infundibulum causes increasing resistance to right ventricular ejection during systole. This progressively increasing resistance to infundibular blood flow in the presence of an alternative route for right ventricular ejection, the VSD, leads to a decreasing relative size of or
failure of growth of the infundibular lumen. Finally, at the point of
stenosis (the os of the infundibulum), there is variable fibroelastic
proliferation of the endocardium. Occasionally this fibroelastic endocardial proliferation is exuberant and results in valvuloid formation
(Figures 1-5). The morphology of these changes in this variant of
TF (fibrous infundibular stenosis) is identical to that seen in other
situations in which boundary layer separation could be expected to
occur and permits intimal cell proliferation at a point of absent shear
effect. Similar proliferations are encountered in coarctation of the
aorta 8 and in some instances of aortic valve insufficiency.9
Several clinical, cardiac catheterization and pathologic observations
support the view that infundibular stenosis is a postnatally acquired
lesion related to decreasing relative blood flow through the infulndibulum. Patients with TF usually are not severely cyanotic at birth but
become so, progressively, in the first months of life.10 Levin et al 11
have demonstrated, by cardiac catheterization, that shunts in TF are
bidirectional in early life. Several patients have now been reported1 215'
in which a VSD without infundibular stenosis was found by cardiac
catheterization in early life, and classic TF was demonstrated in later
life. In addition, several patients have been reported in which atresia
of the right ventricular outflow tract has developed following systemic-to-pulmonary artery anastomosis for TF.10- 1 In this situation the
extracardiac shunt would increase resistance to pulmonary artery
blood flow throuigh the infundibulum.2" This decreasing flow through

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the infundibulum is reflected in its decreasing size and eventual

atresia.
In order to further support this mechanism of formation of infundibular stenosis, cases of isolated VSD and pure PS were measured
and analyzed in the same manner as were the cases of TF. In neither
of these conditions did infundibular stenosis develop. In cases of
pure PS there is resistance of infundibular pulmonary blood flow in
the form of a stenotic malformed pulmonary valve but no alternative
route for right ventricular ejection (no VSD). Therefore, all the right
ventricular ejection is forced through the infundibulum, and this
maintains the normal lumen of that structure. In isolated VSD, there
is no resistance to infundibular blood flow. In this situation the infundibular blood flow is normal or increased, and infundibular stenosis does not develop.
The anterior, superior and leftward displacement of the crista supraventricularis in TF has recently received renewed attention.2 This is
easily explained by the transseptal aortic stream passing behind the
main portion of the crista and pushing it in that direction. Conversely,
because the infundibular pulmonary stream is decreased, it would fail
to displace the crista posteriorly and to the right to its usual position.
Mention should be made of a rare condition of isolated infundibular
stenosis. In a recent report, Zaret and Conti 21 classified this condition
into primary and secondary types. The secondary type is explained
as a result of an associated lesion such as pericardial disease or congenital abnormalities. There remains a small group which they term
primary discrete fibromuscular obstruction. Because some patients
with isolated infundibular stenosis have been discovered to have other
peculiar findings such as spontaneously closed VSD,'' it is possible
that these rare cases of isolated infundibular stenosis are all secondary
to similar conditions.
In the 4 patients in this series (5%) with TF and normally formed
three-leafed pulmonary valves, the final morphology of the heart is the
same as that seen in the majority of the cases with malformed stenotic
pulmonary valves. The proposed pathogenetic hypothesis of division
of the right ventricular ejection stream accounts so well for the latter
cases, that we feel the same mechanism is probably responsible for cases
of TF with normal pulmonary valves. In these cases, however,
the lesion which caused division of the right ventricular ejection
stream in early embryonic life is not apparent at the time of autopsy.
It is possible that a misalignment of ventricular ejection streams accounts for the splitting of right ventricular outflow. Similar ideas have

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been advanced to explain the development of transposition of the

great vessels 23 and truncus arteriosus malformation.24
Finally, the pathogenetic mechanism we propose is not directly
contrary to either of the two previously proposed hypotheses. The
conus arteriosus is unequally divided and the infundibulum is small
or "hypoplastic." However, these changes are not due to an intrinsic
defect in the primordium of this region of the developing heart but
represent a response of the developing heart to the abnormal blood
flow pattern that is established by division of the right ventricular
ejection stream.
References
1. Edwards JE, Bulboliani A, Rogers HM: Pathologic and embiyonic considerations in tetralogy of Fallot. Mayo Clin Proc 22:166-172, 1947
2. Van Praagh R, Van Praagh S, Nebesar R, Muster A, Sinha S, Paul M:

3.
4.

5.
6.

7.
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9.
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1]1.

1]2.

13.
14.

Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and

its sequelae. Am J Cardiol 26:25-33, 1970
Rao BS, Anderson R, Edwards J: Anatomic variations in the tetralogy of
Fallot. Am Heart J 81:361-371, 1971
Lev M, Eckner F: The pathologic anatomy of tetralogy of Fallot and its
variations. Dis Chest 45:251-261, 1964
Rosenquist GC, Sweeney LJ, Stemple DR, Christianison SD, Rowe RD: The
ventricular septal defect in tetralogy of Fallot. Am j Cardiol, 1973 (In
press)
Lev M: The pathologic ainatomy of ventricular septal defects. Dis Chest
35:533-545, 1959
Streeter GL: Developmental horizons in human embryos: description of
age groups XV, XVI, XVII and XVIII, beinig the third issue of a survey of the
Carnegie collection. Contrib Embiyol Carnegie Inst Wash 32:133-204, 1948
Hutchinis GM: Coarctation of the aorta explained as a branch point of
the ductus arteriosus. Am J Pathol 63:203-214, 1971
Hutchins GM, Maron BJ: Development of endocardial valvuloids with
valvular insufficiency. Arch Patlhol 93:401-407, 1972
Keith JD, Rowe RD, Vlad P: Heart Disease in Inifancy anid Childhood,
Second edition. New York, The Macmilliani Co, 1967, p 605
Levin A, Boineau J, Spach M, Canienit R, Capp M, Andersoni P: Ventricular pressure-flow dynamics in tetralogy of Fallot. Circulation 34:4-13,
1966
Gasul B, Dillon R, Vrla V: The natural tranisformationi of ventricular
septal defects into ventricular septal defects with pulmonary stenosis and/or
into tetralogy of Fallot: clinical anid physiologic findings. Am J Dis Child
94:424-427, 1957
Fyler D, Rudolph A, Wittenborg M, Nada A: Ventricular septal defects
in infants anid children. Cir-culationi 18:833-851, 1958
B3ecu L, Ihkas D, Ljungquist A, Rudhe V: Evolution of ventricular septal
defect anid pulmoniary stenosis with left to right slhunt into classic tetralogy
of Fallot. Am J Cardiol 7:598-607, 1961

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15. Watson H, Lowe KG: Functionial adaptations of the right ventricular outflow tract in congenital heart disease. Br Heart J 27:408-412, 1965
16. Fabricius J, Hansen P, Lindeneg 0: Pulmonary atresia developing after a
shunt operation for Fallot's tetralogy. 13r Heart J 23:556-560, 1961
17. Sabiston D, Corniell W, Criley J, Neill C, Ross, R, Bahnison H: The diagnosis and surgical correction of total obstruction of the r-ight ventricle:
an acquired condition developing after systemic arteiy-pulmonary artery
anastomosis for tetralogy of Fallot. J Thorac Cardiovasc Suig 48:577-587,
1964
18. Frater R, Rudolph A, Hoffman J: Acquiired pulmonary atresia in tetralogy
of Fallot with functioning Blalock-Tauissig shunt. Thorax 21:457-458, 1966
19. Mizuno A, Sato F, Hasegawa T, Tsuizuki M, Furuse A, Kotoda T, Saigusa
M: Acquired obstruction of the right ventricular outflow tract in tetralogy
of Fallot after Blalock-Taussig anastomosis. Jap Heart J 11:113-119, 1970
20. Rockoff SD, Bilbert J: Functional ptulmonary atresia: a cause of alngiocardiographic misinte r-pretation in tetralogy of Fallot. Am J Roentgenol
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21. Zaret B, Conti CR: Infundibular pulmonic sten;osis with intact ventricular
septum in the adult. Johns Hopkins Med J 132:50-60, 1973
22. Case Records of the Massachusetts General Hospital. N Engl J Med 280:
714-721, 1969
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human heart from age group IX to age group XV. Contrib Embryol Carnegie Inst Wash 37:87-114, 1962
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embryo. Am J Anoat 134:167-174, 1972

Legends for Figures

Fig. 1-Right ventricular outflow tract in TF. In the center, fibrous tissue surrounds
the opening of the infundibulum. The VSD, lower center, is separated from the tricuspid
valve, below, by a muscle band.
Fig 2-Right ventricular outflow tract in TF with valve-like intimal proliferations in
the os of the infundibulum which are also shown in Figure 4. The VSD has no separation from the tricuspid valve.

Fig 3-Early stages of intimal proliferation in the os of the infundibulum. The pulmonary valve is above and the right ventricle below (VVG, x 40).
Fig 4-Valvuloid from heart shown in Figure 2. There are calcium deposits in the core
of the proliferated intimal tissue. (VVG x 20).

Fig 5-Valvuloid which has reattached to the endocardium at its downstream end, at
the top. Its gross appearance resembled that seen in Figure 1. (VVG, x 30).

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[End of Article]