Clinical Studies

The Syndrome of Pulmonary Stenosis with

Patent Foramen Ovale*
ARTHKR SELZER, M.D.,WILLIAM H. CARNES, M.D.,CHARLES A. NOBLE, JR.,M.D.,WILLIAM
H. HIGGINS,JR., M.D. and ROBERT 0. HOLMES, M.D.
San Francisco, California

T
HE demonstration by Blalock and intact interventricular septum and patency
Taussig’ that persistent cyanosis in of the foramen ovale in adults. The striking
certain types of congenital cardiac similarity of these cases and the fact that
disease can be reduced dramatically by a neither of them was diagnosed correctly
surgical operation has brought into focus during life prompted us to review similar
the great practical importance of correct cases reported in the literature. The find-
clinical diagnosis of congenital cardiovascu- ings in all the available autopsied cases
lar malformations. The great interest in have been summarized and contrasted with
congenital heart disease which has de- the clinical and pathologic features of
veloped in the last decade has therefore similar congenital cardiac lesions.
shifted recently from the non-cyanotic to
the cyanotic group. CASE REPORTS
A considerable number of malformations
of th(> cardiovascular system are associated CASE I. W. H. .J., a thirty-nine year old
white male, entered Stanford University Hos-
with cyanosis. Many of these are not com-
pitals on April 24, 1946, because of increasing
patible with life for more than a few days
dyspnea. His past history revealed that there
or months. A small number of the patients
was some question as to whether or not he was
survive until childhood and only very few a “blue baby” but his early development was
reach adult life. Maude Abbott2 stated normal. At the age of seven he became some-
that nine-tenths of the cases of morbus what dyspneic upon exertion but played
ceruleus in adults were cases of the tetralogy normally. At the age of twelve he was forced
of Fallot. In the last few years the variation to drop out of games. In high school he at-
of the tetralogy of Fallot described by Eisen- tempted to play football but was unable to do so
menger” has aroused interest and has been because of dyspnea.
accepted as a clinically recognizable He never noticed cyanosis until the age of
eighteen when he developed slight cyanosis
entity. In 1945 Currens, Kinney and White4
while swimming. During the third decade ot
reported eleven cases of still another cy-
life cyanosis was very mild, tnostly noticeable
anotic congenital cardiac lesion, namely,
upon exertion and he led a relatively normal
pulmonary stenosis with intact interventric- life working as a tool grinder. At thirty-one
ular septum and summarized the clinical there was slight intensification of cyanosis; at
findings but no consistent diagnostic criteria thirty-four cyanosis became moderately severe
were established. and his activities were markedly curtailed be-
Rcccntly we have had the opportunity to cause of dyspnea. This state of affairs was
observe two cases of pulmonary stenosis with gradually intensified in severity until he was

* From the Departments of Medicine and Pathology, Stanford University School of Medicine, the Divisions of
Medicine and Pathology, University of California Medical School and the San Francisco Hospital (Department of
Public Health, City and County of San Francisco).
 Pulmonary Stenosis, Patent Foramen Ovale--Selser et ul.

FIG. 1. Roentgenograms and electrocardiogram in Case I. A, postero-anterior view showing slight cardiac enlargc-
ment, dilatation of the pulmonary artery and marked dilatation of the left branch. B. left anterior oblique view
showing elevation of the apex and a notch indicating the interventricular groove (arrow). c, four-lead electro-
cardiogram showing prominent P-waves and a pattern of right bundle-branch block.

completely incapacitated and cyanosis became The heart sounds were not remarkable, but
extreme. both were rather loud and snapping at the
Two years before death he developed attacks base of the heart in a sitting position.
of vertigo, with nausea, vomiting and oc- Laboratory findings were as follows: vital
casional hematemesis. He had few questionable capacity, varied between 3.5 and 4 L.; venous
convulsions during the last year. About six pressure, 9 cm. of water, circulation time (arm
weeks before death he was bronchoscoped be- to tongue) 12 seconds. Blood count: hemo-
cause of marked enlargement of the left hilar globin, 183 per cent, red blood cells, 9.4 mil-
shadow in the chest roentgenogram, suspected lion, leukocytes, 9,500 with a normal distribu-
as representing a possible tumor, and was found tion. Urinalysis revealed slight albuminuria,
to have partial constriction of the left main pH 5.5, specific gravity 1.007 and occasional
bronchus from outside pressure. leukocytes and rare erythrocytes in the sediment.
Examination upon entry to the hospital re- Electrocardiogram showed right axis devia-
vealed an extremely cyanotic man with marked tion with QRS complexes widened to 0.11
clubbing of the fingers and toes. Other pertinent seconds and notched in all leads. T waves were
findings included slight cardiac enlargement inverted in leads III and IVY. There was a tall
with a moderately loud systolic murmur at the and prominent Pz. (Fig. lo.) Chest roentgeno-
apical region, somewhat softer at the left gram revealed a normal-sized heart with a
sternal border, and faint at the base of the heart. somewhat elevated apex of the heart and a
 Pulmonary Stenosis, Patent
NIL. 7. Drawing oi’ the hrart and great vessels in Case 1. A, anterior view of the heart and lungs showing position 01
ttlc. heart and the dilated pulmonary artery. B. s-&on of thr heart showing the thickness of the two ventricles and
tllc. dilate-d ptllmonary artery. Imel upper right hand cornrr. pulmonary valve seen from abovr
marked enlargement of the pulmonary artery
and its left branch. (Fig. lo.) In the left
anterior oblique position the apex of the heart
showed a notch, sug*gesting the interventricular
groove and thus showing that it was formed
mostly by the right ventricle. (Fig. lB, arrow.)
The patient had repeated attacks of more
SCVCI‘Ccyanosis with dyspnea. In one of these
attacks, on the fourth day after admission, he
bt~came irrational and then lapsed into a coma
arid died.
Xt autopsy it was revealed that the head,
mucosae and upper chest were deep blue. The
fingers were moderately clubbed and the nail
beds blue. The toes were not clubbed. There
was no palpable subcutaneous edema. The
serosal surfaces of the abdominal, thoracic and
pericardial cavities were normal and there was
no fluid in the cavities. The liver edge did not
descend below the costal margin.
The heart lay more nearly transversely in the
thorax than normally. The entire visible surface
from the anterior view was formed by the right
ventricle and atrium. (Fig. 2A.) The right
atrium was markedly dilated. The right ventri-
cle was dilated and markedly hypertrophied.
Its wall measured 18 mm. thick in the conus
region. The foramen ovale was covered by the
usual endocardial flap, the left margin of which
was free so that a round probe 1.5 cm. in
, A I 1’AH1’ I ‘14 0
Foramen Ovale---S&r et al. 5
diameter could be passed through it. ‘The tri-
cuspid ring measured 12.9 cm. An interrupted
row of small, pale vegetations about 1 mm.
wide was found along the line of closure of the
tricuspid leaflets and there was an appreciable
thickening of the leaflets and several chordae
tendineae. The mural endocardium of both the
atrium and the ventricle was smooth and thin.
The pulmonic valve was extremely sienosed.
(Fig. 2s.) It was composed of a dome-shaped,
stiff, white membrane with a tiny, round orifice
between 2 and 3 mm. in diameter. A continu-
ous row of tiny, pink, translucent vegetations
lined the free margin of this orifice. There were
no distinct separate cusps but four distinct
thin commissures joined the membrane to the
base of the pulmonary artery. The pulmonic
valve ring had a circumference of 5 cm. The
pulmonary artery dilated gradually from the
valve ring to the bifurcation and its wall be-
came much thinner in the distal end but the
intima was smooth and normal. The dilatation
extended into the left main branch of the pul-
monary artery giving rise to an aneurysmal sac
in the left hilar region correspondins; to the
shadow in the x-ray film. (Fig. lo.) The wall
of this dilated artery was thin and the intima
perfectly smooth but for the wrinkles produced
by its collapse when emptied. Thy vessel was
perfectly elastic. There was only a small plaque
6 Pulmonary Stenosis, Patent Foramen Ovale-Seizer et al.

in the intima of the artery 2.4 cm. distal to its generally were delicate. The pulmonic valve
origin marking the former orifice of the ductus was markedly thickened, due principally to
arteriosus. The dilatation did not extend into increased thickness of the central stratum which
the secondary branches of the pulmonary was hyalinized and contained a fine, dust-like
artery. The right main branch of the pulmonary basophilic deposit of calcification. Tiny thin-
artery was about one-half the diameter of the walled vessels extended through this layer
left and appeared normal. almost to the free margin of the valve which
The pulmonary veins emptied normally into was surmounted by a small, acidophilic, hya-
the left atrium. There was no enlargement of the line, amorphous deposit that contained no cells.
left atrium or ventricle. The mitral valve ap- The tricuspid valve also contained a number of
peared normal and its ring measured 8.7 cm. small, thin-walled vessels and small, amorphous,
The left ventricle was 10 mm. thick. The mural acidophilic deposits on its line of closure which
endocardium was smooth; the aortic valve were virtually acellular. The mitral valve was
appeared normal. Its ring measured 7.2 cm. moderately vascularized but showed no vegeta-
The myocardium had a normal color and tions. The aortic valve was normal. The aorta
appearance except for a small white scar near contained only very small intimal plaques com-
the apex of the left ventricle. The coronary posed principally of foam cells. The pulmonary
arteries were free of sclerosis. artery had a normal structure except that the
The ascending ~aortai contained a few very thickness of the wall in the distal dilated portion
thin yellow intimal plaques. A small yellowish- was reduced to about one-half that of the proxi-
white plaque in the arch marked the former mal portion. A cross section of the ligamenturn
orifice of the ductus arteriosus. A thin, short arteriosum showed a complete obliteration of
ligamentum arteriosum, without a lumen, joined the lumen by hyalinized fibrous and elastic
this to the left pulmonary artery. The left tissue. The lungs were normal except for the
bronchial artery arose just distal to the arch presence of occasional eccentric thickenings of
of the aorta by an unusually large orifice about the walls of small arteries. (100 to 200 micra
2 mm. wide. The vessel was tortuous and a diameter.) The injected radiopaque material
little larger than normal. In order to trace its could be identified in small bronchial arteries,
course the lungs, heart and aorta were removed pulmonary arteries and occasional very dilated
en bloc. A thick suspension of bismuth oxychlo- capillary vessels in the peribronchial connective
ride was injected under a pressure of 130 mm. tissue and surrounding alveolar walls. There
of mercury into the cannulated artery. This was marked passive congestion of the other
suspension, which ordinarily does not pass the viscera.
arterioles, poured profusely out of the left
Comment. This patient presented the
pulmonary artery indicating unusually wide
fully developed picture of congenital heart
anastomoses.
The lungs weighed 670 Gm. together. They disease with persistent cyanosis. Secondary
were pale, soft and crepitant. The liver weighed polycythemia and clubbing of the fingers
1,375 Gm. and its cut surface showed patchy and toes offered objective evidence of
lobular atrophy and hyperemia. The spleen chronic anoxia. Cyanosis developed rather
weighed 255 Gm. and was very firm and almost late in life and did not interfere with a
black. The kidneys weighed 340 Gm. together moderately active life until the age of
and were similarly very dark and hyperemic. thirty-four. At that time intensification of
The organs and tissues generally were all suf- dyspnea and cyanosis started the patient
fused. The bone marrow of ribs, sternum,
on a gradual downhill course, with death
vertebra and femur were uniformly deep red.
at the age of thirty-nine resulting from
Histologic examination showed that the
severe anoxia. From the diagnostic stand-
myocardium contained distinct perivascular
cuffs of fibrosis, particularly in the interventricu-
point the confusing clinical factor was the
lar septum. The myocardial fibers of the right radiologic appearance of the widely dilated
ventricle were thickened. The small scar in the pulmonary artery, the left branch of which
left ventricle was composed of old hyalinized was at one time mistaken for a lung tumor.
collagenous tissue containing many small Pathologically, there was a high degree
thick-walled vessels. The coronary arteries of stenosis of the pulmonary valve with a
AMERICAN JO”RN.4’. OF MEDICINE
 Pulmonary Stenosis, Patent Foramen Ovale-S&er et ul.

FIG. 3. Roentgenograms in Case II. A, postero-anterior viw of the heart showing a normal-sized heart with a
hypoplastic aorta and prominent pulmonary artery. B, diodrast-cardiogram interpreted as showing thy con-
trast medium filling the superior vena cava, the right auricle and a stream of the contrast material waching the
left aurick through a patent foramen ovale (arrow). (Courtesy of Dr. Earl Miller, Department of Radiology,
L’nivwsity of Cacforn;a Hospital.)

peculiar cup-shaped deformity of the fused She was next seen at the C-niversit~
valves, a dilatation of the pulmonary California Hospital in November, 1946 be-
artery and wide patency of the foramen cause of dyspnea, weakness, dizziness and faint-
ovale. There was also evidence of large ing on exertion. She stated at that time that
collateral circulation to the pulmonary these symptoms had occurred off and on during
her whole life but were occurring with increas-
branches from the bronchial artery. The
ing frequency during the previous thrcxe months.
right ventricle was severely hypertrophied,
She was found to have moderate cyanosis and
but there was no evidence of chronic con-
marked clubbing of her fingers and toes. The
gestive failure. heart was slightly enlarged to the left, and there
<:ASE II. L. c., a twenty-five year old was a moderately loud systolic murmur over
divorced white woman, entered San Francisco the entire precordium, best heard in the third
Hospital on January 10, 1947, complaining of intercostal space at the left sternal border which
dizziness and episodes of fainting on exertion. was also radiating to the axilla and the left
She had first been seen in this hospital in scapula. The second sound was louder at the
August, 1943 because of salpingo-oophoritis, pulmonic area than at the aortic. The liver was
and at that time was also thought to have con- slightly enlarged and somewhat l.ender. 11
genital heart disease and rheumatic heart blood count revealed a hemoglobin of 16.6 Gm.
disease with mitral stenosis and pulmonary and 6.5 million erythrocytes. An electrocardio-
insufficiency. She gave a history of having gram showed rnarked right axis deviation with
rheumatic fever at four and since then she has inverted T waves in leads II, III, and 1x7~ and a
had episodes of exertional dyspnea, dizziness prominent tall Pz. (Fig. 4~.) Roentgen ex-
and occasional syncope which she attributed amination of the chest revealed the heart to be
to rheumatic heart disease. She was not cyanotic within normal limits of size with a prominent
but was found to have clubbing of the fingers shadow of the pulmonary artery. (Fig. 3.4.)
which she thought she had had for many years. Diodrast cardiography was interpreted as show-
Her hemoglobin was 15 Gm. and the red blood ing an interauricular communication with a
count was 4.35 million. right to left flow of the contrast material (Fiq.
,, ,NUTAKY. 1949
8 Pulmonary Stenosis, Patent Foramen Ovale--S&er et al.

FIG. 4. Electrocardiograms in Case II. A, four-lead electrocardiogram showing the patterns of right ventricular
hypertrophy. a: unipolar extremity leads. c, precordial leads, both confirming the diagnosis of right ventricular
hypertrophy.

3~), large pulmonary arteries and hypoplasia progressively downhill. She became more
of the aorta. Diagnosis of congenital heart markedly cyanotic and dyspneic and fainted on
disease with an interauricular septal defect such mild exertion as turning in bed. During the
and hypoplasia of the aorta was made. last ten days she developed a low grade fever
Following discharge from the University of which was unexplained and she expired on
California Hospital, the patient continued to February 6, 1947.
have dyspnea, dizziness and fainting episodes Autopsy was performed seventy-six hours
with increasing frequency and finally, in Janu- after death by one of us (R. H.) and was limited
ary, 1947, reentered the San Francisco Hospital. to examination of the thoracic cavity because
At that time the cyanosis was found to be severe of the terms under which consent was granted.
and she would faint if allowed to stand for a The body was that of a well developed, well
few minutes or upon any exertion. Her blood nourished, white female measuring 64 inches
pressure was 120/90. There was a widely split in length and appearing to be about the stated
first heart sound at the apex and a moderately age of twenty-five years. The weight was ap-
loud, harsh systolic murmur transmitted to the proximately 110 pounds. The body showed no
axilla. Another systolic murmur was heard at edema, but there was well developed post-
the second and third left intercostal space. mortem lividity over its posterior and lateral
Second sound was present at the pulmonic area. aspects. There was bilateral clubbing of the
The liver was slightly enlarged; there was no fingers and toes.
edema. Laboratory studies were essentially The right pleural cavity contained 100 cc. of
negative except for a hemoglobin of 15.9 Gm. clear, amber-colored fluid, and the left pleural
and erythrocytes numbering 11 million. The cavity contained 250 cc. of a similar type fluid.
hematocrit reading was 60. An electrocardio- The mediastinum was in the midline and small
gram showed the pattern of right ventricular remnants of thymic tissue and fat were present
hypertrophy, essentially as shown in Figure 4~, in the superior mediastinum. The pericardial
with additional confirmation of this diagnosis sac contained 50 cc. of clear, amber-colored
by unipolar extremity leads and leads VI - vs. fluid.
(Fig. 4~.) The heart was transverse in position with the
Her course in the hospital was slowly but anterior surface made up of dilated right auricle
 Pulmonary Stenosis, Patent Foramen Ovaie---Seizer P[ al. !,

FIG. 5. Photograph of the heart in Case II. A, view of the heart, the stcnosed pulmonary valve and the thin-walled,
dilated pulmonary artrry. B> view of the hypertrophied right auricle and ventricle and the patent foramcn ovalc
(forct~ps). CL close-up of thr pulmonary valve viewed from above.

and enlarged right ventricle. It weighed 330 Gm. thickness and the artery was dilated above the
and measured 13 by 10.5 by 7 cm. The wall of valve ring. It measured 2 cm. in diameter just
the right auricle was thickened and on its outer above the valve, and was 3.5 cm. in diameter
surface were small, fibrous, ,granuiar thicken- 2 cm. above the valve ring. In the region of the
ings of the epicardium. The foramen ovale was ductus arteriosus the pulmonary artery was
patent and allowed passage of one finger through closely approximated to the under surface of the
the interauricular septum. On the left auricular aorta and showed a small dimple in the wall.
side was a well developed, thin, fibrous septum On the aortic side there was a deeper, funnel-
with a crescentic opening measuring 1.5 cm. shaped depression which extended obliquely
long. The septum covered the opening suffi- through the wall of the aorta toward the dimple
ciently to have acted as a flap valve preventing in the pulmonary artery. There was no com-
the flow of blood from the left auricle to the munication from either side. .4 large athero-
right auricle whereas flow from the right to the matous plaque was present in the aorta adjacent
left was possible. The left auricle was small and to the obliterated ductus. The wall of the left
thr wall averaged 2 mm. in thickness. The ventricle averaged 11 mm. in thickness and the
tricuspid valve admitted three fingers with ease chamber was normal in size. The mitral and
and measured 8.5 cm. in circumference. The aortic valves showed no evidence of disease and
valve cusps were thickened and there were small were normal in structure. The circumference
warty, pale. greyish-pink vegetations along the of the mitral valve was 9 cm. and that of the
margin of closure near the centers of two of the aortic valve was 6 cm. The aorta was small with
cusps. Some of the chordae tendineae were a wall of normal thickness. Its circumference was
quite markedly thickened. The wall of the 6 cm. immediately above the aortic ring. 5 cm.
right ventricle was hypertrophied and varied 3 cm. above the ring, and 3.9 cm. 6 c-111.ahove
in thickness from 10 to 15 mm. The pulmonary the ring. The lower part of the thoracic aorta
valve showed a complete fusion of all of the was 3.5 cm. in circumference. The coronary
free margins of the cusps so that there was a vessels had a normal distribution and the walls
dome-shaped, fibrous diaphragm with a central were not thickened.
opening which measured 2 mm. in length and The right lung weighed 360 Gm. and the left
1 mm. in w-idth. This was the only opening lung weighed 340 Gm. Both lungs were normally
through the pulmonary valve ring. The margins aerated and no gross areas of consolidation were
of the opening were fringed with numerous tiny, present. They were moderately congested but
Ileaded. translucent, friable, greyish-pink vege- not otherwise unusual upon cut section.
tations. (Fi,q:. 5.) The wall of the pulmonary Histologic examination revealed that the
artery was approximately one-third the usual myocardial fibers in the right vrntriclc. were
,3NI.AR\, 1749
 IO Pulmonary Stenosis, Patent Foramen Ovale-Seizer et al.

increased in size and were as large as those in Comment. This patient developed visible
the left ventricle. The nuclei were centrally cyanosis only a few months before death
placed and frequently were rectangular in out- although clubbing of the fingers and toes
line. The myofibrils were well preserved but was known to have existed for a long time.
cross striations were indistinct. The majority of
Cyanosis and polycythemia progressed with-
the fibers had an increase in the amount of
in a very short time to extreme degrees and
perinuclear pigment, and occasional fibers
the patient died at the age of twenty-five
showed separation of the fibrils. There were
from anoxia. Roentgenologic examination
occasional small patchy areas of fibrosis between
muscle bundles and perivascular fibrosis was revealed a dilated pulmonary artery and,
also occasionally found. Small foci of lympho- with the use of diodrast, a defect in the
cytes and mononuclear cells were present in and interauricular septum with a right to left
adjacent to the fibrous areas. Aschoff bodies blood flow.
were not seen. The cusps of the pulmonary Necropsy showed a diaphragm-type
valve were thickened, with the thickest portion stenosis of the pulmonary valve of high
midway between the orifice and the attachment, degree, a widely patent foramen ovale,
and tapered toward the orifice. The cusps had marked hypertrophy of the right ventricle
an increased vascularity with numerous small
and a dilated pulmonary artery. There was
vessels far out toward the free margin. The
evidence suggesting that the ductus arterio-
thickening was due to the presence of broad
sus may have been patent in adult life and
hyalin collagenous fibers. The surfaces of the
valve were covered with a smooth layer of obliterated shortly before death. The recent
endothelial cells except at the orifice where the obliteration of the ductus arteriosus may
endothelium was interrupted by a proliferation have been the cause of the sudden develop-
of large, stellate-shaped cells with large hyper- ment and rapid progression of severe
chromatic nuclei. These cells showed a slight cyanosis.
tendency toward palisading and extended out-
FINDINGS IN CASES TAKEN
ward for a short distance into masses of fibrin
which were adherent to the underlying con- FROM THE LITERATURE

nective tissue of the cusp. The fibrin was homo-

Maude Abbott’s chart of 1,000 cases of
geneous and contained very few cells. No
congenital heart disease2 contains twenty-
bacteria could be demonstrated within the
five cases of pulmonary stenosis with intact
vegetations. The valve cusps attached to the
root of the pulmonary artery in a normal man-
interventricular septum. 5-26 Sixteen of these
ner. The artery above the valve was thin but had a patent foramen ovale and in nine the
showed the usual histologic arrangement of foramen ovale was closed. All but two of
elastic fibers. The tricuspid valve was thickened these cases have been reviewed and an ad-
with increases of collagenous connective tissue ditional twenty-seven similar cases have
and blood vessels. Along the margin of closure been collected from the literature.4’27-40 In
there was a zone of endothelial ulceration and order to establish characteristic features for
an adherent fibrinous vegetation similar to the differential diagnosis of these cases a
those found on the pulmonary valve. The well documented group of control cases with
structure of the mitral and aortic valves was
other congenital lesions and cyanosis has
normal. Sections through the walls of the aorta
been assembled. This includes twenty-eight
and pulmonary artery in the region of the
ductus arteriosus showed a short small channel
cases of proven tetralogy of Fallot41-62
with the lumen obliterated by a completely and thirteen cases of Eisenmenger com-
organized fibrous mass. The aorta had a wall of plex. 3*31,63-71 Since the primary objective
normal thickness with intact elastic fibers and of this report is to establish diagnostic cri-
no evidence of degeneration or inflammation. teria for fully developed clinical entities, the
Sections from several portions of the lungs control group was limited to autopsied sub-
showed patchy early bronchopneumonia as well jects above fifteen years of age and con-
as quite pronounced congestion of alveolar sisted of Abbott’s cases supplemented by
capillaries in most regions. more recently reported cases.
 Pulmonary Stenosis, Patent Foramen Ovale--Selger et al. 11

(:ases of pulmonary stenosis* with intact lesions. Table II contrasts the incidence and
inter-ventricular septum and patent fora- degree of cyanosis in this syndrome with
men ovale are presented in some detail. that in other forms of pulmonary stenosis
(Table I.) The twenty-three cases of pul- and in the Eisenmenger complex. The first
monary stenosis with both septa intact and part of the table shows that pulmonary
the control group (twenty-eight cases of stenosis with closed foramen ovale is a non-
the tetralogy of Fallot and thirteen cases cyanotic lesion with rare exceptions. The
of the Eisenmenger complex) have been contrast between these two forms of
analyzed and their various clinical pulmonary stenosis has been analyzed in
and pat hologic features compartd with relation to the mechanism of cyanosis
those of pulmonary stenosis with patent elsewhere.7” It may be p ointed out here.
foramen ovale in Tables II to VII. Special however, that a critical review of the six
care was exercised in the compilation of the cases of pulmonary stenosis with closed
tables to avoid error resulting from the foramen ovale and cyanosis” reveals that
comparison of groups containing children only one of them presented the fully de-
as well as adults with control groups con- veloped picture of chronic cyanosis with
sisting of only those above the age of fifteen. secondary polycythemia and clut)bing.2g
Whenever it was thought that the age may In this case no detailed description of the
have influenced the incidence of various foramen ovale is presented and an error
features, cases of pulmonary stenosis with cannot be ruled out. In the remaining five
closed interventricular septum were also cases the evidence of persistent cyanosis was
limited to those over fifteen in order to make not very convincing. On the other hand, in
all groups comparable. the single case of pulmonary stenosis with
‘I’ablc I summarizes clinical and patho- patency of the foramen ovalc in an adult
logic findings in patients with pulmonary without cyanosis the foramen was very
stenosis with patent foramen ovale and in- small. Therefore, it appears that cyanosis
cludes twenty-seven cases from the litera- in pulmonary stenosis with intact interven-
ture and two of our own. The similarities tricular septum depends on the shunt from
between many of these cases justify separa- the right auricle to the left auricle through
tion of this syndrome as a clinical entity not the patent foramen ovalc.
only from the tetralogy of Fallot and the In the second and third parts of Table II
Eisenmenger complex, but from pulmonary pulmonary stenosis with patent for-amen
stenosis with both septa intact as well. Some ovale is compared with the tetralogy of
of the clinical features of pulmonary stenosis Fallot and the Eisenmenger complex. There
with patent foramen ovale are characteristic is no basic difference between the first two
enough to make this syndrome a clinically types in the degree of cyanosis and the age
recognizable entity. These features can best of onset. Severe cyanosis is infrequent in the
be emphasized by comparing this group of Eisenmenger syndrome but the age of onset
cases with others having similar congenital of cyanosis is similar to that in the other
lesions. types.
C,:yanosi.s. The most important single Polyc_ythemia and Clubbing. Secondary-
feature in the diagnosis of congenital heart polycythemia and clubbing of the fingers
disease is cyanosis. Cyanosis is the dividing and toes offer objective evidence of long-
line between various groups of lesions. It is standing arterial anoxemia. Table III shows
shown conclusively in Table I that pul- the incidence of these signs in the four types
monary stenosis with patent foramen ovale of congenital heart disease under discussion
belongs to the cyanotic group of congenital in cases in which the observations were
recorded.
* Pulmonary stenosis is used hereafter to signify
pulmonary stenosis with intact interventricular septum
Auscultatory Physical Findings. Ausculta-
as contrasted with the tetralogy of Fallot. tion of the heart is an important method of
 TABLE I

SIIMM.4RY OF FINDINGS IN TWENTY-NINE CASES OF PULMONARY STENOSIS WITH PATENT FORAMEN OVALE,

ARRANGED IN ORDER OF AGE AT TIME OF DEATH

- - - - -
i Pulmonarv Stenosis
Heart
Cast Age. Physical Signs Cyanosis 3l”b- Blood Count Elecrro- X-Ray CZ3”X Weigh
1 Pulmonar) Other Features
Author I
NO. Sex bing cardiogram of Death Artery
(Cm.1
TYPO Degree
^
Currens 3 mo. Pulmomc systolic 0 0 iormal Subdural Stenosis 1 cm. Open
19454 F murmur; hematoma of an- diameter
PI diminished nulus
Kossman 4 mo. Precordial systolic +++ I.B.C., 7.7 1Vormal Heart Dia- Pinpoint open Large Patent ductus
1942’8 F mlllXlllr million heart failure phragm arteriosus
l:b., 22 Gm. w
Taossig 7 mo. Harsh precordial +++ 1Pulmonarv Heart Fused 1.5 mm. Patent Moder-
194711 M systolic m”rm”r con”s rdi failure cusps diameter ately
forming dilated
dome
Barlow 4 yr. Pulmonic systolic + + Measles F”ooel- Small slit Admits Dilated
1878’6 F murmur shaped goose
dome quill
Andrew 6 yr. Loud pulmonic + 0 Erysipelas cup- Minute Widely Ductus arteriosus
1864” M svstolic murmur shaped ,%perr”rc open admits small
probe; bicuspid
aortic valve
6. Curl-ens 11 yr. Loud m”rm”r, + Cerebral 340 Fused BUtIOn- open
19451 M thrill at base; Pt abscess valves hole 1.1 by 0.3
diminished 9 mm. cm.
long
7. 11 yr. Pulmonic and Since ++ Anoxia Cup-like Round open Ductus arteries”
F apical systolic birth central closed
m”rm”rs, Pz +++ opening
diminished
8. iaundby 11 YI‘. Systolic and dias- 0 Heart Adherent Round 1”; 6 inch Tiny pulmonic
18792” ti tolic murmur at failure valves B/(6 inch vegetations
base form
ring
9. Tuley 13 yr. Sysrolic and dias- Since + Subacute 350 Fused Admirs Markedly Massive pul-
1917’S M relic m”rm”r and 9 yr. bacterial rings small dilated manic vegeta-
thrill at base + endocardi- probe tions
> tis
k 10. \bbott 14 )‘. Moderately loud Progressiv ++ I.&C.. 7.6 Iiight axis IEnlarged Ao0xia Fused Circular Admits Tiny pulmonic
19231’ F pulmonic systolic since 9 million deviation, heart, full- valves ;pz;g pencil vegetations
h
murmur; Pz Y’. <gb; 120 per tall P ness of pul- form
p
diminished +++ cent waves monary mem- diameter
” artery brane
> 11. Gaines 15 yr. Loud pulmonic 0 ,,........ Tuberculosis Ostium 8 mm. 2 mm.
z 1913’S F systolic murmur stenosed diameter perfora-
tion
c0 12. ‘“WXlS 17 VT. Moderately loud Mild sinct ++ I.B.C., 8.7 I tight axis IEnlarged Anoxia 380 Fused O\%l 1 cm. Moderately
1945,’ M’ pulmonic systolic 4, +++ million deviation, heart and valves 6 mm. diameter dilated
C diameter
murmur; PI since 15 Igb., 22 Gm. tall P pulmonary
?J
diminished Waves artery
z Fused I.arge bronchial
l?. Vandam 17 \r. Loud pulmonir Dnset at 2 ++ <.B.C., 10.1 I light ven- Prominent Operation 2 mm. Widely illghtl)
> mdlion tricular pulmonary (aof% cusps dlamrtrr patent dilated arteries
194720 F systolic murmur progres-
r Co”=-
and thrill sive to Igh., 21 Gm. hypertro- artery non- the&?)
0 phy pulsating like
a 14. Nilks 18 yr. Pulmonir systolic +++++ ,,,....... Tuberculosis Funnel 4 mm. 2.5 cm. Tiny pulmonic
18589% F murmur ,I shaped diameter diameter vegetations
z dia-
phragm
fi 15. Peacock 20 yr. Systolic murmur ++ + Tuberculosis 360 Dia- Triangle 6 mm.
1848’2 M and thrill, left phragm admits diameter
0
e sternal border; pencil
2: loud P,
m -
-
 - T
Heart
1( I I-R*\ i\cigh
:&II.)

l- I-
20 vr I Pulmonir T\stolic ince ++ R.U.C.. 7.0 light bundl Vormal Pneumonia 520
M’ m”rm”r 17 vr. million branch heart, and hear!
+++ Hgh.. 21 Gm block, ball enlarged failure
P wa\‘ca pulmonaq
awry
17 Pan1 20 yr. -‘“lmomr systolic ince 7 ) r., Tubrrnrlusi.* \\ idcl\ ) .\ncurvs-
18712; M m”rm”r and severe patrnt ma1 ciila-
thrill, diastolic since 14
m”rm”r
21 )‘. Pulmonic sysrolic ++“+’ ++ R&C., 6.6 Pulmonan CUP- Perforation
F murmur and million infarct shaped 4 mm.
thrill Hgb., 100 pet mem- diameter
ce”f brane
21 )‘_ Pulmonic sysmlic Tubcrculosic 470 Subval- ‘l‘rianglr
F m”rm”r and WIX 3 mm.
thrill dia-
phragm
20. Niergarth 21 yr. s ince Tuherculo- 215 Funnel- 5 mm. 1 cm.
18892’ M mfanq sis shaped diamrtu diamrtcr
+++ dia-
phragm
22 yr. Loud systolic mw t+ Heart 630 F,WZd 5 mm. b>
F mur, left strrnal termin- failure WXl”.X 5 mm.
border ally
/ +++
22. /McPhedran 23 yr. Soft apical systoli< since R.B.C., 12.5 .,. 390 Funnel- 3 mm. 8 mm. Endocardius
19242
, F lnurlnur, PP birth; million shaprd diamclrz diameter tricuspid valw,
diminirhrd +++ Hgh., 140 prr tiny pulmonic
since 16 cent vegetations
13 1’ .oud pulmonir ince birth f-t 5 mm. 1.0 h? 1.3 D”ct”s arreriosna
F systolic m,,rn1,,,- +++ diamrrrr rrn. closed
and rhrill
24 :I OurCasr
’ ,I 25 ,I’. Loud s)ntolic mur ++ inm R.R.C., 11.0 :ighr axis 330 Funnrl- 2 mm. Large
F mur, left sternal since million drviation. shaped diamrtw
border; P9, A? at 24 yr f’+ Hgh.. 16.6 Gm tall P dia-
aprx waveq phragm
25. i currens 30 yr. Systolir murmur ++ Q.B.C., 5.8 Lohar 430 Fi hrons .\drniw
1 1945” M left sternal Is ince million pncun,onra Cusiun 01 pencil
bordrr; loud PJ childhood Hgb > 90 p’c, valws
cent

20. / Frerirhs 34 yr. Diastolic m”rmw ++ Tohrrrulo-

185327 M left sternal hor- -is (%JA
/ der: P? heard
i phragm
79 \r Wild s>stolir m”- +t light axis Xlatrd Anor,a 535
M’ mur. apes dc\iation, pulmollar)
tall P \\a\c> arrq
I

?‘I Paul 57 \I. Prrcwdial mur- , mild sinrr ,

’ ,X7,?, 1, i m”r and ibl-ill. I child-
1 P?a”dihlr.
I
I I
‘4 Pulmonary Stenosis, Patent Foramen Ovale--S&r et al.
TABLE II identifying and differentiating various con-
INrXnBNCr, ONsE’r AND DEGREE OF CYANOSIS IN VARIOIK
FORMS OF PULMONARY STENOSIS AND IN THE
genital cardiac lesions. It is fully realized
EISENMENGER COMPLEX that compilation of data from such a hetero-
I. Cyanosis in pulmonary stenosis with and without geneous group of cases reported by various
patency of the foramen ovale
authors at different periods can only be
I I considered approximate, especially since
Pulmonary Pulmonary
Stenosis with Stenosis with this is an entirely subjective method of
Patent Closed examination, The intensity of the murmurs
Foramen Foramen
and their transmission were not commented
Ovale Ovale
upon in a sufficient number of cases to war-
rant inclusion in the discussion. The location
-I--

Cyanotic patients of the points of maximum intensity of the

total number. 25 6
overtheageof15...... 17 3
murmurs is presented usually as described
Non-cyanotic patients by the authors, except that murmurs re-
total number. ( 4 17 corded as best heard in the second and third
overtheageof15...... 13
I 1 intercostal space at the left sternal border
were consolidated with those described as
located in the pulmonic area, and murmurs
II. Onset of cyanosis in pulmonary stenosis with patent best heard in the third and fourth, or the
foramen ovale in the tetralogy of Fallot and in the
Eisenmenger complex fourth and fifth intercostal spaces were
grouped together as lower left sternal border.
Many murmurs were recorded as precordial
Pulmonary
Stenosis without better specification and are pre-
Tetralogy Eisen-
Age of Onset p$it of menger
sented here as such. In order to bring out
Fallot Complex better the differences in location of the
Foramen
Ovale murmurs those with specified location of
the maximum intensity were then divided
l---l into two groups: (1) those over the upper
o-5.. .i 10 9 part and (2) those over the lower part of the
6-10.. ,_ _.~ 3 5
cardiac projection of the chest wall. The
11-15. .
over 16.. 4 4 third intercostal space provided the rough
information not dividing line. No correlation could be found
available. 8 10
between the location of the murmurs and
the type of pulmonary stenosis found at
autopsy. The murmurs of valvular and sub-
III. Degree of cyanosis during terminal illness valvular stenosis were not distinguishable.
- -
Diastolic murmurs were all described as
Pulmonar! 7
early diastolic, located to the left of the
’ Stenosis
with sternum, except for two cases in which a
Tetralogy Eisen-
Patent
of menger
mitral type of rumbling apical murmur was
Foramen
Fallot Complex recorded. It was noted that of the five early
Ovale
(Patients diastolic murmurs in the tetralogy of Fallot
over 15) three subjects were found to have bicuspid
_ pulmonary valves.
None............ 1 1
In a few cases description of physical
Mild. 0 2 findings included comments on the intensity
Moderate. 8 5 of the second sound at the second left inter-
Severe. 9 17
-- costal space. The second sound at that area
Total. 18 25 12 was described as decreased or absent in five
- L cases of pulmonary stenosis with intact in-
AMERICAN JOURNAL OF MEDICINE
 Pulmonary Stenosis, Patent Foramen &ale--- S&er et al. “5
terventricular septum and in one case each fecting the size? shape and position ol’ the>
of the tetralogy of Fallot and the Eisen- cardiac shadow. However, a rev&\. of the
mcnger complex. On the other hand, it was data revealed that the most striking feature
accentuated in four cases of the former was the appearance of the pulmonary
group and in five of the control group. It artery, regardless of the size and shape of
was noted that in four cases of the tetralogy the ventricular shadow. Table v shuws the

TABLE III
‘GABLE IV
INCmENCE OF POLYCYTHEMIA AND CLUBBING IN PATIENTS
OVER THE AGE OF FIFTEEN

Pul- Pul- 1 I’d- / Pul- ~

monary m0nary
! monary ) -nary ( ,,
Stenosis Stenosis Eisen-
Tetral- ~Stenosis I Stenosls T&al- Eisen-
with with
ogy of with with ORY menger
Fora- Fora- COG
Fallot Fora- ’ Fora- ( of Com-
men men plex
i mrn men ~ Fallot pk:
Ovale Ovale
I Ovale Ovale
Closed Patent
Closed 1 Patent

Polycythemia
Systolic murmurs
present. ( 8 9 5
1 Prccordial.. 3 4 1 1
absent.. 0 2 0
2. In upper half
Clubbing
of card&.. I
present. 11 17 8
projection. 15 20 7 3
absent. 2 3 2
Pulmonic 11 15 .i 3
Area base of.
the heart.. 5 2 I)
of Fallot with a normal or loud second sound 3. In lower half! 4~
at the left sternal border the pulmonic of cardiac
projection.. 5 13 13 -
valves were not grossly abnormal.
f.ower left
Electrocardiographic Findings. Electrocar- sternal ~
diograms were reported in twenty-five of border.. I 4 1 12 3
Apical area. 1 2 1 4
the ninety-three cases. Right axis deviation Diastolic
was, as expected, the prominent feature of murmurs ~ ti 4 5 i 6
all four lesions discussed here. It was present
Total: informa-
in all but one case and that was a case of tion available. 22
the Eisenmenger complex. In four cases I 28 21 ‘l
right bundle branch block was reported,
three uf which were cases of pulmonary contract between the x-ray appearance of
stenosis with intact interventricular septum cases of pulmonary stenosis with and with-
and one Eisenmenger complex. In none of out patency of the foramen ovale and of the
these four cases were multiple precordial tetralogy of Fallot. All patients with pul-
leads or unipolar leads available to permit monary stenosis, with the exception of one
a finer differential diagnosis between a true infant, had evidence of dilatation of the
conduction defect and ventricular hyper- pulmonary artery while in the tetralogy of
trophy. Prominent, tall P waves were re- Fallot the pulmonary artery was small in
ported in eleven cases and were less common ten cases, questionably enlarged in one case
in the tetralogy of Fallot than in the other and in one other the cardiac shadow ap-
two types of pulmonary stenosis. None were peared normal. In most cases of pulmonary
reported in the Eisenmenger complex. stenosis the cardiac shadow was not much
Roentgemlogic Findings. It is rather diffi- enlarged so that the two films shown in
cult to compare the x-ray appearance iti a Figures IA and 3~ can be accepted as repre-
group of patients with so many factors af- senting typical examples of the x-ray ap-
,-Z”“AH\I’. 1940
16 Pulmonary Stenosis, Patent Foramen Ovale--S&r et al.

pearance of pulmonary stenosis as judged Course nnd Prognosis. In spite of the rela-
from the fifteen autopsied cases in which tively small number of cases analyzed a
x-ray examinations were reported. In the certain amount of useful information con-
tetralogy of Fallot, too, the heart was most cerning the prognosis of the various lesions
frequently normal in size. “Sabot’‘-shaped can be obtained by comparing the ages
elevation of the cardiac apex was recorded attained by the patients and the cause of

TABLE VI
TABLE v
PROGNOSIS OF THE FOUR TYPES OF CONGENITAL HEART
ROENTGENOLOGIC SIZE OF THE PULMONARY ARTERY
DISEASE
AND BRANCHES
- I. Age at death of oatients who survived fifteen years
of life
Pul- Pul-
nonary Inonary
PUI- Pul-
ltenosis 2jtenosis retral- Eisen-
monarv monary
with with ogy menger
Stenosis Tetral- Eisen-
Closed Patent of Com-
with with ogy menger
Fora- Fora- Fallot plex
Closed Patent of Com-
men men
Fora- Fallot plex
Ovale Ovale
men men
Ovale Ovale

Prominent /-.___
pulmonary !
15-20. ....... 2 I 6 12
artery. 1 (?)
21-30. ....... 6 8 12
Small
31-40 ........ 4 2 2
pulmonary
Over 41 5 3 2
artery. 10
Pulmonary
artery not II. Causes of death in these patients
remarkable.. 1
- - Heart failure. 3 3 3
Anoxia 1 2 2
Bacterial
in some, but not in all cases. In one case
endocarditis. 6 1 8
there was a right-sided aortic arch. The Pulmonary
characteristic deep concavity of the left tuberculosis. 1 7 5 0
Cerebral abscess 0 3 3 1
cardiac border was present even in those
Other causes. 5 3 3 0
with cardiac enlargement, notably in the
remarkable case of Volini and Flaxmans
in which the cardiac shadow occupied 80 to death. This is shown in Table VI. Pulmonary
90 per cent of the transverse diameter of the stenosis with closed septa and the Eisen-
chest and the heart weighed 750 Gm. In menger complex appear to have a some-
the Eisenmenger syndrome the six patients what better prognosis than pulmonary
with a record of radiologic examination stenosis with patent foramen ovale and
showed a very characteristic appearance the tetralogy of Fallot.
in that the pulmonary artery was very Pathologic Features. The great majority
prominent, there was a very marked dila- of examples of pulmonary stenosis with in-
tation of the hilar shadows and the lung tact interventricular septum show strikingly
fields were intensely congested. Fluoroscopic similar anatomic deformities. Of the fifty-
examination reported in cases of the Eisen- one cases forty-three were due to fusion of
menger complex invariably revealed promi- the cusps. In thirty-two of these the fusion
nent pulsations of the hilar shadows. In was complete so that a dome-shaped or
contrast the dilated pulmonary arteries in conical membrane with a small central
pulmonary stenosis with intact interven- opening resulted. Sometimes the commis-
tricular septum were usually described as sures could be distinguished even when
showing absent or slight pulsations. there were no separate cusps as in the two
AMERICAN JOURNAL OF MEDICINE
 Pulmonary Stenosis, Patent Foramen Ovale--mSelzeret al. 77
cases described in this report. In Table VII differs from pulmonary stenosis with closed
1tw tytm of stenosis are compared in the foramen ovale. This is a rundamencal clif-
tctralogy of Fallot and in the two classes ol ferencc which justifies vet-y strict separation
pulmonary stenosis with intact interven- of these two conditions which arc so often
tricular septum. Valvular fusion occurred in grouped together as “pulmonary stenosis
all three classes but it was the nearly ex- with closed interventricular septum.” The
clusive form of stenosis only in the cases striking contrast is best illustrated in adult
with patent foramen ovale and intact inter-
ventricular septum. The diaphragm was TABLE VII
PATlIOLOGlC FINDINGS LN VARIOUS TYPES OF PI,,.M”NAH,’
relatively thin and delicate in some of the STENOSIS
youngest subjects but it was thick and rigid I. Type of pulmonary stenosis
in the older patients such as the two re- /
ported herein. Histologic examination of the ~ Pul- 1 Pul- /
valves was rarely reported except in the , ~~,“~~~ / ~~~~~ j Tetral-
presence of bacterial endocarditis. Tiny with with ogy
of
bland vegetations were often observed on Closed ! Patent
Fallot
Foramen Forameni
the free margins of the valve orifice. Thick- Ovale 1 Ovale ~
ening and mild deformities of the tricuspid
___...__A __~_~__+ ~~~/ ~~ ~~~~
valve were frequently noted. The frequent
presence of dilatation of the pulmonary Valvular fusion,
diaphragm type. 9 23 2
artery in those with intact interventricular Valvular fusion, partial, 6 5 8
septum is recorded in the second part of Hypoplasia, pulmonary
Table VII. The third and fourth parts of the artery.
Subvalvular membrane, 1:;:
I :
table show that dilatation of the pulmonary Conus separate chamber. 5 0 1
artery is directly related to the degree and .--

location of the stenosis. Total. 29 29 20

I
It is customary to assume that these cases
II. Size of pulmonary artery
of pulmonary valvular stenosis are con-
genital. Typical examples have been ob- I
Small. 1 ~ 3 9
served at autopsy in subjects only a few Normal. 2 ~ 1 5
months old and symptoms and signs have Dilated. . 6 12 5
been present since birth in many instances. 1
It would be difficult to prove the congenital III. Relation of the size of pulmonary artery to the type
of stenosis
origin in many cases in adults in whom -
symptoms appeared late and in whom
Sub-
fibrous thickening and vascularization of Valvular
valvuiar
Stenosis
the valves so closely resembled the results Stenosis
of rheumatic disease. There are too few
reports of careful histologic studies of the Small pulmonary artery. . 8 4
heart to provide a basis for generalization Dilated pulmonary artery 22 1
on this point. Whatever the pathogenesis -/
of the valvular lesion, the circulatory effects IV. Relation of the size of pulmonary artery
to the degree of stenosis
and clinical results would be expected to
he the same in all essential respects. I I
Moder-
Severe Mild
COMMENTS Stenosis Stz&is ~ 1Stenosis
It appears from the evidence presented I
that pulmonary stenosis with patency of the
Small pulmonary artery.
foramen ovale is a distinctive entity. Clini- Dilated pulmonary artery
cally, it is a cyanotic disease and thereby
_)4UTJ4RY, 1943
18 Pulmonary Stenosis, Patent Foramen Ovale-User et a~.

patients who, with the same degree and portance of the open foramen ovale in
position of pulmonary stenosis, with similar right-sided cardiac lesions is not generally
incidence of marked right ventricular hyper- appreciated.
trophy and of cardiac failure, develop Cyanosis in pulmonary stenosis with
chronic cyanosis with secondary polycy- patent foramen ovale varies in intensity
themia only in the presence of a patent and may occasionally be absent. In this
foramen ovale. The cyanosis can only be respect it is not different from the tetralogy
interpreted as indicative of a large venous- of Fallot and the Eisenmenger complex.
arterial shunt through the foramen ovale. With any cyanotic lesion, patients may
The existence of a right to left shunt has present the picture of morbus ceruleus from
been demonstrated in one of our patients birth or may show intermittent cyanosis,
by angiocardiography and in another re- late onset of cyanosis or gradual intensifica-
ported case by cardiac catheterization.30 tion of cyanosis. Pulmonary stenosis with
That such a course of blood flow is compati- patent foramen ovale occupies an inter-
ble with cardiodynamics even in the mediate place between the tetralogy of
absence of right ventricular failure and tri- Fallot, the most severely cyanotic lesion and
cuspid insufficiency has been discussed the Eisenmenger complex, the least cy-
elsewhere in a study of cyanosis.72 anotic of the three. It is doubtful, however,
In pulmonary stenosis with closed fora- whether the degree or time of onset of
men ovale there is no demonstrated cause cyanosis can be used to differentiate these
of chronic cyanosis. Maude Abbott states three lesions.
that in these cases cyanosis is entirely due There are no physical signs pathogno-
to capillary stasis but this explanation does manic of pulmonary stenosis. There are,
not appear very likely. No direct evidence however, important differences between
of capillary stasis has ever been presented pulmonary stenosis on one hand and the
in pulmonary stenosis without cardiac fail- tetralogy of Fallot and the Eisenmenger
ure nor have alterations in the velocity and complex on the other which may be utilized
volume of the circulation and in venous in the diagnosis. In pulmonary stenosis
pressure been demonstrated. An important with or without patency of the foramen
argument against such a view is the absence ovale the systolic murmur is heard in the
of cyanosis in patients with most marked majority of patients in the pulmonic area
pulmonary stenosis and cardiac hypertro- whereas with the other two lesions it is more
phy in this series, unless the foramen ovale often present along the lower left sternal
was patent. A critical review of patients border. This difference suggests very strongly
with closed foramen ovale shows that in the that the murmur of the tetralogy of Fallot
few considered cyanotic the evidence is un- originates in the interventricular septal
convincing. It is not unreasonable to suspect defect rather than at the site of pulmonary
one of three possibilities: (1) cyanosis de- stenosis or, at least, that this represents the
veloped with heart failure and therefore more prominent of the two components. As
was not different from terminal cyanosis of already mentioned no important informa-
other cardiac patients, (2) a patent foramen
tion could be gained from the description
ovale may have been overlooked at autopsy
of the intensity of the systolic murmur, its
and (3) fetal passages permitting right to
transmission or the presence of thrills.
left flow may have existed in the past but
However, the occasional mention of the
closed before death. In spite of the fact that
Abbott,2 Currens, Kinney and White,4 systolic murmur in the tetralogy of Fallot
Taussig3i and others have pointed to the being conducted to the clavicles and the
possible r61e of the foramen ovale as a vessels of the neck suggests the unproven
path for an intracardiac shunt this has not but interesting possibility that such trans-
been sufficiently emphasized, and the im- mission may be a sign of over-riding aorta
 Pulmonary Stenosis, Patent Foramen Ovale~~~Sel~~r rt (11.

;~ntl 111~1sclvidencr against the presence of duction disturbances arc iik(,ly lo i ndica t(>
pulmonary stenosis alone. interventricular septal defects.
Early diastolic murmurs occur most fre- Roentgenologic examination is by far tht
quently in the Eisenmenger complex at most important method in the differential
which point they unquestionably represent diagnosis of cyanotic congenital lesions.
relative pulmonary insufficiency which is The characteristic x-ray appearance of
also shown by the marked hilar pulsations. pulmonary stenosis is due to the post-
‘I’hcy occur less frequently in the various stenotic dilatation of the pulmonary arter)
forms of pulmonary stenosis when they in spite of which the lung fields are morp
indicate organic pulmonary regurgitation. radiolucent than normal. These dilated
Because of the anatomic type of the lesion, pulrnonary arterial shadows show dimin-
one might expect an even more frequent ished or absent pulsations. This is in con-
occurrence of diastolic murmurs in valvular trast to the small size of the pulmonary
pulmonary stenosis which accounts for the vessels in the tetralogy of Fallot with a con-
majority of patients with a closed inter- cave “waistline” of the heart, and the more
ventricular septum. prominent dilatation of the pulmonar>
The intensity of the second sound is of conus, artery and branches with intense
limited value in the differential diagnosis. pulmonary congestion and increased pul-
An accentuated second sound along the sations in the Eisenmenger complex. The
upper left sternal border most likely indi- characteristic appearance of the sabot-
catcs absence of pulmonary stenosis or, if shaped heart of the tetralogy of Fallot is
the latter is present, subvalvular rather than well known. Comparing pulmonary stenosis
valvular stenosis. In true valvular stenosis and the Eisenmenger complex with the
the second sound is often diminished or better established roentgen configurations of
absent in this area. This relationship, how- the non-cyanotic congenital lesion. the
ever, is disturbed by the fact that the second former resembles that of a patent ductus
heart sound at that location is often trans- arteriosus while the latter presents an
mitted from the aorta as pointed out by almost identical x-ray appearance with an
C:urrens et al.” and as shown in some of the atria1 septal defect. The striking uniformity
cases in our series. It was heard in some of the x-ray pictures presented in Table VI
patients in whom anatomic changes in the may be a coincidence. From the pathologic
valves made their closure appear impossible. analysis of the series one expects to find an
Electrocardiographic examination does occasional case of the tetralogy of Fallot
not offer any characteristic information with poststenotic dilatation of the pul-
about the type of the lesion for all lesions monary artery or a case of pulmonary
under discussion are associated with right stenosis with small pulmonary vessels. Oc-
axis deviation. Prominence of the P waves casional exceptions, however, do not weaken
is more common in pulmonary stenosis the importance of the characteristic x-ray
than in the tetralogy of Fallot and was not appearance of the various types of pul-
noted in the Eisenmenger syndrome. It may monary stenosis, especiallv since the dif-
ferences are well explained’by the anatomic
be noted that right bundle branch block
findings.
appeared in three cases of pulmonary steno-
The importance of realizing the fre-
sis and in none of the tetralogy of Fallot.
quency of the poststenotic dilatation of the
This suggests that it indicates a high degree
pulmonary artery and its effect upon the
of right ventricular hypertrophy rather than
radiologic appearance of the heart is em-
an organic defect of the interventricular phasized by the practical value of the x-ray
bundle which should be more likely to occur in selecting patients for the Rlalock-Taussig
in septal defects. Some caution has to be operation. Reliance upon the size of the
exercised therefore in assuming that con- large pulmonary branches in the selection
,“““‘KY, 1949
20 Pulmonary Stenosis, Patent Foramen Ovale--SeZzeer et al.
of patients for operation73 would eliminate of a lesion by one or both of these methods
most of those with pulmonary stenosis with can hc considered definite proof of corrrct-
intact interventricular septum. In spite of ness of the diagnosis.
the very large and dilated pulmonary The question of the prognosis of cyanotic
artery, the appearance of which might make cardiac disease cannot be answered with
the surgeon hesitant, one may expect pul- any degree of accuracy from a small series
of cases. It is important, however, to note
TABI.E VIII
that more patients die as a result of com-
SUMMARY OF DIFFERENTIAL DIAGNOSIS Ii\ VARIOUS FORMS
OF PULMONARY STENOSIS AND THE EISENMENGER COMPLEX plications than of cardiac failure. This may
be interpreted as showing the ability of the
IPulmonary
Stenosis
with
IPulmonar)
Stenosis
with Tetralogy
Eisen-
menger
heart to carry the burden of the structural
deformities for an amazingly long time. It
Closed Patent of Fallot
FOIa”le” FOra”le”
Complex
would follow that symptomatic treatment
Ovale OK3le
of the cyanosis, such as the Blalock-Taussig
I-I- operation, is worth while.
Cyanosis. ......... absent severe severe moderate
Cluhbine. ........ absent marked marked moderate Finally, the relative incidence of the
Polycyrhemia. absent marked marked moderate
Systolic murmurs. pulmonic pulmonic left sternal left sternal
three types of cyanotic cardiac lesion de-
area area border border serves some consideration. It is exceedingly
occasion- occasion- occasion- c”mm””
ally ally ally difficult to determine the frequency of
present present present
2d sound pulmonary various lesions for they are too rare for
area, often di-
minished
often di-
minished
variable loud
reliable statistics from any institution and
Electrocardiogram the calculations from the number of cases
Right ventricular
hypertrophy.. prominent prominent prominent mild reported in the literature are misleading.
P waves, tall , tall often tall not re-
Cases are usually reported only if they
markable
Roentgenogram present some unusual feature so that rare
Pulmonary artery. enlarged enlarged markedly
enlarged conditions are reported in greater number
Hilar shadows moderate moderate very large,
stze size pulsating than more usual ones. It seems probable
P”lmo”ary
absent absent severe
that pulmonary stenosis with patent fora-
congestion absent
men ovale is less common than the tetralogy
of Fallot and that it is more common than
monary stenosis with patent foramen ovale the Eisenmenger complex. The respective
to be aided by the operation as much as the incidences of these three main types of
tetralogy of Fallot.72 cyanotic congenital heart disease in adult
A summary of findings valuable in the life are unknown.
differential diagnosis of the three cyanotic
lesions and of pulmonary stenosis with
SUMMARY
closed foramen ovale is presented in Table
VIII. Information presented here is based on Two cases of pulmonary stenosis with
probability, indicating the features present intact interventricular septum and patency
in the majority of cases analyzed. of the foramen ovale are reported, with an
The question of how precise an identifi- analysis of twenty-seven additional autop-
cation of the lesion can be made by the use sied cases from the literature. This series is
of newer diagnostic methods, such as angio- compared with the reports of autopsied
cardiography and cardiac catheterization, cases of pulmonary stenosis with both septa
must be left open. These methods are of closed, of the tetralogy of Fallot and of the
great value and are becoming established as Eisenmenger syndrome.
most important diagnostic procedures in Pulmonary stenosis with patent foramen
congenital heart disease. Thus far, too few ovale is characterized by chronic cyanosis,
cases have been reported with autopsy con- polycythemia and clubbing. In the degree
firmation to know whether a demonstration of cyanosis it occupies an intermediate place
AMERICAN ,,O”RNA’. OF MEDICINF
 Pulmonary Stenosis, Patent Foramen Ovale--S&er et (IL. 21

between the tetralogy of Fallot and the 6. BISHOP, L. F., JR. and WALLACE: S. C. Pulmonary
stenosis with bacterial endocarditis in an adult.
Eisenmenger syndrome. Pulmonary stenosis
Am. Heart J., 5: 238, 1929.
with closed septa is essentially a non- 7. CL,ARKE, J. J. A case of ulcerative endocarditis
cyanotic lesion. associated with stenosis of the conus arteriosus and
affecting chiefly the pulmonary valve. with
On the basis of cyanosis, cases of pulmo-
ulceration of the main pulmonary artcry. 7-r.
nary stenosis withand without patency of the P&. Sk. Imdon. 44: 29, 1893.
foramcn ovale are placed in different classes 8. HERR: R. G. Stc.nosis of pulmonary artery; con-
genital heart disease. 7i. Path. .%c. 1mzrton. 41 :
of congenital heart disease. Otherwise these
57, 1890.
two diseases are clinically and pathologically 9. OGLE, J. W. Abnormal condition of the valve at the
very- similar and differ from the tetralogy of root of the pulmonary artery with consequent
hypertrophy of thr parietes of the right vrntriclt.
Fallot in important respects.
of the heart. rr. I’&. Sac.London.5: 69. 1854.
‘I‘hr most distinctive feature of pulmonary 10. PEACOCK. T. B. Contraction of the orifice. of the
stenosis with patent foramen ovale is the pulmonary artery from fusion of th(, valves. ‘11.
Path. SW. London. 10: 107, 1859.
x-ray appearance of the cardiac shadow
11. PEACOCK. T. B. Case of stenosis of thr pulmonar)
which is characterized by a poststenotic artery from disease of the valves. probably of
dilatation of the pulmonary artery and its congenital origin. 7-r. Path. .Soc. London, 30: 258,
1879.
branches. This again places the condition
12. ABBOTT, M. E., LEWIS, D. S. and BEATTII;. W. W.:
in an intermediate position between the Differential study of a case of pulmonary stenosis
small shadows of the pulmonary vessels in of inflammatory origin and two cases of (a) pul-
monary stenosis and (b) pulmonary atresia of
the tetralogy of Fallot and the very promi-
developmental origin with associated ventricular
nently dilated and congested pulmonary septal defect and death from paradoxical cerebral
vessels of the Eisenmenger syndrome. embolism. Am. J. hl. SC., 165: 636. 1923.
13. ANDREW, J. Congenital malformation of the pul-
Other clinical features and pathologic
monary valve. 7.r. Path. Sac. London. 16: 81. 1865.
findings of this lesion are discussed and 14. BALLET, G. Des abces du cerveau ronsecutifs a cer-
evidence is presented that it is a well defined taines malformations cardiaqurs. .,lrch. qbn. ‘T-/P
me%., 1: 659, 1880.
clinical entity with enough distinctive fea-
15. BARLOW, T. Congenital heart disease; pulmonary
turrs to make possible diagnosis during life. stenosis with dilated pulmonary artery above tht
Next to the tetralogy of Fallot it is the most stenosis. 7’~. Path. Sac. London, 30: 272. 1879.
16. CASSELL. Bed. klin. Wchnschr.. 28: 1221. 1891.
important congenital cardiac lesion in
17. FINI.AY: D. W. Malformation of thr heart; stenosis
adults with chronic cyanosis, polycythemia of the pulmonary valve, with dilatation of the
and clubbing. pulmonary artery and hypertrophy of the right
ventricle; patency of the foramcn oval? with
Pulmonary stenosis with patent foramen
cribriform opening in the septum of the auricle.
ovale represents a conspicuous exception to ?r. Path. Sac. London. 30: 262. 1879.
the rule that cyanotic congenital heart 18. GAINES. L. M. Stenosis of the pulmonary valve of
the heart with tuberculosis of the lungs as a
disease with dilated pulmonary arteries is
secondary and terminal complication. &z?loltp :%f.
unsuitable for surgical relief. I., 67: 228, 1913.
19. LAFITTE, A. R&rtcissement infundibulairc dr
l’arterc pulmonaire d’origine congenitale; ob-
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,.4Nt‘4KY. 1949