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Absence of the Pulmonary Valve

Report of Two Cases Associated with Other


Congenital Lesions*
LUCIEN CAMPEAU, M .D ., F .RC .P .(C), GRISLAINE GILBERT, M .D . and NICOLAS AERICHIDE, M .D .

Montreal, Canada

IIE COMPLETE, CONGENITAL absence of the right upper lobe compatible with bronchopneumonia

T pulmonary valve is rare . Only ten cases


proved at autopsy have been thus far reported,
were also described . The heart was enlarged and
the cardiothoracic ratio measured 9 .7 by 14 .7 em .
The pulmonary artery appeared greatly dilated and
and most were associated with other congenital
was described as pulsatile at fluoroscopy . The
malformations . The following is a description
pulmonary vasculature was otherwise normal . The
of two other instances of absence of the pulmo-
electrocardiogram (Fig . 2) showed right axis deviation,
nary valve, of which one was associated with right ventricular hypertrophy and an rsR' configura-
an infundibular pulmonic stenosis and a ven- tion in V 1 -V 2 . Routine laboratory tests were normal
tricular septal defect and the other with an except for a red blood cell count of 3,920,000 and a
aneurysm of an aortic sinus of Valsalva . white blood cell count of 16,300 .
Cardiac Catheterization and Angiocardiography : On
CASE REPORTS January 5, 1960, cardiac catheterization and angio-
CASE I. G . F ., a sixteen month old boy, was ad- cardiography were performed under anesthesia by
mitted to Maisonneuve Hospital on November 14, rectal Pentothal . The cardiac catheterization data
1959, for treatment of furunculosis . It was reported shown in Table 1 suggested pulmonary stenosis and
that cyanosis had been present during the first week a ventricular septal defect with a bidirectional shunt .
of life but had subsequently disappeared . He had The catheter was introduced from the right ventricle
been treated numerous times at another hospital into the aorta . The withdrawal pressure recording
for infections of the upper respiratory tract, and from the pulmonary artery to the right ventricle
attacks of dyspnca and cyanosis . In October 1958, (Fig . 3) showed a single gradient at the usual level
a left superior lobectomy had been performed at of the pulmonary valve, compatible with pulmonary
another hospital . valvular stenosis . A selective angiocardiogram (Fig .
Physical Examination : His weight was 15 pounds, 4) showed early visualization of the aorta . a ventric-
6 ounces . He appeared chronically ill and under- ular septal defect and a localized area of constriction
developed . There was slight cyanosis but no of the distal outflow tract suggesting pulmonary val-
clubbing . He had a pectus carinatum . Over- vular stenosis .
lying a left-sided thoracotomy scar were five small After the procedures, which seemed to have been
furuncles and a small subcutaneous abscess . Bron- well tolerated, the infant remained unconscious and
chial rates were disseminated over both lung fields . rapid breathing developed . Right-sided convulsions
The heart rhythm was regular at 110 per minute and appeared and he died twenty-four hours later without
the blood pressure was 100/64 mm . Hg . A to and having recovered consciousness .
fro murmur, grade 3-4, was heard maximally at Autopsy Findings : The heart was moderately en-
the left second and third intercostal spaces near the larged ; the weight was not obtained because the
sternum . A single second heart sound was audible lungs were not removed . Measurements of the
at the base, loudest over the second right intercostal pertinent structures are listed in Table it . The in-
space . The liver extended over II fingerbreadth below teratrial septum was intact . An infundibular
the right costal margin . No edema was present . stenosis was located at 8 mm . below the pulmonic
The roentgenogram of the thorax (Fig . 1) revealed ring . This consisted of a muscular band measuring
ostcolytic lesions of the left fifth rib compatible with 6 mm. in width and 1 cm . in thickness, covering two-
osteomyelitis . The left upper lung field showed thirds of the circumference over the posterior and
emphysematous changes . Lesions localized in the lateral walls . No semilunar valve was identified

" From the Institute of Cardiology of Montreal .

JULY 1961 113


114 Campeau et al .

Fin. 1 . Case 1 . Posteroanterior roentgenogram of the


thorax . Note the greatly dilated pulmonary artery .

(Fig . 5) . A thin, irregular rim of tissue extended


over the posterior wall and two excrescences of a
Fte . 2 . Case 1 . Electrocardiogram showing right ven-
few millimeters in diameter were found on the an-
tricular hypertrophy and an tsR' configuration in lead
terolateral walls at the level of the pulmonic ring . V I -V I,
Smooth thickenings of several millimeters were ob-
served below the pulmonic ring . A high ventricular FIG . 3 .Case 1 . Right heart catheterization . With-
septal defect and an overriding aorta were noted . drawal pressure recording from pulmonary artery to
The main and left pulmonary arteries were grossly right ventricle, showing a single gradient at the usual
level of the pulmonary valve .
dilated . The venae cavae and pulmonary veins were
normally connected . The tricuspid, mitral and
aortic valves were normal . The aortic arch was in a TABLE 11
normal position and the ductus arteriosus was Case 1, Measurement of Heart Structures at Autopsy

Measurement
TABLE I Structure (cm .)
Case 1 . Cardiac Catheterization Data
Pulmonic ring (diameter) 1 .2 (1 .12-a0 .1)*
Oxygen Aortic valve (diameter) 1 .1
Site of Catheter Pressure Saturation Right ventricular wall (thickness) 1
(mm . Hg) (per cent) Left ventricular wall (thickness) 0 .8
Interventricular septum (thickness) 1 .2
Ventricular septal defect (diam- 1
Superior vena cava 60 eter)
Inferior versa cava 60 Main pulmonary artery (circum- 8(3 .490 .4)*
Right atrium 3/0 54 tercnce)
Right ventricle, low 76/2 68 Right pulmonary artery (circum- 2 .5
Right ventricle, high 74/3 78 ference)
Main pulmonary artery 28/10 67 Left pulmonary artery (circum- 10
Right pulmonary artery 23,1 7 66 ference)
Left pulmonary artery 20/8 66 Aorta (circumference) 4
Right pulmonary artery 5/0
(wedge)
Aorta (through ventricu- 81/43 82 .5 ` Normal measurement fur a twelve to eighteen month
lar septa) defect) old child . From : GOULD, G . E. Pathology of the
Heart, p . 1064 . Charles C Thomas .'

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Absence of Pulmonary Valve 1 15

Fit . . 4 . Case 1 . Angiocardiogram, o seconds after injection into the right ventricle . Antero-
posterior (left) and lateral (right) views : a, right ventricle ; b, localized constriction suggesting
valvular stenosis ; c, aneurysmal pulmonary artery ; d, early filling of the aorta ; c, ventricular
septal defect ; f, left ventricle .

FIG . 5 . Case 1 . View of the opened right ventricle and pulmonary artery illustrat-
ing the following : a, thin rim of endocardial tissue ; b, small excrescences at the level
of the pulmonic ring ; c, smooth thickenings below the pulmonic ring . Inert shows the
view of the intact pulmonic orifice from above .

closed . The coronary arteries were normal, Other noted at the level of the pulmonic ring showed mvxo-
findings included an absent upper left lobe of the matous connective tissue (Fig 6) .
lung, passive congestion of the lungs and edema of
the brain . Comment : The antemortem diagnosis was
'The histologic examination of the excrescences valvular pulmonary stenosis and ventricular

Jtn.Y 1961

116 Campeau et al .

Flu. 6 . Case 1 . Left : Photomacrograph ( X4) of the origin of the pulmonary artery
and pulmonic ring section : a, pulmonary artery wall ; b, excrescence and thickening
at the level of the pulmonic ring ; c, myocardium . Right : High power magnification
(X200) of the excrescence showing the myxomatous connective tissue .

septal defect . The large pulsatile pulmonary intercostal spaces near the sternum . A to and fro
artery was explained, although not satisfac- murmur was heard with a maximum intensity at the
torily, on the basis of a post-stenotic dilatation second and third intercostal spaces to the left of the
sternum . The systolic component was widely trans-
and a left to right shunt . The diastolic murmur,
mitted to the anterior chest, neck and back . The
however, was puzzling . This patient was one
diastolic murmur was shorter and variable in inten-
of the five who died following cardiac cathe-
sity. This to and fro murmur was described as
terization, with or without selective angiocar-
continuous at times. The aortic second sound was
diography, in a series of 1,218 examinations of normal intensity but the pulmonic second sound
performed in this institution up to August 10, was absent . The rhythm was regular and the heart
1960 . rate was 120 per minute . The liver extended down-
CASE 2 . M . T ., a seven year old girl, was ad- ward 4 fingerbrcadths below the right costal margin .
mitted on March 19, 1958 . She seemed normal at Ascites and edema of the legs were present .
birth and during early infancy . At four years of age The roentgenogram of the chest (Fig . 7) showed gross
she began to show exertional dyspnea which did not cardiomegaly with a cardiothoracic ratio of 16 .5/19
progress until six months before admission . She cm . Both ventricles seemed enlarged . The lung
had an infection of the upper respiratory tract and vascularity was slightly increased . At fluoroscopy, the
subsequently complained of increasing orthopnca, pulmonary artery segment was pulsatile . The electro-
dyspnea and edema . She became and remained cardiogram (Fig . 8) showed right axis deviation, marked
bedridden . No cyanosis had been noticed . right ventricular hypertropy and an rsR' pattern
Physical examination revealed an acutely ill girl in VtV,. The routine laboratory tests were within
weighing 39 pounds . The lips and finger nail beds normal limits except for albuminuria and a hemo-
were pink but the feet, including the toe nails, had a globin of 12 .8 gm. percent .
cyanotic hue . There was no clubbing . The jugu- At right heart catheterization, a significant right ven-
lar veins were distended and the carotid arteries tricular-pulmonary artery pressure gradient was
were markedly pulsatile . The blood pressure at the recorded but due to technical difficulties a pullback
arms was 180/60-0 mm . Hg and at the thighs, 200/ pressure tracing was not obtained and the type of
80-40 . The thorax had a normal appearance and pulmonary stenosis was not established (Fig . 9) .
the lungs were clear to percussion and auscultation . The pressure in the pulmonary artery was 26/20
A systolic thrill was present over the second and third mm . Hg and the right ventricular pressure was

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Absence of Pulmonary Valve 117

Fm . 7. Case 2 . Posteroanterior roentgenogram of the


chest showing generalized cardiac enlargement .

170;-20 mm . Hg . The oxygen saturation values


were ostensibly normal except for a difference of Fin . 8. Case 2 . Electrocardiogram showing right
ventricular hypertrophy and an rsR' pattern in leads
0 .4 vol. per cent (Van Slyke) or 3 .5 per cent (cuvette
VI-V I .
oximetry) between the right ventricle and the pul-
tnonary artery, a step-up of doubtful significance . Fro . 9 . Close 2 . Right heart catheterization . Discon-
Because of the precarious condition of the patient, tinuous pressure record of the pulmonary artery (Left)
the procedure had to be discontinued before an and right ventricule (right) . The pulmonary artery
arterial oxygen saturation and pressure could be diastolic pressure is equal to the right ventricular end
diastolic pressure.
obtained . Angiocardiographic studies were not
performed for the same reason .
The diagnosis considered was pulmonary stenosis 11) . An incision, 5 mm . deep, in the infundibulum
with ventricular septal defect associated with aortic (made by the surgeon's instrument) extended
insufficiency . Because of a steadily downward through and slightly above this structure which had
course, a "salvage" operation was attempted but an been thus perforated . No pulmonie cusp could be
unresponsive cardiac arrest occurred soon after the identified (Fig . 10) . An endocardial thickening,
transventricular pulmonary valvotomy and dilatation possibly a rudimentary cusp, was noted over the
of the infundibulum, anterior wall (Fig . 10 and 12) . The pulmonary
Postmortem Examination : The heart showed evidence artery seemed smaller than normal, with a circum-
of a right ventriculotomy . It was not weighed be- ference of 2 cm . A high ventricular septal defect
cause the lungs were not removed from the specimen, measuring 2 cm . in its largest diameter was identi-
but it was grossly enlarged . Both venae cavae fied . The pulmonary veins drained into the left
drained normally . The foramen ovale was patent . atrium and the mitral valve was normal . The left
The tricuspid valve appeared normal . The right ventricle was dilated, and its walls measured 1 cm .
ventricle was dilated and its walls measured I em . in thickness . The aortic valve annulus measured
a t the greatest thickness. The infundibulum was not 2 cm . in diameter . The right and left sinuses of
stenosed ; at 1 cm . below the pulmonic ring it Valsalva were larger than normal (Fig . 13) and the
measured 1 .5 cm . in diameter. The pulmonary right and left cusps obstructed almost completely
orifice, however, was markedly constricted, meas- the ventricular septal defect (Fig . 10) . A slight
uring 8 mm, in diameter . It was partly obstructed pressure at the bottom of the sinuses caused the free
by a round structure projecting into the lumen at the margin of the left cusp to invert into the. left ventricle,
usual level of the semilunar valve . This was subse- suggesting a severe aortic regurgitation during life .
quently identified as an aneurysm of the left aortic A small fenestration of a few millimeters in length
sinus, measuring 8 by 5 by 4 mm . and located at the was observed at the margin of the right aortic cusp .
usual site of the right posterior cusp (Fig . 10 and The left aortic sinus communicated with the aneurysm

JULY 1961

118 Campeau et al .

Fro . 10 . Case 2 . Photographs of the opened pulmonary artery and right ventricle illustrating the following : a, absence
of pulmonary cusps ; b, aneurysm of the left aortic sinus ; c, thickening, possibly a rudimentary cusp ; d, right aortic
cusp ; e, left aortic cusp seen through the ventricular septal defect ; f. ventricular septal defect ; g, incision made by
the surgeon's instrument ; h . edges of the sectioned aneurysm (at the site of the incision made by the surgeon) ; i,
section removed fee histologic study. P .A . = origin of the pulmonary artery ; An = aorta ; R .V . = right ventricular
cavity .

FIG . 1 Diagrams illustrating the formation of the


semilunar valves by the partitioning of the truncus
arteriosus cummunis . Left (nomal) : The aortopul-
monary septum (1) divides the lateral endocardial
cushions (2) in two, giving rise with each intercalated
valvular swelling (3) to three pulmonic (4) and three
aortic (5) cusps . Right (Case 2) : It is postulated that
the septum (6) divided the truncus unequally, including
in the aortic trunk the posterior intercalated swelling (7)
and the two lateral cushions (S), resulting in three aortic
cusps, the left (9) and right (10) of abnormal size, and a Fro . 12 . Case
single cusp pulmonary valve, rudimentary (11) in this showing the following : a, large right (coronary) sinu
case . (12) Aneurysm of the left aortic sinus ; (13) b, large left (coronary) sinus ; c, posterior sinus of
fenestration at the margin of the right aortic cusp . normal size .

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Absence of Pulmonary Valve 119

Fin . 13 . Case 2 . Left : Photomacrograph (X4) of a vertical section through the


rudimentary cusp at the level of the pulmonic ring showing a, pulmonary artery wall ;
b, "rudimentary cusp" at the level of the pulmonie ring : c, myocardium . Ragkt:
Low power magnification (X80) of the "rudimentary cusp" showing dense con-
nective tissue .

previously described at a point near the left-right pressure and systemic systolic pressure (arm
commissure . The aorta was of normal size but was cuff) were equal, but the systemic and pul-
partially transposed, overriding both ventricles . monary resistances were unfortunately not
The remainder of the examination was not remarkable measured .
except for a passive congestion of the lungs, liver and
brain . CosalENTS

Comments: The diastolic murmur was most Review of the Literature : Thirteen instances of
likely caused by the pulmonary valve and the congenital absence of all pulmonic cusps have
-7
aortic valve insufficiency . The aortic valve been reported,' of which ten are cases
regurgitation appears to have been significant, proved at autopsy (Table ttt) . Ehrenhaft"
as suggested by the wide systemic pulse pressure . discussed the case of a fourteen year old boy in
The murmur had been described as continuous whom an absence of the pulmonary valve was
at times and the possibility of a spontaneous found at surgery . Although he believed that
rupture of the aneurysm of the aortic sinus into it had been destroyed by subacute bacterial
the right ventricular outflow tract was raised . endocarditis or rheumatic fever, it may have
The absence of a left to right shunt at cardiac been aplastic . There was not postmortem
catheterization and the anatomic findings pre- confirmation . Miller and associates 6 described
viously described have convinced us that the a clinical and pathologic syndrome in six
perforation was made by the surgeon's instru- children who presented cyanosis, a to and fro
ment during the transventricular valvotomy . heart murmur, pulsating aneurysmal pulmo-
In spite of the constriction of the pulmonic ring nary arteries, a bidirectional shunt at the ven-
or orificial pulmonary stenosis and the obstruc- tricular level and an inundibular pulmonary
tion to flow produced by the aortic sinus aneu- stenosis . Four of these patients have died
rysm, this patient was not cyanotic. The large and postmortem examination revealed an in-
aortic cusps partially closed the ventricular fundibular pulmonary stenosis, a ventricular
septal defect (Fig . 10), but we postulate that septal defect, an overriding aorta and absence of
the systemic hypertension resulting from the aor- any true pulmonary valve . The other two were
tic insufficiency prevented a significant right to presumed to have similar malformations, in-
left shunt . In fact, the right ventricular systolic cluding the absence of a pulmonary valve .

JULY 1961
120 Campeau et al .

Pathology and Embryology : This anomaly was has also been attributed by Roberts and Loubelt
associated with a ventricular septal defect in to a misdirection of the aortic septum with
thirteen of the fifteen cases reported thus far, crowding together of the coronary anlagen .
including the two cases described herein . The association of large aortic cusps with an
Ehrenhaft's patient' was reported to have an absence of pulmonary cusps again suggests an
isolated absence of the pulmonary valve but no unequal division of the truncus arteriosus
postmortem confirmation was obtained . Case (Fig . 11) . In our Case 2, the anterior pul-
2 of Smith and associates' also had this anom- monary cusp should have been present if this
aly as an isolated malformation . In addition mechanism were responsible, but the endo-
to the ventricular septal defect, eight had an cardial thickening at that site may represent a
infundibular pulmonary stenosis, and all of rudimentary cusp . The formation of the aneu-
these except one also had an overriding aorta . rysm of the aortic sinus, on the other hand, is
(tetralogy of Fallot) . Three had a constricted undoubtedly explained on another basis .
pulmonic annulus or an orificial stenosis, but
without identifiable valve tissue, a distinction HEMODYNAMIC AND CLINICAL SIGNIFICANCE

which separates it from a true valvular steno- Absence of the pulmonary valve is necessarily
sis. Only two cases2 ' 4 did not have a pul- accompanied by severe pulmonary valve regur-
monary stenosis of any type in combination gitation . Isolated pulmonary valvular insuf-
with the ventricular septal defect . Finally, ficiency appears to be well tolerated in the ex-
one case also had an associated single coronary perimental animal's -14 and in humans .ts -19
artery and another, an aneurysm of an aortic Although significant hemodynamic derange-
sinus of Valsalva (Table in) . ments and cardiac enlargement resulted, conges-
The congenital nature of this anomaly is not tive failure could not be induced in dogs by
proved . The absence of histologic evidence complete excision of the pulmonary valve .'-
of inflammation or destruction, the rarity of One of the patients with an isolated complete
acquired pulmonary valvular disease, and the absence of the pulmonary valve appeared to
frequent association of other congenital mal- have been only moderately handicapped .'
formations favor this assumption . All these He complained, however, of shortness of
cases had no pulmonary cusp that could be breath from four years of age and had a mar-
identified as such (Table in) . Some had a kedly enlarged heart. The other patient'
complete absence of valvular tissue, and others with isolated absence of the pulmonary valve
had a thin fold, a fibrous hand, smooth or ir- was believed to have congestive heart failure
regular thickenings at the level of the pulmonic in utero . This relatively good tolerance in post-
ring . Case 2 of Smith et al . 7 had short irregu- fetal life may be explained by the low pressure
lar and fused leaflets, the only case with identi- system of the right side of the heart . As
fiable leaflets . The histologic studies revealed suggested by Smith et al ., 7 this anomaly is of
fibrous tissue," avascular loose connective greater consequence in uteru because the pul-
tissue, 7 myxomatous connective tissue (Case I) monary artery pressures are equal to the sys-
and dense connective tissue (Case 2) . These temic pressures and a free communication of the
considerations suggest that this anomaly in systemic and pulmonary arterial systems through
some cases represents an hypoplasia and in the patent ductus arteriosus results in an
others, a true agenesis of some or of all the pul- aortic run-off into the pulmonary artery, thus
Inonic cusps . increasing the pulmonic regurgitation . We
Simmonds' explained the reduction of pul- presume that when pulmonary hypertension or
monary valve cusps, particularly of bicuspid a large left to right shunt is added, the regurgi-
valves, by a maldirection of the aortic septum, tation is similarly increased and the right ven-
which has improperly divided the endocardial tricular overload may be less well tolerated .
cushions, the precursors of the cusps . Ventricu- However, the two patients with a large associ-
lar septal defect, overriding aorta and pulmo- ated ventricular septal defect became signifi-
nary stenosis, anomalies commonly associated cantly incapacitated only during the last few
with agenesis of the pulmonary valve and with years, and they lived to the age of thirty-two and
bicuspid pulmonary valve,' may also result from forty-six years, respectively 2,4 On the other
an abnormal position of the aortic septum .'O A hand, an associated pulmonary stenosis may
single coronary ostium, an anomaly observed minimize this regurgitation by reducing the
in one of the patients with no pulmonary valve, 4 pulmonary blood flow .

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TABLE III
Reported Cases of Absence of Pulmonary Valve . Associated Malformations

Authors Sex and Age Findings at Site of Right Ventricle Pulmonic Pulmonary Ventricula (
(years) i Pulmonary Valve Infundibulum Ring Artery Septum Aorta Other Structures

Roger and M, 6'/s Absence of valve Localized stenosis Constricted Small High defect Transposed Transposed atria (situ!
Wilson' (?) (3 -8 mm.) (1 .5 cm .) inversus) ; atrial sep.
tal defect, mitral by-
poplasia and atrophic
left ventricle
Lavennes M, 46 Thin fold of tissue Thin fold over Dilated High defect Overriding Small fenestration of
et al .2 posterior third, (1 .8-0 .8 the noncoronary
without true cm .) aortic cusp
stenosis
Ehrenhaft'* M, 14 No leaflets Dilated .. ..
Campeau M, 32 Fibrous rim 2 cm. in No stenosis Not constricted Aneurysmal High defect Small Single coronary artery
et al .4 length (2 .5 cm .)
Onesti and M, 9 weeks Small irregular thicken- No stenosis Mild constriction Dilated High defect Overriding Patent foramen ovale
Harneds ings of fibrous tissue (1 .5 cm . cir-
cumference)
Miller Children (6 Absence of valve Stenosis Dilated Defect Overriding
et als cases)}
Smith M, 4'/, No valvular tissue Moderate stenosis I Mild constriction Dilated High defect Normal
et al .r
M, pre- Irregular, short and ad- Normal Normal Dilated Normal Small Patent foramen ovate
mature herent leaflets and ductus arteriosu :
Case 1 M, 1'/, Two small excrescences, Localized stenosis No constriction Dilated High defect Overriding
smooth thickenings .)
(1 em
Case 2 F, 7 Single "rudimentary Normal Constricted Smaller High defect Overriding "Aneurysmal' dilata-
cusp," aneurysm of (2 .3 cm . cir- than (2 cm .) lion of two aortic
aortic sinus cumference) normal sinuses of Valsalva

* Direct surgery observation, No autopsy.


} Two cases without autopsy confirmation .

122 Campeau et at .

The diagnosis of isolated organic pulmonary fundibular stenosis had these findings as well .
has been recently reviewed .'' - so
valve regurgitation A right bundle branch block or an rsR' pat-
A short gap between the pulmonic second heart tern in V,-V, in addition to the right ventricular
sound and the onset of the diastolic murmur is hypertrophy was reported in five' " of the seven
frequently observed . A systolic click is not cases with a description of the electrocardiogram
infrequent . Of interest is the finding of a (two cases reported herein) . The patients of La-
diastolic thrill and rumble over the tricuspid or vennes and associate
the mitral area, which according to Segel et had a qR in V, but an rsR' in V s , which can
al .'s may be the equivalent of the Austin Flint possibly be interpreted as right bundle branch
murmur of aortic regurgitation . The electro- block with right atrial and right ventricular
cardiogram usually shows a right ventricular hypertrophy .T' This bundle branch block is
diastolic overload pattern or right bundle probably due to the right ventricular diastolic
branch block with or without right ven- overload resulting from the pulmonary valve
tricular hypertrophy . At fluoroscopy, the pul- regurgitation .
monary arteries are dilated and abnormally The pulmonary artery pressure was described
pulsatile, falsely suggesting a left to right shunt . in only three cases . Ehrenhaft's' patient had a
At cardiac catheterization, the pulmonary pulmonary artery pressure of 27/10 mm . Hg
artery pressure curve may suggest the diagnosis . with a right ventricular pressure of 50/7 mm .
The most significant findings are a diastolic Hg . Another case" had a pulmonary artery
pressure approaching the right ventricular end pressure of 180/23-28 with a right ventricular
diastolic pressure and a less prevalent or absent pressure of 180/0 mm . Hg . Finally the pa-
incisura . The pulmonary artery and right tient described by Smith et al .' had a pulmonary
ventricular pressure patterns become almost artery pressure of 44/33 with a right ventricular
similar in cases of severe regurgitation . Segel pressure of 75/ 2 mm . Hg . None of these cases,
and associates" described an early systolic nega- except possibly Ehrenhaft's case, had a pulmo-
tive wave believed to lie a Venturi effect and nary artery diastolic pressure equal to or
not unlike the pattern observed by Wiggers approaching right ventricular diastolic pres-
in aortic insufficiency. A pressure gradient of sure, a "pathognomonie" sign of pulmonary
10 to 30 mm . Hg may also he observed due to valve regurgitation . No explanation can be
an increased flow through the pulmonic orifice . offered for this discrepancy . In our Case 1,
More recently, diagnostic methods with the aid none of the previously described signs of pul-
of dye dilution technicss" and angiocardiogra- monary valve regurgitation were observed (Fig .
phy" have been described . 3), whereas in Case 2, the pulmonary artery
Clinical Findings : Loud systolic and diastolic diastolic pressure was equal to the rightt ven-
murmurs over the left side of the sternum tricular end diastolic pressure (Fig . 9) .
were described in all cases of absence of the Combined Absence of Pulmonary halve and
pulmonary valve, except in Case 2 of Smith Tetralogy of Fallot : Absence of the pulmonary
and associates (auscultatory findings not given) . valve confuses the diagnosis of the anomalies
The systolic murmur was due to associated with which it is associated, and which are usually
anomalies except in Ehrenhaft's case in which recognized otherwise without difficulty, such as
no other malformation was described . The the tetralogy of Fallot. We suggest, as do Miller
pulmonic second sound was inaudible' 4 A , ' et al .' that the combination of this anomaly
(our Case 2), or a single second sound was and the malformations of the tetralogy of Fal-
heard over the pulmonic area 2 (our Case 1) . lot results in a characteristic clinical syndrome .
This single second sound was noted in patients Cyanosis, systolic and diastolic murmurs, an
with an overriding aorta and was presumably aneurysmal pulmonary artery, a right ventric-
transmitted from the aortic valve because of the ular-pulmonary artery pressure gradient, and a
abnormal position of the aortic orifice . The bi-directional shunt at the ventricular level were
second pulmonic sound was not mentioned by observed in the six cases of Miller et al ., in Case
Roger and Wilson' and by Millers and associates 1 of Smith et al.' and in our Case 1 . Smith
in the description of their cases . The pul- and associates' patient, however, had cyanosis
monary artery was markedly dilated and pulsa- only in early infancy and the murmur was
tile in all cases except in two' (our Case 2) . described as continuous instead of "to and fro ."
These two patients had severe constriction of This was the only case without an overriding
the pulmonary orifice but the patients with in- aorta . We add to the description of Miller

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Absence of Pulmonary Valve 123

and associates," partial right bundle branch rysmal pulmonary artery, a right ventricular-
block associated with right ventricular hyper- pulmonary arterial pressure gradient, and a
trophy, which is rarely observed in a true bidirectional shunt at the ventricular level .
tetralogy of Fallot . Not a single case of right It is suggested that the combination of ab-
bundle branch block has been found in a series sence of the pulmonary cusps and abnormally
of sixty-three cases of tetralogy of Fallota3 and large aortic cusps favors the hypothesis of an
we have observed an rsR' pattern in V, or in unequal division of the truncus arteriosus as
V 2 in only two tracings of fifty patients with a the embryologic error responsible for the reduc-
diagnosis of tetralogy of Fallot . Of these tion of pulmonary cusps in some cases .
two patients, one had an associated bicuspid
ACKNOWLEDGMENT
pulmonary valve and the other had pulmonary
We acknowledge, with appreciation, the criticisms
fibrosis due to healed tuberculosis . The pres-
of Dr . Paul David, Director of the Institute of Cardi-
ence of a normal or loud single second sound ology. We are indebted to Dr . Osman Gialloreto,
at the pulmonic area should not rule out pul- Chief of the Hemodynamic laboratory, to Dr- Louis
monary regurgitation, because in tetralogy Philip Legresley, Chief of the Pathology Department,
of Fallot the aortic valve closure sound is usually and D, Dr. tales I .aherge, Chief of the Radiology
Department, Institute of Cardiology and Maisonneuve
heard over the second left intercostal space . Hospital . We also thank Mr . Jean Gauthier and Miss
Organic pulmonary valve regurgitation could Jacqueline Dupuis of the Medical Illustration Depart-
have been suspected in our Case 1 and dye ment for their collaboration .
dilution with a double lumen catheter or aa REFERENCE-
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124 Campeau et al.

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