Pulmonic Stenosis, Ventricular Septal Defect,
and Right Ventricular Pressure above Systemic Level
By J. I. E. HOFFMAN, M.D., ABRAHAM M. RUDOLPH, MI.D.,
ALEXANDER S. NADAS, M.D., AND ROBERT E. GROSS, M.D.
IT IS USUALLY possible to distinguish
pulmonic stenosis with an intact ventric-
ular septum (designated as pulmonic stenosis)
from pulmonic stenosis with a right-to-left
shunt through a ventricular septal defect (te-
tralogy of Fallot) by routine clinical, electro-
cardiographic, and radiologic examination.1-3
If the diagnosis is still uncertain, it can as a
rule be made by phonocardiography and car-
diae catheterization,4 particularly if there is
very severe pulmonic stenosis with an intact
ventricular septum. Characteristically in se-
vere pulmonic stenosis the right ventricular
systolic pressure is much higher than in the
systemic circulation and rises still higher after
ectopic beats; the right ventricular pressure
tracing is triangular; and right ventricular
systole is prolonged so that on phonocardiog-
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raphy the second heart sound is widely split
and the systolic murmur passes beyond aortic
valve closure. Unfortunately these commonly
accepted criteria may be unreliable. In this
paper 10 patients are reported who, although
meeting most or all of these criteria for the
diagnosis of severe pulmonic stenosis (espe-
cially with systolic pressures much higher in
the right ventricle than in the systemic ar-
teries), nevertheless were proved later to have
an associated ventricular septal defect with a
right-to-left shunt. An attempt is made to in-
dicate how even under these circumstances a
correct preoperative diagnosis may be made.
From the Sharon Cardiovascular Unit, Children 's
Medical Center and the Departnient of Pediatrics,
Harvard Medical School, Boston, Mass.
Supported in part by a grant (H-2515) from the
National Institutes of Health, U. S. Public Health
Service, a grant-in-aid from the American Heart
Association, and the Massachusetts Heart Associationi.
Dr. Rudolph is an Established Investigator of the
American Heart Association.
Circulation, Volume XXII, September 1960
Material and Methods
These 10 patients were admitted to the Chil-
dren's Medical Center for investigation and oper-
ation, and all but 1 were seen after June 1957.
Cardiac catheterizations as described previously
from this laboratory5 were done in all, and phono-
cardiogranms were taken with a Sanborn Twin-
Beam photographic recorder.
Results
Clinieally all of these patients had onie or
more signs suggestive of valvular pulmonic
stenosis with an intact ventricular septunm.
There were large "a" waves in the jugular
venous pulse in 4; the systolic thrill in 7 anid
the stenotic systolic murmur in 8 were maxi-
inal at the second or third left intercostal
space; the second heart sound was thought to
be widely split in 4; and 2 with elinically se-
vere pulmonic stenosis had no eyanosis. The
electrocardiogram showed P pulmonale in 6
and muarked right ventricular hypertrophy in
all 10 the R waves in lead V, were 20 to 30
mm. high in a and over 30 mm. high in 4. On
x-ray, 8 had cardiae enlargement (marked in
4) and 4 had what was initerpreted as post-
stenotic dilatation of the pulmonary artery.
Any of these signls may be found in an other-
wise classical tetralogy of Fallot but all of
them individually or together favor severe
valvular pulmonic stenosis with an intact ven-
tricular septum and high right atrial and
ventricular pressures.
Cardiac catheterization supported the diag-
nosis initially when right atrial pressures over
10 mm. Hg were found in 7, and in all 10 the
right ventricular systolic pressure was above
systemic levels. In 1 the right ventricular sys-
tolie pressure of 125 mm. Hg was only 15 mm.
Hg above that in the brachial artery, but in
all the others it ranged from 150 to 250 mm.
Hg, with a systolic gradient between right
405
 46 4IJOFFMJAN, RDI)()LP,ll NADAS, C'-ROSS

PLMWtC Srum TETRALOGY OF FALLOT

ATYPCAL: TETRALOGY OF FA.OT

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Figure 1
Right ventriculaur prnssunrc curtvs 'thi slinul/nacomtt o/i sprf,,,4,surc rlUrrcs (top) avd
br-achial arcfryi pressure cutr i-c (bottom).

venitriele arid systemiciartery of 35 to 120 nim.
1ig. An iIntact ventriclular septum was furtlher
suggested wheni in 7 the r ight ventricular sys-
tolic pressure rose 15 to 40 nun. lfg Wabove its
previous level after a premature leat; in the
othier 3 right ventricular prIessiur-es werie niot
recorded during premature beats.
The siape of the rihlt ventriCLalrt1 prcss1uri-
cUrVe was rouIgh'ly triaiigular (fig. 1) as foUnld
in pulmotnie steniosis but n1ot in tetralogY of
Fallot. In 6 the ratio of the curve width 20
per cenit above the inidsystolie pressuire to the
width 20 per cenit below it was smnall anld well
under 0.70, the lower l:imit of the ranlge estab-
lished for tetralogy of Fallot;4 ill the other 4
the value of the ratio fell in the zone commnonr
to both groups.
Despite this cumulative evidence of severe
p)U1linoitiC stenosis atll 10 patienits had venttric-
lar septal defects witlh right-to-left shunts.
This was provedi preo)peratively itn 2 by pass-
iiug the, catheteri fromi righit v (litriele to aorta
throulgh tle defeect. T4i thlese 2 as well as iii
.) other patienits, inlicator-dilution curves
sshoweTd a righIA-to-left shulint at the ventricular
level; ini 2 of these ttie shun-ft was shown by
inijeetions made iinto the bod3 of time right vei-
triele but nlot into the infundibuluni. TIdica-
tor-dilutioii curves were also donie irn 2 of the
remainingg 3 patients aiilI sliowedl a riglht-to-
left slhunt at the atrial buit not at the veuitric-
ular le-vel. The magnitude of these right-to-left
shunts varied widlely, so that systemic arterial
oxyge.n saturation raniged froimi 47 to 93 per
cent; in 6 of the 1.0 it was betwveen 80 and 89
ter cenit.
Circulation, Volume XXII, September 960
 I IjAINIC
TSTEN SS 47
407

RV mnt
i
RV body LV
rigure 2
Elect 10(0 rtio'7,rams(t(o)p), ext{er nal phonocarclworams (eenter), and pressure curees
(b4tt-oiti) to show that thc notch1 in the (tsr nth limb 0! the rigiht uentricular body
prA'r.sSaTu Carfer Ociafccr (t thU be',ld rt' the sq/t4oli(,hpr ss.a in tie right rentricalar inflan-
dtibnld or thU 1(- ft rtaftrich XVotr' als,O thc eaulgJ dia(mond-slhaped mai'mar.

T[here was a notchl or aibrup)t chiange in di- anld 8 bad fourth he,-art soulnds of sliglht to
r ectioi of the, ase.ndling limib of thie right VenI- marked inrteniisity.
tricular pi1essure cur-ve mn l; thlis inotct alwvays Ident-fleat ion of the 2 components of the
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oeccurred about the, level of the. svstemiiie svs-
tolie pressure, aIndl wvas also near tite systolie
Pr essure ill thie infundfibular chamnber in. 3
(figt. 2).
At; cardiae eatliet erizat ilol the site of the
steniosis wvas ktdgeId to be (lefinitely valvular
a8ll(;ifliiitliluular in. 3, valvular and(I possibly
infundibular in 3, anI( i ifundibilar alone ill
2' itn. 2 the pulinoii,ay arter.1-y was ntlt entertledt
l)lt einealnni'ooram in I siowed atn iiiftndlibu-
Jar ehamtber. Four shiowed a zone of negative
pressiure as thie eathieter ti) passed fr1omii the
pulniinay ar.tery to atid(i through the stenotie
a'rea (BernlOntil Iielet) an(d I of these was a
?atielit wN"ithL pureI'0 inIfuuibiilaclr StenToSiS.
Plhonoeardiograims were t-aken in all these
)atients (fig. 3) Thie first- hleart so80111( w-as
normn-ally
1 split in all calld was eitlher normal or
rXeduleced in intensity. Five biadl a swstolie eliek
P.US to 0.14 secund after the beginn1-in:fg of tle
first lheart sound ; in 3 this eliek vas otloder.-
int expirationi itt I it incr-eased (iulling intsJ)irat-
tion atd in 1 dlid no-t chlan}ge in intensity withl
r-espiration. Four hadsof(Jt thiird lhea-rt sounds
Circulation, Volume AXX', Sept(cnbcr 1960
seeonid lheart somnd may be diffieult if pul-
miioinie closuilrc is -very soft antd is obscured by
the systolic murntur passing beyond the aortic
(losingr soulld and-( if the absen'ce, of a suiitable
external refere-nice makces it difficult to be sure
thlat a smial. vbtion
l dloes inideed represent
l)ulmooiie closure. Iln 6 the seeond heart souind
was regarde(ld as, sintle or narrowly split (up
to 0.03 seconitl) 111id in 4 it was probably wide-
ly split (0.06 to 0.10 seconid).
rElie systolic( niniuriiiiii was dliamond-shiaied
in 9 anld decrescenlo in 1I but hadl many varia-
tionis in conifiguratilon. tIn all it began imme-
diately or shortly after the first heart sound,
which was alway-s distinct, and it begran before
tile svstolie elick ini tile 5 whio had it. Iin the
ml-ost severelv affected patient there was a soft
lecereseneado systolit imurmur enrlitig at or be-
fore tile single seconid heart sounid (fig. 3C).
In 3 thie systoli c mnurmur inervaset';l ralpidly to
a mnatxinrnu ini thle fitst quarter of systole, e-
mauited at thiis intensity for a sihort timtie, anid
tfll-n (ecreased(1 to eltd at or beyond the secoind
Ilert souutdtl (fig. 3-)-F). Four othfers (fig. 3(-0
 408S 4I1I/'FMAN. RIDOLPIlI, NAI)AM, (Il)RSS

positioll of thie thrill aHid in at least 8 y at

indenltation in the right -venitricular outflow

I trliact somyie dlistaeiee below thie pilltlhoIia >
valve; in addition there}wer e 4 w-ithi valvuilarl
steniosis an(d thrilis in the pulitiloiarv artery.
One patienit had a correeted transpositionnwith
the right ventriele aiteriorly anid oii the ( pa-
tient s ) left atndl the pulilonar>r artery ellnerg-
wii'g to the right of an(] poster ior- to the ;aortai.
'I'lis patient hladi whait was alinost a sing-le
ventriteile wt the lower tliree quailters of thIe
ventricula141<r sep)tIumn1 m1is.sig11(1 a wdwithl -mart ke(l
1i111i(iselar1 Ihvpertrophy b)el()0Xow the pIllnon1ar>v
aan.d aortie. valves. Alt th.e othiers iad(1 higl
Inelni/ira nou,s venit etio lar stl;eptal defteet s; one ot
thlese was reeor:ded as being '`qiqite small,' 8
1l.lj(:ha ra
t\ of05tI 1 .( (,1l 1.21 andl44 hadi
(f 0.;-) to 10tn- 1le areas
1
of 1.' to 29.0 (,III.2
Discussion
In 1951 Sol ie, Joly, (a1arlotti, and S>ol t
tdeserib)ed rigidvet velltri-icla i systol ie j)pissutres
above systemni(e- levels
' in 3 of 22 patientsx witl
tetralogxv of Fallot. MeGoon ami -(1 Kirklin III
repor,ting 32 patients with ''an essen-tiaillt y in-
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taet vxen.trieillar septum,n,'' mnentioned 1 patieiit

with a ventri-icul:ar sept:al defect and an ahnor-
Figure 3 niallv higlh righlit vontrienilar pressure. In our
I1ho oe,oardiot;rt /s. A:. '1 /])pictt pu1 10//it StI 1/05/s. series 9! of 10 patient;s were seen in. a period
1B. h'1 it ftt/e l f icy/ of Fu/lot. (2 Pa/titit/s 11/ wlhen tahoit 235) patients with pihilmonie stenosis
ti/is /iis. ,21 IS, secondti left i//it/S
st 2 1 ost
)f l ptac ; al'li about 35 with tetralogy of Fallot were
tI1S, fourth it f i/te /cstasil sp)t (5'; Sj, first heart
SO/f1 /l; S s /ti bc/irt so/f/lti; S
eo/ /of /rth heartf
catheterized. In the same period soine with
so/lnf/t; Sil,M //slfttoliti C1//f//o; S(, sytolic cHitck. tetralogy of Fallot and a fewr writh puflmonie
stenosis were operated on withouit cardiac
) iamove typilc-al diaimi(i1-shiape( systolic
catheterization and many others in both
mnI rnulIs iievertlmeIls(I s were: mnaximiial
that groups were seen hult niot. operated on. There
earlv in sv stole, so thalt t hex resembled kit e a
mia- be still rmore patients with this syndrome
Iying onri its side wvithI its tail poil/.thing toward whio have been diagiiosed as piilmoniie stenosis
the 2 of tfi/sc Iurimlri/rs enlded at, and and(] inxwhoin the error has not yet been this-
2 enledl afterr thlie semolld hteart so/ntid. Finlallvy
covered, but even witlhouit themn this varianit
2 hadl sylni/eti ial (liamond-shiaped systolic of fetralogy of- Fallol seemns to he relatively
1iiirs withl their maxima iii. miidsvstole, to/lu/mon.
I/lIt 1 elded well before the seeond heart soliud Physiologic Mechanism
ali dtfeatolcr at or inst 1/cyomd it (figr. 3K and(l Tn tetralogv of Fallot ther-e is us-uially rapidl
L); in ea/li, sf/I/I ot)mi)le\es di liave early pl ressu, e e quiTlibrium across the ventricular
dliInlolth-shapl)cd miunurirs. septal defect so that systolic pressures ini the
A.natoimi(c details were not (-.td at open-heart right aiid left ventricles anid aorta are similar
(oper,ation in 9. All had inifumalibular stenosis, anid rise slightly or not at all after ectopie
NvI hie wvas suggrested 'b> the low intravalvullar becats, rixght anid le]ft ventricular pressure
Circulation, Volunme XXIi, Scptvmber 196(/
 1IL NIC STrENOS-4(S 409t

tt-iriit(gs liave similar shapes vith almost par-

allel sides and flit or romute(l tops, amid( vel-
trienlar ejeetimn etitis at alIGot the samhfe tie
ill two ventl'ieldes. Conseqenpetly the secon(d
lheailrt soit is single oi- itarrol x Split allnd the
svystolie minr mi. d(oes tot imass beyond aortie
aT.lVe clo(sujre.4 Byvcotrast, all 10 patietits
reporXted herel^ aet:\eil as if thle velitrietilat1<4 sep- RVSD
tint wEere itttaet, int that the tight Ve1tliele
coll(1 gleIletr ate a-I sy-stolie press-ure hig-her- tha.-it i.RV
tliat; ill the svstetoie eil-au!lsItiott attd (ottld fill-t
tfiter elevtte tlhis aftet- eetop)i beats. lit a o-oup
of patientits with tetr-aloyrv of Fa0lot stuIdlied inl
tlis laborator y, 30 m.m. 1g wvas about the
ittaxoitto-ll.l (differ,ettec founi.d betwN-een right veti-
trielalm-r atdtsvstlenie aruterial svstolie pies
slilels Whenl sVstolie p-ressures ill the r-igTht veIn-
triele andl aiorta wer,e alm.ost identicall, so that
(lifferett(ees grn.eater than this probably. ilndicate
some. failtre of pr essre equilibraitioll h)et7e,et(t
the twio ventr-icles. In the onie patienit wjT i tt a
systolie radlient of onily 15 niiin. ITg betweetn
righolit ventriele atid systemie artery evidenee Figure 4
of ani intalct septiui was inferred fronti a 25" kigh l sholviny
tea/toitle .NIittJ tUa//nt//li/'js(i / o1/
mino. JTi rise in right ventricular systolie Pres' ttiettri)uip 1711,f tell t tfrt/
/ the vt/l/Qit/ aJ the
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sore after ectopie beats and fromii the (lenioii-
stratiott of a widlely split second heart souttd
wvitlh the, systolic inurntuir eniding after aortie
vaIlve closure. Tli this patient, too, as well as
itt 5 others, tlte shape of the riglht vetitrienairM
PreSSutre curveswas typical of pulinonic ste
ttosis.
Tltis failure of ral)id pressure equilib)rationi
aeros-Ss tlte veittrieular septal defet suiggests
that thie vetiticlar selittm mav be futlctioll-
allyv initatt dliruing ialt- or all of sy-stole anl.d
thl(e following J)ossihle reasons based ott oilr
experi cie witlth these 1T patients mayv be ad-
need
1. The defeet tayv be occludled by the septal
IWa/flet of the tricuspid valve. In one patienit
at autopsy this leaflet had ain anomalotis 1ri-
gint frioi thte i-imarginl of the ventricular septal
(lefeet (fi(g. 4) aid couldl eoneeivalyfir hfazerve ob-
strtitetl it (lutiiit systole. Inl anlother patient
at opteratioi the tricuspid val.vAe lea Ifflet mwas
folnldl to he 1p)artly adherent to the rimn of the
defeet.
Circuelation, Volumac XXII, September 11960
te't/ict?/11/1 se.T}t/ deftect. 1A', riyht atrium; I/,V
riuhit rottic/e; V1 SD- I, retric u/ar septti d/otft.
2. 1If thte ieifet is ver> sttttllt1lttt pllt¾ess-iit
eqitililbration011 ass it eoitld he dIelaved. Soui
attd htis lolleagtles" ii iroke(d tltis exlattatiot
ill pattieiits Nh itll tlissvtltdrolltte whto tad ittillt
iotal right1t-o-left Shtittts attld 2leCord, vatl
Elk, attd l lointt fonitt(d a. d e tect ittitli. iII di-
tIteterittl thtell patiellt . 4 ltI 2 pal ieltts Ili
MITt1- se11ries had small defects, thottgllt 1 ot(d I1ts
had att arterial oxygen s,attiratioti (If l 82 1 I'
3. If thIte lefe(et is siftl atedl itt 1t t it i1tt 'li
lilitr e1alltaber, it illtav become isolafed fromtt
tlte lhodv of the irigylht vent,ricle dnrittg. systo!c
aIJtd thits maIy itIot he attIc to deeon press it.
TIhltis may have oe(eiirredl in 1 patient CretPoted
bysl,<AlcGloon 81(t J\;-jllsli7
Aitj(j(})l an b Irkit J)Ut u IVf1S
a 11(t-tt 'vll
ot
itt o01r series.
4. It is possiile that the vettrttietlat selptal
defec t eould be ocel toted by ilt-raltioln of
tlte ]In perftrojpieid mtttsele of ttle septlni amltI
tite eristca snr {ttritlaris so thafit toxxardl
 410
the end of systole the ventricular septum
would be effectively closed. Recently, proof of
this hypothesis was obtained in one of these
patients at operation. The right ventricle was
opened while the circulation was maintained
by the pump oxygenator; the heart was not
stopped. With each contraction the ventricu-
lar septal defect was squeezed closed by the
hypertrophied muscle around it and the in-
fundibular chamber was cut off from the bodv
of the right ventricle.
One or both of these events might have
caused the notch in the aseending limit of the
right ventricular pressure tracing in this pa-
tient and 6 others. A similar notch was noted
only once in pulmonic stenosis in a patient
with marked infundibular stenosis.
Preoperative Diagnosis
Accurate preoperative diagnosis of this le-
sion is essential in indicating the need for sur-
gical repair with extracorporeal circulation
rather than with hypothermia and in making
right ventriculotomy mandatory. Phonocar-
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diography was of great help in suggesting
that these patients did not have pulmonic ste-
nosis with an intact ventricular septum inas-
much as (a) the systolic murmur was maximal
early in systole, whether or not it passed be-
yond aortic valve closure or (b) the systolic
murmur ended before a single or closelv split
second heart souiid, whether or not the mur-
mur was maximal early in systole. It is true
that in mild pulmonic stenosis right ventricu-
lar ejection may not be prolonged so that the
systolic murmur may be maximal in midsys-
tole and end at the aortic component of a
normally split second heart sound, but these
patients will not show the features of marked
right ventricular hypertrophy.
These phonocardiographic criteria may not
hold if there is puluonic stenosis and ventric-
ular inversion (corrected transposition).
Three patients with this syndrome have been
seen with phonocardiograms typieal of tetral-
ogy of Fallot but with no evidence of an asso-
eiated ventricular septal defect on cardiac
catheterization. The diagnosis has not yet been
proved at operation. anid until then phono-
HOFFMAN, RUDOLPH, NADAS, GROSS
cardiograms in this syndrome should be inter-
preted with caution.
If after clinical, radiologic, and phonocar-
diographic examination cardiac catheteriza-
tion is required, it is obvious that in patients
with pulmonic stenosis complete reliailce to
exclude an associated ventricular septal defect
cannot be placed on a high right ventricular
systolic pressure that rises further after ec-
topic beats and on a triangular right ventric-
ular pressure curve. The only direct preopera-
tive demonstration of this defect is the
passage of the catheter through it from the
right ventricle to the aorta; failing this, indi-
cator-dilution studies or angiocardiography
should always be done. The site of injection of
the indicator or radiopaque dye is important,
for if it is injected into the infundibulum or
into a stream that does not pass through the
defect, the right-to-left ventricular shunt may
not be revealed. This happened in 2 of our
patients with arterial oxygen saturations of
89 and 93 per cent, in both of whom right
atrial injection showed a right-to-left shunt.
Failure to demonstrate a right-to-left ventric-
ular shunt by these technics, especially when
the arterial oxygen saturation is near normal,
is thus not unequivocal evidence of its ab-
sence. Another possible source of error occurs
with marked tricuspid regurgitation and a
right-to-left atrial shunt, for then indicator
injected into the body of the right ventricle
might be swept back into the right atrium and
then across to the left side; a good angio-
cardiogram should help to avoid this error and
has the additional advantage of demonstrating
the anatomy of the outflow tract of the right
ventricle.
Summary
Ten patients with pulmonic stenosis are re-
ported who, despite having right ventricular
systolic pressures much above systemic levels,
had ventricular septal defects proved at opera-
tion in 9 and suggested by indicator-dilution
curves in the other.
Because of the high right veentricular sys-
tolic pressures these patienits had clinical and
electrocardiographic evidence of marked right
venitricular hypertrophy and were initially
Circulation, Volume XXII, September 1960
 PULMONIC STENOSIS
thought to have severe pulmonic stenosis with
an intact ventricular septum.
One patient had an almost single ventricle
with pulmoinic and aortic stenosis.
In all the others the right ventricular systol-
ic pressure failed to equilibrate with pressures
in the system-ic circulation. In 2 patients this
was probably due to obstruction of the ven-
tricular septal defect by the septal leaflet of
the tricuspid valve. In the others the sugges-
tion that the ventricular septal defect was
closed off during systole by muscular contrac-
tion was confirmed in 1 patient at operation.
This syndrome has been reported in other
series and may occur in as many as 5 to 10
per cent of patients diagnosed as having se-
vere pulmonic stenosis.
It is important to know before operation
whether or not there is a ventricular septal
defect associated with the pulmonic stenosis,
for this influences the decision about doing a
right ventriculotomy.
The preoperative diagnosis of this variant
of tetralogy of Fallot may be made by phono-
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cardiography and cardiac catheterization.
Summario in Interlingua
Es reportate dece casos de stenosis pulmonic in
que in despecto de nivellos del tension systolic dex-
tero-ventricular multo supra le nivellos del tension in
le circulation major-le presentia de defectos ventri-
culo-septal esseva demonstrate al operation (9 casos)
o suggerite per le curvas del dilution de un indi-
cator (1 caso).
In consequentia del alte tensiones systolic dextero-
ventricular, iste patientes manifestava evidentia
elinic e electrocardiographic de marcate grados de
hypertrophia dextero-ventricular e esseva considerate
initialmente como sufffrente de sever stenosis pul-
monic con intacte septo ventricular.
Un del patientes habeva quasi un sol ventriculo
coin stenosis pulmonic e aortic.
In omne le altere casos, le tension systolic dextero-
ventricular non se equilibrava con le tensiones in le
circulation systenmie. In 2 patientes isto esseva prob-
abilemente le effecto del obstruction del defecto yen-
triculo-septal per le lobo septal del valvula tricuspi-
dal. Quanto al alteres, il esseva suspicite que le
Circulation, Volume XXII, September 1960
411
defecto venitriculo-septal esseva claudite durante le
systole per uni contractioni muscular, e iste suspicionl
poteva esser confirmate in uIi caso al operation.
Iste synidrome ha essite reportate per altere autores
in altere series de casos e occurre possibilemente in
usque a 5 o 10 pro cento dcel easos diagnosticate como
sever stenosis pulmonie.
Es importante saper ante le intervention chirurgic
si o non le patienite ha uin defecto ventricular-septal in
association con su stenosis pulmonic, proque isto
exerce un influentia super le decision de si o noin uni
ventriculotomia dextere debe esser effectuate.
Le diagnose pre-chirurgic dce iste variente del tetra-
logia de Fallot pote esser facite per phonocardio-
graphia e catheterisiiio cardiac.
References
1. KEITH, J. D., ROWE, R. D., AND VLAD, P.:
Heart Disease in Infaney and Childhood. New
York, The Macmillan Coampany, 1958.
2. NADAS, A. S.: Pediatric Cardiology. Philadelphia
and London, W. B. Saunders Company, 1957.
3. WOOD, P.: Diseases of the Heart and Circulation.
Ed. 2. London, Eyre and Spottiswoode, 1956.
4. HOFFMAN, J. I. E., RUDOLPH, A. M., NADAS,
A. S., AND PAUL, M. H.: The physiologic
differentiation of pulmonlic stenosis with and
without an initact ventricular septum. Circu-
lation 22: 385, 1960.
5. RUDOLPH, A. 3I., AND CAYLER, G. G.: Cardiac
catheterizationi in infaants and children. In
Pediatric Clinics of North America. Phila-
delphia and London, W. B. Saunders Company,
November, 1958.
6. SOULIE, P., JOLY, W., CARLOTTI, J., AND SICOT,
J. R.: Rtude compar6e de 1 'hemodynainique
dans les tetralogies et dans les trilogies de
Fallot (Etude de 43 cas). Arch. mal. coeur
7: 577, 1951.
7. McGooN, D. C., AND KIRKLIN, J. W.: Pulmonic
stenosis with intact ventricular septum. Cireu-
lation 17: 180, 1958.
8. MCCORD, M. C., VAN ELK, J., AND BLOUNT, S. G.,
JR.: Tetralogy of Fallot. Clinical and hemo-
dynamic spectrum of conmbined pulmonary ste-
nosis and ventricular septal defect. Circulation
16: 736, 1957.
9. BROCK, SiR R.: The Anatomy of Congenital
Pulmonic Stenosis. London, Cassell and Co.,
Ltd., 1957.
10. BLOUNT, S. G., JR., VIGADO, P. S., AND SWAN,
H.: Isolated infundibular stenosis. Am. Heart
J. 57: 684, 1959.