Tricuspid Stenosis with Pulmonary AtresiaA Cineangiographic-Pathologic Correlation

By MILTON H. PAUL, M.D., AND MAURICE LEV, M.D.

TRICUSPID stenosis associated with pul-monary atresia or stenosis is a relatively

uncommon complex that has been recognizedpathologically for mnaniy years.' More recentlythe clinical findings have beein docunmentedand summarized.2The course of the circulationi ii tricuspid

stenosis with pulmoinary atresia is essentiallysimnilar to that in tricuspid atresia with theright atrial blood being shunted into the leftcardiac chambers through a patent foramenovale. The henmodynamic role of the ob-structed, blind right velntricular chamber re-mains, however, inadequately defined. Thisreport deals with cineangiocardiographic andphysiologic observations in an infant withthis complex and presents a correlative anal-ysis of this material with the pathologic sub-stratum.

Clinical ExaminationA 15-week-old female infant, cvanotic since

birth, was admitted for study because of inereasingcvanosis and respiratorv distress.

Examnination revealed generalized slight cyano-sis. The liver was enlarged 4 cim. below the rightcostal margin and was pulsating. There was apronminent lower left sternal border systolic cardiacimpulse. No thrills were palpated. The first heartsound was of normal intenisity, and there was aloud earlv systolic click, masaximum at the upperleft sternal border, The second heart sound wassingle-sounding and of slightly increased intensityalong the left sternal border. A harsh short grade-III systolic murmur was maximum at the mid-left sternal border.The electrocardiogram (fig. 1) indicated right

atrial hypertrophv and severe left ventrieular pre-

From the Cardiovascular Laboratory and the De-partment of Pathology of the Children 's MemorialHospital, and the Congenital Heart Disease Researchand Training Center, Hektoen Institute, Chicago, Ill.

Supported in part by a researeh grant (H-3351)from the National Heart TIistitute, U.S. Public HealthService.

ponderance. The x-ray showed the heart to hemoderately enlarged, with an abnormallv promi-nent right atrial segment, decreased pulmnonarvvasculature, and left aortic areh.

Cyanotic spells and episodes of severe respira-tory distress recurred with increasing frequenecin the hospital, and the infant died 5 days aftercatheterization at 16 weeks of age.

Cardiac Catheterization andCineangiocardiograms

Cardiac catheterization was performed underiiild sedation (Demerol-Phenergan-sparine mix-ture) and the catheter was inserted via the rightsaphenous vein. From the right atrium it waspossible to enter a ventricular chamber located inthe right anterior portion of the cardiac silhouette.This chaniber was entered repeatedly, but at notinie could the catheter be manipulated to enteran outflow tract. No interatrial communicationcould be demonstrated by passage of the catheterinto the left atrium. Blood samples for oxygenianalysis and blood pressures were recorded (table1 and fig. 2).

Cineangiocardiograins were made at 48 framesper second with the rapid (.1 see.) injection of3 ml. of 90 per cent Hypaque, twice into the rightventricle and once into the right atrium (fig. 3).The right ventricular chamber remained opacifiedfor an unusually long period, over 480 frames (10see.), from the instant of injection. Regurgitatioifrom right ventricle to right atrium was clearly-demiionistrated throughout this period.

Postmortem Examination of HeartOn gross examination the heart (figs. 4 and 5)

weighed 39.5 Gm. (fixed) and was enlarged. Theapex was formed bv the left ventriele. From thebase 2 arteries emerged. The larger was situatedposteriorly and to the right; the smaller anteriorlvand to the left. The mutual relationships of thevarious chambers were normal. The cardiac meas-urements are given in table 2.The right atrium was markedlv enlarged and

thickened, being larger and thicker than the lefta-trium. The right atriunm received the superiorand inferior venae eavae and the coronary sinusin a normal manner. The limbus was well formed,and the foramen ovale was patent about 0.8 Cm.

8 C irculation, Volume XXII, Augu8t 1960198

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'I'le triculsp)i (ioifice was ver.y sriaiia (1 tihe ti-cuspid valve consisted of, 3 aittenuate(l (cusps, w ithvetIxN Smal11c |papilla 1 r11useles.

'lit right venitr icle was a minlulteI tutierlitits xvall was marikedi thickened, its thickness beillngequal to that of the left Ventricle. No) ar1terenerged f romi this (iamber.The lef't atriuill WaIS 1o11m111al ill size or s-lighti

larger than noraral and it was (listinctlv smiallertitan the right arinam. Its wa1ll was of normalthickness anti detfiiteil thlininler- th.-an thf.t (If tIerigt atr-ium. Ther initia-l or ifice was irorimaltl orsilitiv increased in size andi tire iritral vtave avsmtrirr'ralLk formed.

The left ventricula(r1. chamllber1 was mr1oraal ill size,but its wxall was thicker thail nonrial. Thiej ten1-tricular se])tum Was intact. Frorim tIis crIbererimerged tlief anor,ta. Tire aortie orifice was niormrailCirudlatiou, 'Volwume XXII, August 1960

199

Figure 1Elfecj 'e'Stroc( irdl(yoWzlmt. fJt'lolrvn Ituir.ft11,tet lot f> tt''0 f1'}?'/()hf 'fl{'.

or sfilitjriv illrareised it) size arid tie Valve xn,xI[ic1rsplTd With a.Ir'iht mrlrterior rrmtdl a left po)steriorcI-Is ). rjhe coronar\v o>stinl Wx1'err i -rrmral aInd tire(10oror11111Vx arteries wvere 11nor111ma11 iii dtirtributirl. Tired tilrts arterio()Slswas>ts llaitelit ari(d led ilto) tirepllnlrmoniary triniiik, w hricm wa rlistirmetlv sNrmallertim.iri time aorta. The ivall off tie piflimirmroim y trunikwas thirinrier' th,-ani norril. Thire iuhni)iit1oiVi tlririikeride tlhiirrdly at the hase of tie hreait, 1)ut, it gavx(Of tire 2 lLimilmmoitr^ ar^teries iIIil.1a0riorriml IIrarlirrer.Thbr'achimeplicldtalic ar1teries a'ro)se molrirnlriv

1i1 tire 1iglrt atriliurt there vas at- 11aeceeituatiormolf tire rorrloal enddoctmidiad tiickerinir ox-el tIrelirI1bius, tIre elittrx (f tIre ilnferiO verlrl-l (ca11vm, tirei)llsteio(lr crest, arid the (mdldocatdinra11 pr()oxilmll totire trienlspid ;miiiiiilus. l1m tire mi:lit ventricle tieeimtldmcrhrlmlill 'a1S diffuselx thrickermed arid wx hitenied,arid ai gro>ss diagnosis of fibrowbeiasto(.sis c-ould be

TRRA Si'll) WITH I't'l,Al()NA.'RY

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200 ~~~~~~~~~~~PAUL,LEV

RIGHTATRIUM

9 .., 9 - 9 -9''!J-t.;;;~j- .717-21 14¾i

ft

i.i K-

IK41CIi

rffII IIZt :i i:tci 222i.

fI-II.F -t-

4--

.--lo.4."I.I

I1

mm Hg100

'ii

5U

RIGHT

VENTRICLE.minmms S~~~~~~~~~~~~~4""-~.~,~,- ,,. Ii-I-

.I

Figure 2Prt .1/i/ic Iracitoia !'on,rgm at ri/f inid r-ig/it iveatricie.

Table 1Ct(a/turI.-(tiI/ji liti/ti

Oxygren Oxyg(en content,satuiration, % ~ ~Vol. %c

(5.5

4.1

7,/u

S.'1

21.8

1.8

ii9

5.2)

Pressure,mnm. Hg

:I tita v 1.4 ii/foa 7 ;)10l

90/1590/65

ititi,-ide. No ttl)ffolr ftl clf li 2i- s c'fill/ dletectedi ill

life tricuspid Hots ver dfilfuscly thic(~kened,

tihf tlii kettui il/v/fvix 1io thff edge.,l S ubstance, anid

ibtse. r'lf/,ev 1v/r ('fit teetd ibv ffti t/)orinaliv s hiort

/lfffild"te tof sliitit pm Ip ilt ry itit-itd s. rIT h .re Av s

f/a lk /d tiiickettiu f/ tift luff of (if/suite of the

ttitral vifiv/ tlat Wtfis tdtiftfft nodullitf inl nttItitre,

iti olvi n-tg ttfplte a tetior 1/taflet tttnd to a.

less extentt tiff il/leVi/ iefttet. Inl 1/lace of pul-

uf/utie val1vultir sttrcttu/f thfere w/l severtti fnsed

folds oif elidne,:fulu:fi ti-'-ue. -All the aortie enspts,//jpecit/iiy tli/f 1ig ft tftftelif01, ANT'iv tIhicker' titan-

Discussion

An opportunitly was presented during cair-

diac catheterization to stildv the Ifemodynanne, fun-ction of the obstruTcted righIt voni

tricuiar cthamber iM a hleart with tr-icuspid

stetios>,7 pulmonary atresia, anfd ani intact

ventricular septumn.

'l'lic pa,"tiflo tify oir t1 i /reseiit cats(

fails iiito the tisinl i)atteirli of this com11plex.The tricuispid or-ifiec ini mtost cases is sm,iiall

clue to a s malIl a nnuiilar opeiiung rathier thian

tto a stetiosis- of the valve strutture itscif. rriie

vatlve tisuafllv consists of thic,keiied leaflet tis-

su,e Cotni1e eted li)v shio rt ehor01dae to s mail l)api)llariy niu11se'les. Thle wihuinoliii orifice is smtall1 or

c-omipletely absiuit. V/lieu. smiall, the auiiuilar

openling" is redluced iii s..ize wiithi a tylpical ring

type of ipulmtouiary. vaivular steiiosis.

WhIen the or.ifiee is obiitcratcd, aggclutinated

valvulair cusps miay- be reeogiiized or no dis-

tinect valve mi'ay be recognized. As ini all eases

of tricuspid sten d attresia,

atrial septal defTect (patenit foram-en ovale),

hyvpertrophyw of the righit atrium and left ven-

tricle, aCnd a dindii-untive, right venitricular

ehamnber. There is, in mnost eases, a markedly

Circ'ulationn, Volume XXII, August 1960

rm Hg9

201

10.

0

7-4

Catheter- site

-Sutperjotr velia c':vxlflght trittitaf

11/guflt vvcutrtiec (,1l'ettfor'.1;.lI it

'HM471-i Iii,

f :'f il ii I' F -., ili-` f-i-.'7,

200

"44:=11T,..

-t.

-,I

. " i"

44.414

44

rH

lit

-t-"i.J.

III 4.1.:I-, .. ... 1.1.1,

I!,11 ..;

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201TRICUSPID STENOSIS WITII PULMONARY ATRESIA

Figure 3Cineangiocardiograms (lateral) following injection of 3 il . of Hypaque (90 per cent)into right ventricle: A. Onset of injection into right venitricle (1). B and C. Completeopacification off riight ventricle (1) and?6 r-eguJtr'gita tiont in1to right atri-ani and atrial op-pencdage (2). U anid E. Opacificationl of left atr-iumin an2d left ventricle (3). PA. Opacifica-tion of aorta (4) fillinig front left ventricle, and pulm)onary artery (5) filling via patentductus arteriosus. Note that the right rentricle (1) remnains heavily opacified in F,approximattely 8 seconds after completion of dye injection.

tlhieneiied riglht ventiicular wall witlh diffusefibroelastosis of the en-cdocardiurn. Increasedfocal mydocardial hypertrophy is noted in the

Circulation, Volume XXII, Auguet 1960

ight atriumi ill most cases anid there arehiemitodyliam-iie chiang(res in the m:litral and aor-tic valves. The ventricular septum is usually

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PAUL, LEV

Figure 4 Figure 5It'lf'II t I/it1b)/ito Ii tt/ 11 1: of tIlt l ci/I litt/th I.

r

j.. t. ftisso /If; Itc-1111/ I~, It ft It Il / lit It 111, Ithe 1/111/ r1. lit tlet: 1- l(1 / slItf179/lI (t ittlitIt

ond~~~~~~ ~~~~~~~~~~~~~L[fEi f {ltochietn ca l r ; C, c tvi-o-wz fr} s 8 lol s; TV11-f 1cusfp>J#I /

fl/l] f't v'e}n}ttlrl'. l'bOte2, ondf It, por82ictaJl walre1 oJW f/he l'ighllt ,'1ti

intact. A platelit dlullltu1s arteriosiis of 1ode(r-ate ol small tal iber i usually iresenit canti itl'elP lt sec llts liet 1ri11118I llmitelotl' blood tlo IlIt

Il ellSe aliloiie fi iidiiigs take Onim

l .11 ".t hlel e ll li ,fr i,,t itcll\ h1 aXs lxth ei li

za titi .ll 111(1M iiil 'ahigiol .11'tli ogl'alidie fi dllin gs.

Tl'e i 1je( tioll (of iiil. of tontiast materialiiit t oe 8si111 11ted ve tit l l e ha nihe of

.pp1roxi7liiatelI 2 tot :3 iiil. vohititie. p1robahllyrepla('etl a larg"e( )Ptj)i'tntiin of thle ventrieu-Jarl bXelood lld.|e t1ellsl prov)\ided> *1 c#tetillUOus.bult (1i11lillis11ill0, Opac>Xeific.ation of tlse '}chambterI1lt'r a ot1(11 1()0 selmlilts (11' 25) eardiia< co itrae-

tiol1s. Tli (.i le lloJigiltardi rademllrateiionistratetl

t111t the al1lto lili l'-all steii0tite t1'illspi(I o 1i-file 11811W a s a ieg iur'gitailt orihfie. 1rThese o

serrvat io)i I s aare ecessarI'i I 1 11.a(11we-ithI thie

1't1.t'( l vat'ahitt'r traversillt thlec trie'uisi id valve

a1d11( the i'tole (of the (cathetet' ill iiidii'iliig 01

aignietithig thite regiritatiti is nlldefilled. Itsteal's quite liktelv. 111v ever, that there is 11su-

allv a b1)ilidireti(llall Iliovemllelit of btlood aeross

the triiespitl (iee ilt this con(litionl. It eala

be J)t0 11siilaicll that the bitdiieeltioIwal mniittve nicilt

(1 i)1()t)l a1sso)ciatedl w ithi the trieusipid reogur-

gitatiol rlreVellts the dIi imiinutive ventiwi thl (A)strucltedl oultflowX fi mll b)eeom)imn obl1it-elralet stasis .timd tIiroml b)osis. 1Il1iis latterfindinlg' IitsX 1eTell desellleed ill triculls)i(l Stel)-

sas 'ith pulmionary atresia.'4

rth1'1ti-1h at lriil jp'esstlr trlenillgs shlow very

lplro01i111'lit .itrial systolle "A w .\(es tfliat a1re( '1 M lv (eflectet ill he p 'esYstolit plilase ofIllet v-et ri'lt'ih.r t1l iiisil jllSthlf11l1a prIW5Ssure'X

the10 atial presstlit''s 1'et'01'tetl ill ('aly v(' I-

triit'ili. diastotle .rt Ilot tilli g'l'(r Iterl' thall't|(ltle larly di.id i tsi l ie J)ress1llres In. till'

1 ii-lg t Vetlltl e .(lld 1(11d)olo Ieflett llenliodv-ailitie.ll' sigiliietmieit Ivic-lisp)id stenosis. It

see' ns li el ho,w veVt'I', tli.t the usual trails-

val lvollpiI' CSr'58 I'T' g1ratient ti'aenimrs seen ill

isolcated tfrit'uspitl valvular stenlloFs. ainght i11t

he o1)tailletl ill tilns eoiM1lex because of till'

extreit'iely sii]si )lvollnes oIf lootl flo-wingo

tiross the sienoltie aitl regri'gitaiit tr'ieflps)itlotrifiel2. lr1'essi1're (t'iiiuvts rleor'ded fronit tille'ighlt -VIlltl'il'lt' i'it'th fron1 7 5/12 t-o 90/15

111111. Ig (1ll'illig ti' t cathete'rizaftiol, till'

tr'iallo{}1ifar e'jectt ionl ciirve'( is i)t tludikse thllto)bs1e tved ill tfl' Obstitletedi ('1ctal)t'Ib of Severe

i}mhiboniarIy valvular stenosis.

In tins eomiplex tIle triiuspind valve is pat

tllt 1lld( blootl elitel' s thle riglit veiitie]le. I11'8uset' tft tilte ot)hstiltr ilto at tIle outltet anti tIlt

sft'Ilosis at flit illht, lot veXtX ', 'olsi'derla.l )lt

jo'ight v I1Iit liSe ItlliiT l)h1't lt sit) i i l)1'l.St1 II t

l ihlt entl i]ll'r wall is eoltespondiuglythliekened. TIl'e eItvated righit te'ntricnlar

pr'es.s;re' 11m.1> lead1 to the dtvetop)lnllt of tri-en-spid 1't'gnrgitatimill. Tf]e right vt'ntrieular-volulne is siliall, probablv bmt'tause of thle siiall.

Circulation, Volume XXII, August 1960

202

1.1 Jill/Ill1)111/h/Irf OrJ'clit,! 1 lit/IJ ll

It 11111t

I ot'

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203TRICUSPID STENOSIS WITH PULMONARY ATRESIA

Table 2Heart Me(surements (Fixed Heart)

Wall thickness

Right ventricle

Pulmoniic area

Tricuspid area

Apical area

Left ventricle

Maximum

Apex

Circumference of orifices

Tricuspid

Mitral

Aortic

Internal wall nmeasuremiientsRight ventricle

Inlet length

Inlet perimeter

Outlet length

Outlet perimeter

Left venltrieleInlet length

Outlet length

Perinieter

SummaryAn opportunity was presented during car-

diac catheterization to study the hemodynamicfunction of the obstructed right ventricularchamber in a heart with tricuspid stenosis,pulmonary atresia, and an intact ventricularseptum.

Cineangiocardiograms demonstrated thatthe anatomically steuotic tricuspid orifice was

also a regurgitant orifice and it is postulatedthat the bidirectional flow of blood associatedwith the tricuspid regurgitation prevents theobstructed ventricle from becoming obliter-ated by stasis-thrombosis.

3.6

3.0

3.2

1.6

3.2

0.6

2.4

3.3

4.1

volume of blood entering and leaving thischamber. The fibroelastosis in the right ven-tricle may be the result of stasis, increasedendomyocardial tension, or some other unde-fined factor.5The right atrium is large and thick-walled

due to the large volume and high pressurepresent in this chamber as a result of the tri-cuspid stenosis and the relative functionalstenosis of the patent foramen ovale.

Summario in InterlinguaSe presentava le opportuiiitate, durante catheteri-

sation cardiac, de studiar le function hemodynamicdel obstruite camera dextero-ventricular in un cordecon stenose tricuspidic, atresia pulmonar, e un in-tacte septo ventricular.

Cineangiocardiogrammas demonstrava que le ana-

tomicamente stenotic orificio tricuspidic esseva etiamun orificio regurgitante, e ii es postulate que le fluxobidirectional de sanguine associate con le regurgita-tion tricuspidic preveni que le obstruite ventriculoes oblitterate per stase e thrombose.

References1. LEv, M.: Autopsy Diagnosis of Congenitally Mal-

formed Hearts. Springfield, Ill., Charles CThomas, 1933.

2. KEITH, J. D., ROWE, R. D., AND VLAD, P.: HeartDisease in Infancy and Childhood. New York,The MAacMillan Co., 1958.

3. LEV, M\., AND ROWLATT, V.: A Semiquantitativegross and Ihistopathologic method of studyingthe congenitally malformed heart for clinicaland physiologic correlation. To be published.

4. GLABOFF, J. J., GOHMANN, J. T., AND LITTLE,J. A.: Atresia of the pulmonary artery withintact interventricular septum. J. Pediatrics37: 396, 1950.

5. BLACK-SCIIAFFER, B.: Infantile endocardial fibro-elastosis: A suggested etiology. Arch. Path.63: 281, 1957.

Circulation, Volume XXII, August 1960

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MILTON H. PAUL and MAURICE LEVCorrelation

Tricuspid Stenosis with Pulmonary Atresia: A Cineangiographic-Pathologic

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1960 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.22.2.198

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