TOTAL,
UNCOMPLICATED,
ANOMALOUS
PULMONARY
VENOUS
CONNECTION
Morphologic
Observations
on
I
3 Necropsy
Specimens
From
Infants
Frank E. Sherman, M.D., and S. Richard Bauersfeld, M.D.
L)eparlments of Pathology and Pediatrics, Children’s Hospital of Pitt.sburgh and School of ?tledicine,
tTnmicr.sity of Pittsburgh
(Adc’ptd S(’ptd’mmi)(’r 2:3, 1959; slIi)Iiiitted July 16.)
ADDRESS: (FE.S.) Children’s I lospitai of Pittsl)umrgim, I 25 I)eSoto Street, Pittshumrgii 13, Pemmnsylvania.
PEnmATRIcs, April 1960
656
T
Oi’i\ I, Iimmcommipl ica te(! , ammomimaiouis 1iliiioimaiv
\(miitis comimiection is aimalma-tomimic amid! cliimical emmtitv iii yimiclm till of time
retunmm
dirculatiomm fromn time hmmmgs is siiummted!timroimgim ammomimalous cimammimels to draimm with
tii( svstemmmic returmi imito time right atrium.
An iimteratrial comimmunicatiomi to sti)piy
i)lOO)d! to
time
left heart is a cd)IT11)Onent oftIme amioiiialv. The routes
by
vimichm the1)ulmnonarv returmm gaimms access to time nigimt
atniumim arv fronm case to case but right
heart hv[)ertro1)hv amid! immereased!
I)tmlmo-iiarv circuiatio)mm are comistaimt acquired! a!-terations. CaSeS comniplicatedl by other mmmajor
commgemmitai card!iac d)r extra-card!iac
ammoina-lies iua’e beeim Omnitted! from timis
I)resentl-tio)ii. Time cases to) be d!isdussed! simould! not
i)(’ commftmsed With CiS#{128}’5of partial anomaloums
1)tiimmmiictrY vemmous commnection vimere one or
mumo)re 1)1ml1ummmrY veiims drain normally.
Time nmodlermi iiisto)rv O)f timis emmtity begins
vitii a review I)V Brody1 imm 1942 that served!
to olefimme amid stimumulate immterest in time
amioiiialv viiiehi 111) to that timne ‘as
re-gar(!ed! largely as aim ammatoimmic curiosity. Time
timimiimg of this review was sucim that interest
iii tii( lesid)mi \VOS imot comifimied to time
aca-(!emiiic mmiorpho)logist i)ecatmse advances in
card!iae pimvsio)logv ammo! imm surgerY were
StmOhi as to rapio!l’ establish total anomalous
l)11imuimiarY vemmo)uS eoimnectiomm as a clinical
emmtitvof
commsid!erable surgical impo)rtammce.\hLim\’ reports of ciiimical oTiagmmosis of timis
mimoiformumatiomm lia\e receiitlv a)peared!, amid
surgical experieimce vithi \arviiig results
has i)e(’Im reported! fromum a nrmmimi)er of
sources.-Time scope of the present paper is limited
to anatomic observations on 13 cases that
came to necnopsy. Emphasis is placed on
anatomic findings of climmical significance.
PROCEDU RE
Iii assessimig time incidence of vemmoums
ano)ma-lies in any necropsy series, techniquie amid care
of the prosector are major factors iii
determimi-imug validity O)f time data. 1mm situ cardiac
dissec-tion with idlentificatiomi of all afferemut amid!
effer-emit cardiac vessels has beemm a routine
proced-tine iii our laboratory for the past few years,
amid! it would seem that timis imm situt dissectiomm
has increased! the observed mmumber of veimous
amuomumalies. Formnerlv, techmmmique was at time
dis-cretiomm of the imidividumal prosector. Remumoval of
organs separatel’ prod!uced worthless
speci-nmeims for the study of venous conmuectio)mms, ammo!
l)lock dhssection done before idlemmtif\immg
‘as-cular commmmections nmad!e imuterpretation of the changes difficult.
PATIENT
MATERIAL
AND
INCIDENCE
Twelve cases have beemu observe! imi 1,533
mmecropsies o!uring a I)eri(! of 8 ears 4 iimommtius
(January 1951 througim April 1959). No cases
were observeol in 801 imecropsies durimmg the
precedimmg 10 ‘ears
(
1941-1950). Commgemuitalcard!iac ammomumalies were the nmajor lesiomis iii
304 of the necropsies immtime 1951-1959 period.
Total, uimcomplicateo!, ammomaloums pulnmomuarv
venous commmmectioim thuis accounted for 3.9% of
fatal commgemuital heart disease. The imuck!ence imi
other reported series is: 2% of 8008 commgemuital
cardiac lesiouus; omme imm 1,000 cases; amid! two
iii 300.1(1
Time mmmuseumum of commgeimital heart d!isease at
Cimildremi’s Hospital of Pittsburgh contains 326
nec-Amm embryologic classification of total
ammornalous puilnionany venouis connection is
desirable not d)nly becatise it has
patho-genetic implications, buit also it creates
rca-sonable groups for diagnostic and surgical
Pumnl)05e5.
Neill’s classification13 is adaptable for
climmical purposes. In Table I, it has been
altered 1w numbering and lettering time
vanid)uis classes for convemmience. Diagrams
(
Figs. 1-4) illuistrate time types fotmnd in thisA.
ARTICLES 657
ropsv immaterial aimd 35 are from outside
sources. All O)f the 13 cases studied! are in the
mumuseumum,
audi one of these, Case 9, is from anoutsi!e
souirce.
Time immcreasimmg mmumumi)ers of
cases
I)eingre-portedi iii recemmt years fromum muecropsies indicate
thiat this aimomumaly is coimsiderabl less rare than
fornmerlv thoumgimt. Brodv’s reiew’ included 24
ummmco)mumphicated! ammd! commmpiete cases. Darlimug ct
(ll.’ iii 1957 i)rotmgimt time literature up-to-date
amid ad!d!ed 17 cases of their
owmu
to make ato)tal of 80. Simmce themm 1:3 cases have been
re-ported frommm mmecropsies.2 ‘ ‘ \Vith the 1:3
Portedl here, the total recorded observatiomms of
this anonmaly made at necropsv stands at 106.
CLASSIFICATION
AND
EMBRYOLOGY
TABLE I
ANOMALOUS PULMONARY \ENO(S (oNNF:i’moN; CLASS1FICATmON OF NF:mIL’3
(Modified slightly by numbering and lettering)
‘I’ype 1. I)iredt drainage into time might atriummn if time
auricuiar septum grew ai)mlornmaily far to time
ieft (Fig. I).
‘IYI)C 2. Draimmage into the right domimmimon (ar(iimmal
sys-tern (superior vena dava, azygos vein).
Per-sistemmee of (onmnmunications l)etween time
pumi-mimommary venous I)1eX1mS amm(I tile right 1m(Irmmof
time simmus venosus.
‘J’y)e 3. I)raimmage into time ieft (011)10011 (ardimmal
sys-tern. Persistence of (onmnmumuicatiomms Ilet weemm
puimommary vemmous plexus and left horn of sinus
venosus.
Type 3A. Left superior vena cava (Fig. ))*
Type 313. Coronary sinus (Fig. 3).
Type 4. I)rainage into the umimbilico-vitellimme systemmm.
The puimimommary plexus (lraimms immto a (omummlomm
cimamimmeicioseiy associatNi ‘itim time esopimagums;
it themi pierdes time dliaplmragln . . .through time
foranuen of Blair an(l enters time portai system.
‘fype 4A. Portal veimm (Fig. 4). Type 413. I)uctus venosus.
* Edwards and Hel,mmholzm4 object to the term,
“per-sistent left su/)erior rena coma,” since it has no
rommmnmummmi-cation with time heart. They suggest time term, “rertieal
anomalous pulmonary vein.”
VENA CAVA
ATRIUM
NE VENA CAVA
V.
FId;. 1. Case 1. Specimmmen viewed fromum time posterior aspect
Wii(iIl this diagraimm vas mimade. Time long anomumalous vemmous
trummmk fromim time left lummmg joins time shorter puimmuo:mamy veimus frommi
ti1d right Si(!e in a simallow dliverticumlumnu on tIme posterior wall
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FIG. 2. A diagrammatic representation of the most
comuumuuonanonualous course, type 3A, in this series,
viewed anteriorly. Relations were purposely
dis-torted to show the venous route. Cases 2 through 7.
series. A short review of the embryology
of the pulmonary veins aids in
understand-ing this classification. When the lung bumd
develops, it is drained by a venous plexus
that communicates freely with the common
cardinal and umbilico-vitelline systems.
Commection to the left atrium is made by an
evagination (pulmonary venous trunk) from
the sinuatrial region of the developing
heart. This evagination communicates with
the pulmonary venous plexuis and proper
pulmonary return is established as the
sep-turn primum forms to the right of this struc-tune. If the septum forms to the left of the
pulmonary venous trunk a “type 1” anomaly
is formed. If the pulmonary venous trunk
fails to develop, on develops and is later
Fic. 3. Diagram illustrating the pulmonary return in
type 3B cases. The view is anterior. Cases 8, 9,
POSTERIOR ASPECT
Right innominots
Internal jugular subclavian vein
Lett superior intercostal vein
Right atrium
Interior vena
ligament
Hepatic
Fic. 4. Amuommiaiotis course of time puummmonany veins in
tYpe 4A cases viewed! posteriorly. Returmm flow is to
tlm(i portml veimi throumgh time Imepatic sinusoids, the
imUl)atic veins, and inferior vena cava to the right
atriummim. Cases 11, 12, and 1:3.
resorbeo!, timemi nettirn via common card!inal or ummbilico-vitehline system is maintained
ammd! omme of the oilmen ‘anieties of anomalous
puilmTnonarv veno)us commnection is
estab-lisheol. Aitimoumgh time ammomalous retuirn may
be to ammv vein d!erived!
from
the commoncardinal or uimbihco-yitehhine systems (Fig.
5), time nmajoritv of reported ammomaiies fall
into Neihl’s types 3A, SB, and 4A. All but
time first of time present cases were in these
timnee categories.
ANATOMY
Venous Distributions
TYPE 1, DRAINAGE DIRECTLY TO THE
RIGHT ATRIUM (CASE 1) : The path taken
ii’ time I)tiimonlnY veins in this case is
dia-grammirned in Figure 1. All veins emptied into
a shallow atnial diverticumluim which had a
\\‘id!e openimig into the night atrium
im-mediately inferior and posterior to the
su-perior vemma cava. Three short veins drained
time right lumng, one from each lobe. Veins
from each lobe of time left lung joined to
form t simigle trummk 17 mnmn long. It had an
extnapericandlial course, but its inferior and!
posterior sumrfaces were bordered by
pen-cardium and it lay close to the left atrium.
Suirgical anastomosis of timis left pulmonary
vein to the left atniuinm woumid have been
feasible. It would have been impossible to
have directly connected time right
pulmo-nary veins to the left atniumm.
TYPE 3A, PULMONARY RETURN VIA LEFT SUPERIOR VENA CAVA (CASES 2-7) : All six
cases of this type were similar and
con-formed to the iilustration given in Figure 2.
(An exception was Case 2, where the left
upper veins drained superior to the major
confluence and directly into the left
su-penion vena cava.) In these cases, the right
pulmonary veins formed a common trunk
posterior to the penicardiumm. Timis trunk
joined with the veins from the left lung
posterolateral to the main pulmonary
ar-tery. From this confitmence the thin,
trans-luicent left superior vena cava ascended
anterior to the left pulmonary artery and
iii the left border of the mediastinum to
join the left innominate vein at its origin.
TYPE 3B, PULMONARY RETURN VIA THE CORONARY SINUS (Fic. 3) (CASES 8-10):
Cases in this category vary anatomically
Fmc. 5. Diagrammu, nuodified after Hickie et al.,’
illuis-trating the various veins that have been described
as receiving pulmonary return. Instances of both
total and partial anomalous connections were used!
ARTICLES 661
EI(;. 6. I)rawimmgs mimade fromim Case 9. The specimmmemmvas vieweoi fronm time posterior aspect. (Left) Time
imitact ihhmmstratrs time apparemmthy mmonmmmal relatiomus of time pumimimommary veimms to time left atriummi. Time
smmmall left atriummm aimd time (!el)reSsiOIi i)et\Veemm time coronary smuts ammd right atriummu, caused!
b
emmlarge-mmmommt of timd coronlmrv simmums. mrd the univ cimamiges miOte(I extermmailv. (Right) Dravimmg slmotvs time relatiomis
of time lar( coromiarv simmims that simtmmmts tIme i)imlmmimmtry r(ttmrli to time rigimt S1(l(.
fromim all other tvl)es iii three nmajor reSl)ectS:
1
)
Time emmtire shmummt lies witiiimm time1)eri-cardiutimi; 2) time d!iStal cimammmmel of time shummt
is a imormal ammatornic cormmpomment O)f time
heart, however munch emmlarged! ammo!
dis-torted! this coronary simmus mimigimt be; amid 3)
time vall of time cimammnel is composed large!’
of mmmvocarc!iummim.
Time corommarv simmus is so) iimtiimiatelv
iii-corporated into time wall of time left atriumim
that it requires careftml observation tO)
dis-tiiigtmish time ammomnalotms cimammnel fnonm time
small left atniumim wimemm viewing time
epi-cardial surface. A er’ shiglmt d#{128}pression
separates timenm (Fig. 6). Time pulniomminy
veimms take a mmornma! course to their jummctiomm
witim time
nmvocardial wall of time coronarysimitis, vimichm times’ jo)imm iii time saine manmmer
timat
time
mmonmumahlv \VOtild! joimi time leftatriummi. This false appearammce ad!dis to time
o!ifficultv of d!iagimOsiS i)\ extenmial
inSI)ec-tioim of time hmeart.
TYPE 4A, PULMONARY RETURN VIA THE
PORTAL SYSTEM (CASES 11-13): Each of
these cases d!rained! by a conmmon trunk,
fornied!
by
a coimfitiemice of pimlmmmany veinsfronm i)otii lumigs. This truimmk olesceno!e!
an-tenio)r to time esopimagums, I)iercedl time
dia-)imragmfl, ammd! joimmed time Portal circumlation
as ihiustratco! iii Figtmres and 7.
Findings Common to All Cases (Table II)
GROSS EXAMINATION: Regardless of time
roumte taken by time anomalous ptmhmnonary
veins certain amiatomic findings were
pres-eimt in all cases. Within time penicand!iuimn,
these ‘ene: 1) An interatnial
commmmtmnica-tion, usually a simple forameim ovale; 2)
hvpertrophv of time chambers of time right
heart; 3) dilatation of time puilmonary
an-tens’; 4) a nonmnal or hmvpopiastic left
yen-tnicle ammo! left atniutmm; ammo! 5) a mmormal
aorta. 1mmtime type 3B cases, time emitine simumnt
lay within time penic;mno!iuirn. Oumtside time
I)enic1nd1itim, timese fiumdimigs were comistant:
1
)
Extnapericardiai route of time ammonmalouscommimectiomms in types 1, 3A, ammo! 4B; 2)
prox-imity
of
time comnmomm anommmaloums venotis tntmmmk to the left atrium (a surgicalanasto-mnosis would imave been ammatommiicallv
feasi-ble iim all instances); and 3) increase!
cm-culatiomi throuigim time lungs iimdicateo! by the fine lobumlation o!ume to interstitial edema,
browmm discoloration of varying intensity,
an! d!ilatation of pumlmonarv vessels.
Aim interatnial commumnicatiomm is a
neces-sary component of time anomaly becatmse all blooo! reaching time systemic circumit muist
flow timroumgh it. Time dumcttis arteriosus was
patent in
five cases,
but all were very yotmngFmc. 7. Posterior dlissecti(mmm (Case 11) illustrating the anonmalous pulnuonary venous trunk imm type lA case.
j)nOgressimig immvolutiomu iii each case. This
comnmummmicatiomi did not seemmm to) be a factor
imi svsteimmic circu!atiomm imm an case. The
immteratrial o!efect was mmot large iim any of
time 1)r5d’mmt cases ammo! all patients d!ICd! in
immfammcv, tim(’ old!est beimmg 14 mommtims of age.
This votmloI su)pont time suggestiomm that
cases with large imiteratrial conmmumnications
offer a i)etter pno)gnosis.
Soimme cimaimges that are commstammt iii all
ty1)es
are
imot
!ume to) elmmh)ryOlo)gicdevelop-miment but arc secommdlarv to altered function.
Time dliSI)rol)ortiomi ill time size of time two
sides of time heart is time mnost obvious of
these cimammges, but omw must mmot overlook
acquireo! clmammges ui time lungs o!ue to
necir-culation of large qtmammtities of blood!.
Time right-sided chmammmhers are both
di-lated ammo! imvpertrophie(1. In most instances
timey are estimated to imave a volummme of
about foumr times time cornespommdimig
charn-ber on time left. Congestive failure was a
major factor in time o!eath of all patiemmts. Whether time cimanibers of time left imeart
are small becauise of aplaia (a
develop-mental decrease or absemmce of certaimm cells or tisstmes) or atrophy (a iiormmmal imumber of cells and tissumes, bumt each cell is small)
is a question of fummdamental mmportammce. If
surgical connection is to) be feasible, the
left heart muist imave time capacity to)
en-large and carry on mmormal function.
MIcRosCOPIc EXAMINATION : Sections of
the small left atnia were made, iii all cases,
thnoumgim the central portion several
miili-meters from time append!age. Time imistologic
pictumre was that of atrophy ratimer than
ARTICLES 66:3
mmmuscle fibers were mmarnow vitim scanty
cvtoplasmmm aimd ahummmdant nuclei. Time
sub-eno!ocardial fibrous amid! elastic tissumes were
usually
scaimtv.
Time epicard!iuimn ano!sub-epicardial tissues were not altered. An
ef-fort to dlumammtitate rougimiv time muiscie fibers
‘as mimade i)\’ coummtimmg time mmumnber of fibers
that a hue tt right ammg!es to time
endo-carditmimu amm(l epioard!itmmum votmlo1 pass
through. This miuimmi)er 1)r0)\e! to vary
iumarkeohlv froimm case to case i)Otii iii the
studs’ group i)eimmg reported amid! imm
comm-trols commmposeo! of normmmal atnia fromn infants
of time same ages. Jim botim groups time mean
lmummmi)er Wt5 45. This does mmot indicate an
absemmce of imypoplasia because time altered
atnia are mumucim smmmaller than normal ano! if
Fmc. 9. Fimhl timickmmess of the left atritmnm fromum Case
7. The myocaro!iumm is quammtitatively simmmilar to the
control (Fig. 8). (IIemuuatoxyiin amid eosimm, X 120.)
timis small size were duie to atrophY of
Imypom-function alone, the number of fibers in time
contracted wall should be in excess of
mion-mal. It must be remembeneo! that time left
atrium in timese hearts has not beeim
sub-jected to the normal pimase of fiuial growth
in its embryologic developmemmt, that of
iii-corporating time pulmonary venous trunk. The fact timat aim adequate m.vocardial wall
is present ano! that it appears to be capable
of considerable, if miot normal, enlargement
is reassumning for surgical considerations.
Alterations in the Lungs
GROSS EXAMINATION: Changes in time
Fic. 8. Full tlmickmuess of time left atniummmu from a 1- .
. . . lungs are caused by increased blood flow
month-old infant with a nonimual heart. The
epm-cardiummmm is labeleol “E.” (1lemmuatoxviin amid eosimu, and, when present in certain clinical
sugges-Fmc. 1 0. 1vtmmphatic (lilmtatil)mm in time septtmmmm be
t.V(dml t’hv(l I)lilmmmommarv lOi)mml(’i 1mmil tV1)e 4A case
(Case 1 1). ii(t mrmI i\itmI)imatics P” arc also diiateol.
Timickdmmed1 alveolar :miis cave little air space.
(I I(iilllt(l\\lill m11(l(lliil, ><I0.)
tiV(’ f tOtLi limO)IimaiOtms I)mmimimo)mmaImY7
C()mimmectiomm. These cimamiges are : dilatation
of all vessels includ!immg capillaries; aim
in-creaseol imumuih)er of l)tmlmimfl1rY
mulacro-pimages, omiie ;tI)I)hie! to alveolar ahls,
ammo! somume Ivimig freely iii focal grou)s; mno! aim unusual tV1)(’ of puhmmmouuarv edenma. This
edenma is immterstitial aimd iimvolves time pleumna
ammo! time
fii)rolms S(’1)tt o!ividimmg timeseco)mmd-arv lO)i)1iiCS vimich alSo carry (hiiate(! veimms
amid lyni1)iiatics ( Figs. 1(1 amid 11
).
Thesveiliimg of these smimall S(’1)t0 iS SO severe that
times’ caim
1)0’ Seeli grossly omitue
j)leuralstir-face in a i#{149}eticulam I)ttto’rii (Fig. 12). This
ciiammge is i iiiamked accemittiatiomi o)f time
retictihar that caim i)e seemi in time
lungs of miiust iiufammts. It is
mumore
severe immthe tVI)e 4A cases iii wiiicii time ptmhmimnary
retumnim lmas to overcome time resistamice of
the P0)rtll systeni. It is 115() timis group of
cases timat is utmost likely to siiO)\\’ mumuitil)le,
smmmall, wid!ely dissemiminatedi foci of
in-creased! demmsity o)mm chest roemmtgeiiogramns.
This roen tgemmograpimic I);Itto’rmi is liii rl
char-actenistic wimeim seen iii time imeoiiatal
I)enio!. I
MICROSCOPIC EXAMINATION : Flistologic
)umlmOIiary vascuilar cimamiges ate of some
interest. Time large !ilateo! elastic arteries
occasiommaily have simmahl focal subimmtimumal
scars
ammo!
immimmimmial frayimmg of elastica isimoted. Time intiscumlar arteries amid arterioles
general ly have impertropim RId! simmootim
inims-dc coats. Absemmce of diegemmerative chammges
amid! intimal scars iii timese vesSelS wo)uld!
stiggest that this imvpertropimy is reversible.
Time dilated daj)ihlanies ire often ruptured!,
resultimmg in inimmuite hmenmo)rrhmages. Time imemo.
siolenimu formeo! by inacrophage activity in
these imemmmornhmages , althougim not severe,
gives time lummgs a faiimt brown timit.
Hy-eremmm ic, I)rO\vntilitedI hummmgs Wi tim reticu
-lateo! pleuiral surfaces suggest a severe
left-to-right shmtimmt, ammo! are likely to) accommmpany
cases of total anomimalous pmmlnmomm;try venous
conmmectiomm.
Structure of the Anomalous Trunks
Time type :3A amm! 4A cases hmave bug
aumommmalouis venoums tntmimks . These trummiks are
dilated and! imave timimm tramislumeent walls.
The o!iameters record!edl imm Table II are omm
fixed sPecimens ammo! are nmimcim simiahler timaim
in time fresh state. Histologic oi)ser\atio)uis
immeight cases reveal timese truinks to be
al-tered! by scarring. Time fibrous sear tissue
is ahummo!ant imm time advemmtitia ammd it
re-I)ltceS time thin musctmlar mneo!ia to varviumg
c!egrees. In thmree of these cases there are
focal regions wimere time smootim muscle of
time meo!ia is conmpletelv replaced! iW fibrous
tissue. An intirnal fibrous scar was noted! iii ouie case.
Terminology
DISCUSSION
Time ternm, “total, ummcommmplicated,
stig-Fl(:. 1 1. Immterlobumlar scpttmmmm imm a type 313 ease (Case 8) oontaimuimmg engorged veins amid
snmalier, h)tmt still (liiated, iymmiphmatics. I)iiated capillaries thicken time alveolar wails.
(Uemmmatoxviitm amid eosimi, X 120.)
ARTICLES 665
gested! by Edlwardsmh is ao!ummitteo!iy awkward
iii its leimgtim, butt it imas time d!istinct
ao!van-tages d)f being specific, exact, ammo! clear in its
!efimmitiomm. Eohvands used time wono!,
“con-nection, to imidicate a simunt of pulmonary
venous blood to time nigimt atniumn via
vascu-Ian channels, ano! to exclud!e non-vascular,
physiologic sliumits sucim as atnial septal
ole-fects. Oversimmiplificatiomm of terminology in
this imew ammo! o!evebopimmg emmtitv shotmldi I)e
avoided. Time term, “trammsposition of time
I)1uhmmmmm1rY \.eilms”:l, m seenms a poor Oiie
1)ecause it iniphies an abimo)rmTlal position of
otherwise umorimmai veimms, a situation thiat
does iuot exist. Also, it commmmotes some
aimal-ogy’ With time \vehi-estai)hisiiec! arterial
tramms-positioum, amm amiommmalv that is umnrelated to
total, ummcomplicated , ammomnaious puuimonan
venotms conumection.
Anatomic Factors Favoring
Surgical Correction
Five aumatomic factors favor surgical
cor-rectiomm:
1) Time anomalous channel alwayslies close to) time left atriumn ammo! imma positiomm
that wotulo! I)ermimit aumastonmosis witimouut
corn-1)id’te o!isntmptiomm of timat chammnel, so that ami
escape
route
cotmld be rnaintaimmeo! imm evemmtof left vemitnicimlan failure. 2) Time left atriunm
ano! vemmtriclc contain neaso)nable imutimbers
o)f myocardial fibers that shotild he capal)ie of enlangiumg ano! increasing time size ammo!
functiomial capacity of time left heart. 3)
There ‘ere no) major extracardiac ammonmalies
in timese cases. 4) Time ptilmmmonary vascumlan
changes did not incltiole scans or chrommic
degenerative processes. 5) The surgery is
pneo!ominantly extracaro!iac ano! vascular
in type.
Factors Unfavorable to Surgical Correction
Other characteristics of this anomaly are
uunfavorabie to surgical attack: 1) Time
imm-fants imm this series die! at an early age.
If they are tO) i)e saved, time well-knowmm
risks of thoracotomy in young infants vihl
have to be taken or overcome. 2)
Anonma-louis trumnks in tYpes 1, 3A, and 4A that
mtmst serve as puilmonany veins are not
normal veins. They are o!iiated, thin-valleo!
channels with varying degrees of scarring
666
A
lmc. I2 Ltmimgs 1mm situ imm a type 4A case (Case 11) ihlumstratimmg time reticutiar pattern made
l)’ ((lenii :111(1 vascumlar (lil1mtatiomm of time secondary immterlobu!ar septa.
smmmooth nmuscie co)ats. Time ammatorny o)f timese
channels immay immcnease time hazaro! of
timnom-bosis iii vemious aumastommmoses. 3) Time small
left atritmmmm unakes wide ammastomnosis
c!iffi-cult. 4) \lustaro! ammo! Dolaum, whmemm writing
ai)out time somali size of time left heart, state,
“It is felt that timese ciiaumil)ers are umot large
enough to accept time wimole Oltufle of
pul-mumommarv \‘(liOti5 return. It is now felt that
time left superior ‘emma cava should be
con-stnicteo!, i)tmt not comumpletely occluded.”
Times’ hat! imoteo! sbowimmg ano! irregularity
of time heart at operation wimemi occlusion
of time left superior emia cava was
at-temumpted in type 3A cases.
Diagnosis at Thoracotomy
At thoracotomy time diagmmosis is oi)vious
imm type SA if the left chest is emmtereo!. The
large anomalous venous trunks have timin
tramislucent walls and time bright red color
of time oxygenateo! blooo! is usumally cleanly visible.
Diagnosis of time type 3B cases is umiore
difficult because extnapenicaroliallv time
puil-monary veins are nonnmal. \Vitimimm time
pen-caro!iumm, time large conomiary sinus vitim its
myocardial wall hbemmols witim time left atnitini
in suucim a way as to mimic time mmormal left
atrium. Close immspectiomi viil reveal a slight
ARTICLES 667
where time large sinus bulges above the
trtme left atrium. Unfortunately these
struc-tunes are difficult to visualize at
thoracot-omv even if one has been fortunate enough
to have made a left-sided approach. If the
preoperative diagnosis has been incorrect,
it is most likely to have been a suspected
intenatnial septal defect. If the surgeon is to
avoid time catastrophe of suturing the orifice
of time coronary sinus, he must keep the
possibility of anomalous pulmonary venous
connections in mind when repairing such
cases. The enlarged coronary sinus orifice
lies more posterior and inferior than the
usual interatnial septal defect. It is closer
to time inferior vena cava. If the mitral valve
can be directly palpated through the
sus-pected orifice, one is sure that the defect is in time intenatnial septum. The coronary
sinus has a fibromyocardial wall separating
it from the left atrium.
Types 1 and 4A also are difficult to
diag-nose at thoracotomy. It would require an
unreasonable posterior mediastinal
dissec-tion to demonstrate these shunts. The
sun-geon’s suspicion of the anomaly is again the
major factor in making the diagnosis.
In-spection of the typically hypoplastic left
heart should reveal that the pulmonary
veins do not enter the left atrium. As in
the 3B cases, palpation through the orifice
in type 1 will not reveal the mitral valve.
SUMMARY
Anatomic observations on 13 necropsy
specimens of total, uncomplicated,
anoma-louis pulmonary venous connection are
pre-sented.
These cases accounted for 0.8% of all
deaths and 3.9% of congenital cardiac
deaths in a consecutive necropsy series.
Neill’s embryologic classification is
rec-ommimended because it is suitable for clinical
purposes. Time pulmonary venous return in
one of time cases drained directly to the
right atrium (type 1); in six it drained via
a left superior vena cava (type 3A); in three
it drained via the coronary sinus (type 3B);
and in three time pulmonary venous return
drained via the portal system (type 4A).
Findings common to all cases were: 1)
interatrial communication; 2) enlarged
right heart; 3) small left heart; 4) increased
pulmonary circulation; and 5) normal aorta.
Anatomic findings are related to their
surgical implication. The disproportion in
size and implied functional capacities of
the right and left sides of the heart is
stressed, and the authors agree with
pre-viously recommended surgical correction
in multiple stages.
Factors favoring surgical correction are:
1) The anomalous channel is so located
that anastomosis with the left atrium is
anatomically feasible. 2) The small left
heart contains a reasonable, if not normal,
number of myocardial fibers that should
be capable of hypentrophy and increased
function. 3) This anomaly is uncomplicated by other cardiac or extracardiac anomalies in most instances. All cases in this report
were uncomplicated. 4) Pulmonary
vascu-lar changes in these infants are without
scarring. 5) Myocardotomy in most
in-stances can be limited to the atnial wall.
Factors unfavorable to surgical
conrec-tion are: 1) Age at death in this series
varied from 15 days to 14 months. The risk
of cardiac surgery in infants is well-known.
2) The anomalous veins are scarred vessels,
and may predispose to thrombosis. 3) The
size of the anastomosis is limited by the
small size of the left atrium.
Anomalous pulmonary venous
connec-tion must be excluded in all cases of
left-to-right shunt at the atrial level. Anatomic
variations that might lead to a proper
diag-nosis are summarized for the various types
included in this study.
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