Download The Clinical Application of Cava-Pulmonary

The Clinical Application of Cava-Pulmonary Anastomosis*
Charlotle, North Carolina
T
H
E BETTER UNDERSTANDING OF P H Y S -
i o l w and the improvement in surgical techniques have made it pmsible for
anomalies of the heart and great vascls,
regarded as incurable a few years ago, now
to be corrected completely. A number of
patients, however, are still seen with malformations which are imposvible to repair.
In still another group, the anomaly may be
repaired completely from a technical standpoint, but the patient's size, poor general
condition or associated secondary changes
make the surgical attempt extremely risky.
if not hopeless.
This is the reason why palliative proced u r n such as the Blalock and Pots opcrations are still widely used, and newer
methods of palliation such as the cava-pul*From the Department of Thomic and Cardiovascular Surgery, Charlotte Memorial Hospital.
This work wa, supported by grants by the John
A. Hartford Medical Reaearch Foundation.
monary anastomosis have been accepted.
The latter procedure was described by
Carlon'" in 1950, and consists of creation
of an end-to-end communication between
the peripheral stumps of the divided superior c a d vein and right pulmonary artery. In this way the venous drainage of the
upper part of the body bypasses the right
heart and flows directly into the p u h o nary circulation. It was demonstrated in
long range animal experiments that the
blood flow t h r o u g h this anastomosis is
comparable to that of the inferior vena
cava, and the anastomosis remains patent.-' Similar obsen-ations were reported
by other in~estigators.~7"C's~'~~Ynnn
On the basis of these experiments a suggestion was made that the cava-pulmonary
anastomosis, as a palliative procedure might
be beneficial in some forms of congenital
heart disease a w i a t c d with impaired pul-
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monary blood Row. Shumacker," Meshalkin," Glenn,""' Rasmusen" and the authors"=* were among the first to give a
clinical report with this procedure.
This operation is relatively new and the
indication5 are controversial; therefore, we
would like to discus5 the merits and d i d v a n t a p of this method and enumerate the
conditions under which it may be applicable.
First a word about the physiologic consequences of this procedure.
The main hemdynamic effects are on
the cardiac work and the volume of the
pulmonary blood pow.
EFFECTSOF THE CARDIAC
WORK
The work load of either ventricle could
be roughly expressed by the amount of
Flouae 2: Portemanterior x-ray film of four-yearbeold aid who undcmnt lnvtomotic
twthe superior cava and right puulmnary art e y because of cyanotic h e m disease. Compare
the vavular -kings
of the
1,: ~h~ vu.
culnr markings arc markedly d e u e u e d on the left
side; them is n o r m a l v a r c u l a r i t y on the right
(anastotnotic) ide.
blocd ejected and by the circulatory resistance against which the blood is discharged.
T h e classic (subclavio-pulmonq or
aorto-pulmonan) bypass operations, although improving the oxygenation of the
Fro. 3A
PIO.3B
F ~ o u n s3: R m p r a t i v c (A) a n g i d o g ~ ofl ll-year-old boy with transposition of the p t v-b,
atrial and ventricular r p t a l ddcctl and pulmonary rtenorh. The injected dye p u v r from the nght a t n m
to the left atrium and left ventricle. Same atient thme yean after surgery (B),the contnut m a t e d
flows f m l y thmugh the wide open uva-p& nary anastomah into the right lung.
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CAVA-PVLMONAR Y
blood, does not alleviate the cardiac workload. On the contrary, because of the resulting left-to-right shunt and increment in
the pulmonary arterial pressure, the work
of both ventricle is increased.
However, with the cava-pulmonary anac
tomosis, 30 to 45 per cent of the cardiac
output bypasws the right h ~ a r t . ~ Cutting
"
down on the blood flow, the work of the
right ventricle is significantly Ieswned.
Both in the systemic artery-pulmonary
artery and in the cava-pulmonary anastomosis blood is shunted from the s!5tcmic
into the pulmonary circulation.
In systemic arterial anastomosis, the
amount of shunted blood depends mainly
on the size of the anastomcsis. If the anastomosis is too small, the flow is inadequate:
if it is large, a dangerous degree of left-torixht shunt may develop.
In cava-pulmonary anastomosis, the vdume of the blood flow thrmgh the new
channel is "set" because it represents b l d
"taken away" from the right heart so there
is no possibility of over-loading the pulmonary circulation. If the pulmonary blood
flow is abnormally low before the operation, it will be significantly increased afterwards; although when it is normal, there
will be no change.
There is also a difference in the quality
of the b l d shunted. In the classic bypass
procedures, the shunted blood is arterial or
mixed venous. Through the cava-pulmonary anastomosis, only venous blood enters
the pulmonary circulation. This creates a
more physiologic and efficient situation.
.4ccordingly, the cava-pulmonary anastomosis may be of benefit in the following
conditions:
1. Obstruction t o blood pow (anatomic
or functional) in the right heart or main
pulmonary artery.
2. Decreared blood pow through the
lung because of abnormal routing.
We have applied this operation in 14
patients, their ages ranging from six weeks
to I I years. Two patients were lost in the
ANASTOMOSIS
postoperative period; the rest of the patieno showed significant circulatory improvement. The procedure was used in the
following conditions:
TR~cusprnS T E N ~ S I S
True congenital stenosis of the tricuspid
valve is extremely rare. Circulation obstruction at this level is usually caused by h y p
plasia or atmia of the right -"+ventric-
Rc- (A) (upper) and postoperative
angidof a bpy, y m n
with Ebtein's h u e . In the k t p~cturethe d;
FIGURE4:
(B) (I-)
is r m m t in the greatly dilated G h t atrium. The
portoperative film shows a m U functioning cavapulmonary mnrtmmh.
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PAUL W.
SANCER,
el al.
ular ostium. .4s a rule, there is an atrial
septal defect and the development of the
right ventricle is also defective. The latter
chamber might be completely absent or it
may persist as a blind sac. Sometime it
receives its blood through an inten-entricular septal defect.
These features make the anomaly unsuitable for corrective surgery, but these
patienu are ideal candidates for cava-pulmonary anastomosis. Successful operations.
even on small patients in poor general condition, have been reported by several authors,.A.v.l.a."
We had the opportunity to perform
cava-pulmonary anastomosis on two chidren suffering from this disease; the postoperative followup of one of them now extends above five years. The operation resulted in almost complete disappearance of
the cyanosis and gxat improvement in
exercise tolerance in both cases.
DEFECTIVE
DEVELOPMENT
RIGHT\'ENTRICI.E
Application of cava-pulmonary anastomosis was reported by Gasul and m i ates"'" in this anomaly. There are two
main types in this malformation; in one
the right ventricle is abnormally small and
OF T H E
In Ebstein's disease, the tricuspid valvular apparatus is misplaced into the midportion of the right ventricle, reducing its
functional capacity. It is usually a w i a t e d
with atrial septa1 defect and right-to-left
shunt. The symptoms of this disease are
commonly severe and p r o p i e before
the patient4 develop conptive heart failure.
S!~temic-pulmonary anastomosis does not
relieve the symptoms, and closure of the
septal defect may even be disastrous." A
significant number of patients have been
reported""s"'
in whom the cava-pulmonary anastomosis brought considerable improvement and even complete disappearance of symptoms.
We have used this procedure on one patient with Ebstein's diseaw with the mast
gratifying rsults.
In this unusual combination of anomalies, the pulmonary obstruction protects
the lung, but also greatly reduces the volume of pulmonary circulation. Pulmonary
stenosis is always valvularU since there is
no i n f u n d i b u l a r chamber. Patients with
this malformation are highly suitable for
cara-pulmonary anastomosis and dramatic
improvemenu with long lasting palliation
can be expected."""'~"
F ~ o u l s5: Re- ( A ) (upper) and postoperative
(B) (lower) mgiocudiognm of a child with rtcnark of the right main branch of the pulmonary
anery, ventricular septa1 defect and right-sided
aortic ~ h T h.e ~icturetaken one-half war after
creation of cavr~ulmonary anastom& show. r
p o d filling of the right pulmonary artery fmm the
superior caval vein.
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CAVA-PULMONARY ANASTOMOSIS
usually the pulmonary orifice is stenotic. In
the other type, the right ventricle is normal
in caliber, but it has a thin, flabby wall
which cannot work as an effective pump.
The s)stemic-pulmonary shunting proced u r n do not offer benefit in either of these
situations and, according to Tauwig, they
should be treated by cava-pulmonary anactomosis."
In this condition, the pulmonar! valve
and the main pulmonary trunk are normal,
but one or Imth main branches have more
or less extensive stricture. The anomaly is
rare and it usually occum in combination
F I O U6:
~
Preoperative a n g i o c d i o g ~ lof a
two-leu-old girl with t a n l o g y of Fallot m d atrial
v p u l defect. ( A ) (upper) Note the avucular
lungs and the filling of ihe d c a n p o v d aorta fmm
the nght ventricle The andocardiogram taken one
m u after ewa-oulmonuv anastomosis IB) ( l o r &)
shows exceilent filling of the right pulmonary
vnrelr.
with other deformities of the heart and
great vesxh.
One of our patients, a one-year-old girl,
had not only stenosis of the right main
hranch of the pulmonary artery, but also
a large ventricular septa1 defect and rightsided aortic arch. She was significantly benefited front a cava-pulmonary anastommb.
The functional results of this operation
in tetralogy of Fallot are reported to be exc e l l e n t , ~ . w . ~ . m . Our
n ~ ~ ~experiences
.~
on
nine patients have been most rewarding.
Patients suffering from this disease can be
divided into three ~grouroups:
1. Those with an anomaly which is
technically operable and w h m general
condition offers a reasonable chance for
survival with romplete repair.
3. Those unsuitable for direct surgical
attack (for example, hypoplasia of the
main pulmonary artery, a k n c e of the pulmonary valve, etr.).
3. Those who are technically operable,
but becauu of their small size or poor general condition, there is a temporary contraindication for complete surgical repair.
It has been our practice not to perform a
palliative procedure on patients in group
one, but to do a complete repair under cardiopulmonary bypas. The mortality rate
in these procedures is still disproportionally
high as compared to other forms of congenital heart diseaw. An alarming rate of
recurrence has also been reported by the
most competent ~ u r g e o n s ; " ~ "therefore,
~
some surgeons are discouraged from ever
doing complete repairs of tetralogy of Fallot and turn to palliative procedures, i.e.,
Blalock, Potts anastomosis or cava-pulmonary shunts. We do not agree with this
practice.
There can be no doubt that when a completely corrective procedure is technically
impossible, one of the palliative operations
should be performed. In this group we
prefer the cava-pulmonary shunt over s)stemic-pulmonary anastomosis.
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1
jo
P A U L W . SASCER,
The g r e a t e s t difficulty is in deciding
which procedure to select in the third
group. These are the patients who cannot
withstand open heart surgery but may become candidates for complete repair in the
future. \\'e believe that the cava-pulmonary
shunt gives the best palliation in this g ~ o u p .
The procedure. however, has a disadvantage which does not exist with the Blalock
or Potts anastomaws. The division and
anastomosis of the two p a t vessels in cavapulmonary anastomosis creates a situation
which is \,en difficult, if not impossible, to
reverse.' T h e q u e s t i o n arises, however.
whether it ic necesqay to do an)rhing with
the cava-pulmonary shunt at the time of
the open repair. IVhat will happen to the
patient as he grows older and develops emphyerna? IViU the anastornocis remain
open indefinitel)? Until all of these q u e
tions can he satisfactoril) answered. the
operative indication in this group will remain controversial. It is encouraging that
neither we",UY nor then"""^'" have obwried any harmful effects from the anastomosis in long range experiments and clinical observations of over four years.
Theoretically, the caoa-pulmonary anastomosis would be beneficial although there
is no reported case of appl>ing this procedure in isolated pulmonary stenosis. The
reason for this is that direct attack on the
stenosed pulmonary valve with the closed
technique can be performed on the desperately ill patients. If the stenosis is infundi-
el al.
otwaw, "I
I ~ CChert
bular or asociated with hvpoplzsia of the
main pulmonan arten;, this is a situation
where the cava-pulmonary shunt is the procedure of choice. The same is true for
lhrotnbosir of the orrgin of the right pulmonary artery, irremorable tumors of the
right atrium, and sznple i.entricle with pulmonary stenosis.
The quextion ariws that if the superior
cava flow can he bypassed. why not b!pasc
the entire right heart hy performing both
superior and inferior caval shunt? Interestingh enough. the results of experimental
inferior cava to pulmonary arten shuntc
have been extrernel\ discouraging." We
have not been able to keep an animal alive
longer than 30 minutes when both cavas
%<.ereanastomoced to the p u l m o n q circulation. The right heart, however, has
heen b y p m d for ac long as eight days b)
doing a s u p e r i o r vena cava-pulmonary
anastomosis and transplanting the inferior
vena cava into the left atrium.% These experimentq were intended onl) to investigate
physiolo+c facts: however, this combination of procedure* might be useful in complete repair of tranrporztion of great aessell. Naturally, in this condition the blood
flow from the pulmonan. veins should be
redirected into the arterial (right) atrium,
which should not create a special technical
problem. Another possibility is to use the
cava-pulmonary anastomosis in t r a n s p i tion; that is, in combination with the BafIes'
procedure.
Frcuar 7 :
Pressure mekluremenu in the superior vena cava (upper curve) and right pulmonary a r t c v
(lover curie) one year following cava-pulmonary anastomosis The elevation of the venous pressure is only moderate. The effect of breathing on the b l w d flow is also well demonstrated.
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V o l u m 48. ho 2
A u p ~ I%(
t
CAVA-PULMONARY ANASTOMOSIS
Approximately half of the patients exhibit some degree of facial edema. This
swelling is usually not severe and disap
p e m in the first two weeks. This swelling
could be prevented or its disappearance
hastened by keeping the patients in a semisitting position in the first few days after
surgery.
Persistent cyanosis and swelling of the
upper part of the body indicates occlusion
of the anastomosis. So far we did not experience such a complication.
Chylothorax as a postoperative complication is mentioned by several authorsm'*
and it occurred in two of our patients. Both
of them reacted well to chest acpirations.
The development of chylothorax following
cava-pulmonary anastomosis is not completely understood. It could be due to the
elevated venous prescure or to the injun of
minute I!mphatic channels during the mediastinal dissection.
In our previous experimental and clinical studiesu we have demonstrated that the
development of "a:ygor steal s)ndrome" is
a very undesirable complication of caoapulmonary anastomosis. The aqgos is the
greatest collateral channel between the superior and inferior caval veins. .After the
anastomosis is c o m p l e t e d , it should be
ligated as it doec not increase the blood
flow through the ana3tomosis; instead it
significantly drains the superior \,ens cava
and thus steals blood that would go to the
pulmonary artery to be oxysenated. This
complication could be easily prevented by
ligating the azygos vein after the cornpletion of the anastomosis.
If one of the two involved vessels, namely the superior vena cava or the right pulmonary artery, is too small for suitable
anastomosis, the operation should not be
performed. Such an anastomosis inevitably
leads to a "superior vena cava syndrome."
The small size of the patient is not regard-
'5'
ed as an absolute contraindication for surgery. Edwards and Bargeron" demonstrated that good results could be obtained even
at the age of 14 weeks. O u r youngest patient was 12 months old.
The main contraindication for this procedure is the presence of elevated pulmonary arterial prexcure. Increase in pulmo.
n a n vascular resistance creates a situation
with which the venous p m u r e is unable
to cope. T o disregard this fact leads to
tragic consequen~es.~
\\'hen the patient's anomaly is of such a
nature that a completely corrective procedure is possible with reasonable rick, t h i ~
operation is contraindicated.
The hemodynamic effects of superior
vena cava to right pulmonan artery anastomosis are briefly presented. This operation may be beneficial in congenital heart
conditions in which there is an obstruction
of blood flow in the right heart or pulmonary circuit or because of abnormal routing, or when the venous blood flow to the
l u n , ~is decreased.
This procedure has been successfully applied in tricuspid stenosis, non-functionins
right ventricle. peripheral stenosis of the
pulmonan artery, Ebstein's disease, transposition w i t h p u l m o n a r y ctenosis, and
tetralop of Fallot. The operative results in
these conditions have been satisfactory.
The merits and disadvantages, as well as
p a ~ i b l efuture applications of this operation, are briefly d i s c u s ~ d .The main contraindicationc for this procedure are: blood
vwels too small for anastomosis and increased pulmonary vascular resistance. This
procedure s h o u l d not be applied if the
anomaly can be completely repaired with a
reasonable operative risk.
REsurfk
Ler essais h&rnad,namiques de I'anartomose enIre le veine cave rup6riere et I'anCre pulrnanaire
droite sont brikement prCsent6er. Cette operation peut rendre senice dans des maladies rardiaques congenitales dans lequelles il y a une
obstruction du Rot sanguin dans le circuit pulmonaire ou le coeur droit. au parce qu'il y a une
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voie anormale, ou lorsque le quantitC de sang
veinew: se rendant aux poumons est diminuCe.
Cette opCration a CtC utilidr avec s u c c b dans
la stCnose tricwpidienne le ventricule droit nonfoncriomel, les stCnoser d r i ~ h C r i a u e sdes a r i h e s
pulmonaires, la maladie d'Ebrtein, la transporntion avec stCnow pulmonaire, la tCtralogir d r
Fallot. Les rbultats opCratoim d a m re* maladieoni CtC satlfaisants.
Les mCrites et les dhavantages, a u s i hien que
I n applicatifutures pmsihln de rette opCration, snnt brihement tiudiCn. Les contrc-indirations .
principals
B I'op&ration soni: d a vaiaeaux
.
sanguins trop petirs pour I'anastomou, r t trnc
rbistance ~ s c u l a i r e p u l m o n a i r e a u m e n t h .
Cetie opCration a Ctk utiliste avec succ.5 dans
I'anomalie peut Ctre compl&tement r6parCe avec
un risque opCratoire raisonnahle.
. .
.
Die himodynamischen A u s w i r k u n g e n ciner
Anastomore M.ischen der Vena cava ruprior und
der Aneria pulmonalis sind kltrz dargestellt. Diese
Operation k a n n n i i t ~ilr h spin hei kongenitalen
Herzfehlem, bei denen der BlutfluP in das re.
Herz oder den Pulmonalkreislauf k h i n d e n ist,
auch als Folge ahnomr entrpringender CeTdk
oder w e m der ven6se RiirkfluP zo den Lungen
herabgesetzt isr.
Diese MaPnahme wurdc erfalgreich durchgefiihn k i Tricuspidalstenore. funktionslaem re.
Ventrikel, peripherer Pulmonalstenosr. EpseinSyndmm, Transposition mit Pulmonalstenosr und
Falot'scher Teiralogie. Die operaiiven Erfalge
waren k i diesen Erkrankungen zufriedenstellend.
Die Vor - und Narhieile wwie die meglichen
kiinftigen Indikatinnen diewr Operation rind k u n
diskutien. D i e Hauptkontrindikationen d i e s e l
Verfahrens sind: ru kleine BlutgrfaPe fiir die
DurchWhrung der Anastomow und crhshter pulmonaler Gefabwiderstand. Aurh s o l l t e d i e s e
Methode nicht durchgefiihn werdm, wlange eine
Anomalie mit en<aglichem Operationsrisiko volls t h d i g k h o k n werden kann.
E
r
e reference lirt will appear in reprints.
,p~ts,
na,charlotte,
N,,&
carolin.,
Sanger, 1850 Eplt
PULMONARY CHANGES IN MALIGNANT TROPHOBLASTIC DISEASE
In W e n Africa and paN of the far Mlt. maligcan play an important part in the earlier remgninant tmphoblastie 6lreluc Is freguent in mmpariron
lion of this dlseasp as patlents may freguently p r e
with the incidence m o d e 6 In m m p e and the
sent a medical rather Ulan a gynemlogle problem.
United States. Thls mmmunlcatlon is based on a
elinleal and radiologie study of g6 patients with
Errsr. K T.. C o u r ~ o r r W.
,
P. AND DI V H r ~ m l c r s r .
mall-t
Mphobla~Ue 6l.cseen in the UnlP "Puimon,ry Chmges m Malrgnmr Tiophoblmic DISvenity College, Ibdan. Nlgeria The radiologM
clw." Bnr. I. R d l o l . . 38.161. IWI.
TACHYPNEA AND IiYPERPNEA
Resting tachypnea or hyperpnea wru rtudled in 11
emdent ventilation. It is luggnned that patients
with tachypnea or hyperpnea should be puelitioned
patlents. A hlstory of exertionel dyspnea war frequent. The hydrosen Ion m n a n m u o n of the arclosely for dyrpnea, and their puimonar). response
terial blmd wru normal, and the arterial blmd carto exercise should be tested to characterize and
Mn dlexlde tension and bicarbonate mncenmtlon
quantitate thelr llmltations of function. Possible
showed moderate reductions. Expiratory flow rates
pathophysiologic mechanlmr Include maldlstributlon
of ventilation asroeiated wlth pulmonary departltlocwere slightly reduce6 and improved after nebuU7.4
isopmterenol. Demared alveolar to expired ventllaing in crltlail
and loss of distal pulmonary
Uon ratlo. Increase6 ventilatory equivalent for oxyvessels. eswclall? alveolar capillarler.
pen. and reduced dlfluslng capacity for m M n monKILBUIN. K. H ' ' T I c ~ ~ and
~ c *H-ocr."
Ann. I s r .
oxlde during steady state exercise demonstrated inN r d . . 62 486. I%>.
MISLEADING SIGNS IN MITRAL INSUFFICIENCY
of pulmonary hypertenston a. a
with t r l ~ r p i d insumcieney. In revlewlng 125 pammptlcation of rhevmaue heart dlseasc Is important
tients with proved mitml regurgltatlon by cardiac
In determining the patient's promorla. Progressive
ratheteriratlon, 22 demonstrated all the phydml tlndright venVicular failure and pulmonary thrombcings of right venVLNlar and p u l m o m artery hycmbollc dlhave been o h w e d to be frequent
peractivity without elmation of right heart p w
mmplications remlung In death Most pstieats who
sums or ejection volume. The elinleal impression of
exhibit the m a 6 of right ventricular heave. palpable
pulmonary hypertenslan was in error in ail cases.
pulmonary outdow tract. and Inme P2 M U have
pulmonary hypeNmlon or r@ht ventricular hyperMANCHES
G~
. H..
~ , Brocx. P. &ND G 0 . u ~ . R.: " M d n d rn S y u ,I, M d Iluufh<*rr,." I M I A . 191:87. 1 % ) .
activity due to I n e u c d right ventricvlar flow. as
The -mition
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