Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan Kreutzer) -Florentino J. Vargas et al.

J
THoRAc CARDIOVASC SURG
1987;93:523-32
Anomalous systemic and pulmonary venous
connections in conjunction with atriopulmonary
anastomosis (Fontan-Kreutzer)
Technical considerations
Anomalom systemic or pulmonary venous connections can coexist with certain forms of complex
cyanotic heart diseases that are reparable only by atriopulmonary anastomotic procedures, thus
compticating the intraatrial separation of systemic and pulmonaryvenous pathways. Anomalous systemic
or pulmonary venom connections were encountered isolated or in combination in 17 patients (10%)
among a series of 170 modified Fontan-Kreutzer procedures. Fourteen of these 17 patients (82.3%)
survived their operations, which utilized different techniques to deal with the various forms of anomalous
systemic and pulmonaryvenous connections. There was one late death (5.8%). Extracardiac exclusionof
a left superior vena cava with an end-to-side left cavopulmonary shunt proved to be a more successful
alternative than the use of complicated intratrial baflles. Because of the complexity of the anatomic
variables, repair of anomalous systemic or pulmonary venous connectionsin conjunction with a modified
Fontan-Kreutzerprocedure requires a detailed preoperativeanatomical and physiologic diagnosis,and an
individualized plan for each patient must be formulated to provide unobstructed venous pathways.
Florentino J. Vargas, M.D.,* John E. Mayer, Jr., M.D., Richard A. Jonas, M.D., and
AIdo R. Castaneda, M.D., Boston, Mass.
Although the Fontan-Kreutzer operation was originally conceived for physiologic correction of tricuspid
atresia.I, 2 the procedure has since been modified and
extended to other more complex lesions.':" Included
among these are anomalies in which rerouting of the
pulmonary and systemic venous pathways is necessary." 7,12,13 The coexistence of anomalies of systemic
venous and pulmonary venous connection poses both
technicaldifficulties at repair and an increased operative
mortality." 12, 13 The purpose of this article is to outline
several technical aspects of the surgical management of
patients in whom rerouting of systemic and/or pulmonary venous return was required in combination with a
modified Fontan-Kreutzer operation.
From the Department of Cardiovascular Surgery, the Children's
Hospital; and the Department of Surgery, Harvard Medical
School, Boston, Mass,
Received for publication March 17, 1986,
Accepted for publication April 8, 1986,
Address for reprints: Aldo R, Castaneda, M,D" Department of
Cardiovascular Surgery, Children's Hospital, 300 Longwood Ave"
Boston, Mass, 02115,
Patients
Between August 1981 and October 1985, 17 patients
with various types of anomalous systemic and pulmonary venous connection had a modified Fontan-Kreutzer procedure. Twelve were male and five were female
(2.4:1). Ages ranged from 16 months to 27 years (mean,
9.6 years). Nine patients were aged 7 years or younger.
Segmental cardiac anatomy" was diagnosed as {S,D,D}
in seven patients, {A,L,L} in four, {A,D,D} in four, and
{S,L,L} in two. Ten patients had a common atrium.
Eleven had a common atrioventricular (AV) valve. Two
had double-inlet AV valves, two had a straddling right
AV valve, and two had either stenosis or atresia of the
left AV valve. Dextro-loop of the ventricles occurred in
11 patients (64.7%). Ventricular anatomy included a
single ventricle in 14 patients, hypoplastic left ventricle
in two, and hypoplasticright ventricle in one. Pulmonary
stenosisor atresia was present in all but one patient. This
patient had had pulmonary artery banding as part of a
complex palliative procedure. The details of the systemic
and pulmonary venous anomalies for each patient are
found in Table I. The systemic venous anomalies
included persistent left superior vena cava (SVC) drain523
The Journal of
Thoracic and Cardiovascular
Surgery
524 Vargas et af.
Table I. Surgical management of systemic anti/or pulmonary anomalous venous drainage in patients who had a
modified Fontan procedure (N = 17 patients)
Patient
Previous
operation
Age
5 yr
Diagnosis
Anomalous venous drainage
Central shunt
Polysplema {A,L,L} common atrium, CA VV,
SV, DORV, PS
Asplenia, {A,D,D}, common atrium, CA VV,
2
22 Mo
RBTS
3
27 yr
LBTS Waterston
4
6 yr
RBTS
5
16 mo
None
{A,D,D}, common atrium, double inlet--<iouble
outlet SV, PS
SV, D-TGA, PS
Asplenia {S,D,D} common atrium, CA VV,
SV, D-TGA, PS
{A,D,D}, common atrium, CA VV, SV,
D-TGA, PS; dextrocardia
6
9
RBTS
Asplenia {A,L,L} common atrium, CA VV,
SV, L-TGA, PS
7
5 yr
LBTS
{S,D,D}, common atrium, CA VV, SV,
D-TGA, PA, JLAA
8
10 yr
RBTS
{S,D,D}, common atrium, CAVY, SV,
D-TGA, PS, LJAA
Bilateral SVC (LSVC to LA side), interrupted
IVC (az. cont. to RSVC) hepatic veins-left
side common atrium; CS absent
Bilateral SVC (LSVC to LA side); IVC-LA
side; CS absent
Bilateral SVC (LSVC to LA side); CS absent
Total APVR to right side of common atrium;
bilateral SVC, (LSVC to LA side) IVC-LA
side; CS absent
Bilateral SVC (LSVC to LA side); interrupted
IVC (az. cont. to RSVC) hepatic veins to
right side common atrium; CS absent
Total APVR to LSVC-LA side; absent RSVC,
IVC-LA side; CS absent
Bilateral SVC (LSVC to LA side); interrupted
IVC (az. cont. to LSVC) hepatic veins-left
side common atrium; CS absent
Bilateral SVC (LSVC to CS)
Legend: APVR, Anomalous pulmonary venous return. az. cont, Azygos vein continuation. CAVY, Common atrioventricular valve. CPV, Common pulmonary vein. CS,
Coronary sinus. D-TGA/L-TGA, Dextro/levo transposition of the great arteries. DORV, Double-outlet right ventricle. FC, Functional class. HLV, Hypoplastic left
ventricle. IVC, Inferior vena cava. JLAA, Juxtaposed left atrial appendages. LA side, Left atrial side. LAVV, Left atrioventricular valve. LBTS, Left Blalcok-Taussig
shunt. LCO, Low cardiac output. LlVC, Left inferior vena cava. L/RSVC, Left/right superior vena cava. NYHA, New York Heart Association. PA, Pulmonary atresia.
PAB, Pulmonary artery banding. PS, Pulmonary stenosis. PVR, Pulmonary venous return. RA, Right atrium. RAVV, Right atrioventricular valve. RBTS, Right
Blalock-Taussig shunt. RPA, Right pulmonary artery. sp ADKSR, Previous Alvarez-Damus-Kaye-Stanscl repair (restrictive bulboventricular foramen) followed by
RBTS. sp APWR, Previous aortopulmonary window repair. sp. CoAR, Previous coarctation repair. sp IAAR, Previous interrupted aortic arch (type B) repair (10 mm
conduit). SV, Single ventricle. SVC, Superior vena cava. SVR, Systemic venous return.
ing into the left atrium (seven patients) or draining to
the coronary sinus (seven patients), 'left-sided entrance
of the inferior vena cava (lVC) (four patients), and IVC
interruption with azygos continuation (three patients,
two of them with hepatic veins connecting to the left side
of a common atrium). Pulmonary veins connected
anomalously to a left vertical vein, which drained to the
innominate vein (one patient), to the right side of the
atrium (two patients), and to a left SVC (one
patient).
Surgical techniques
A pressure line was routinely placed in the left jugular
vein when the diagnosis of bilateral SVC was made
preoperatively. All patients were placed on cardiopulmo-
nary bypass; deep hypothermia «20 C) and circulatory arrest were utilized with 10 patients, and in the
remaining seven patients, cardiopulmonary bypass with
hypothermia to 25 0 C was used. Cold crystalloid
cardioplegia was added during aortic cross-clamping
and repeated every 30 minutes. The persistent left SVC
was cannulated (either directly or through the coronary
sinus) in only two instances.
The atrium was opened through an incision 1 em
away from and parallel to the AV groove; it extended
from the base of the right atrial appendage to the right
atrium-IVC junction in most cases (Fig. 1, A). In all but
one patient, in whom an intraatrial tube graft was used,
an intraatrial baffle was placed to direct pulmonary
venous return to either a common AV valve or to the
0
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Fontan-Kreutzer operation
525
Results
Procedure
Patch diversion PVR-CAVV (LSVC, RSVC, and hepatic
veins to the right); modified Fontan (atriopulmonary
conduit interposed)
Patch diversion PVR-CAVV (LSVC, RSVC, and IVC
to the right); modified Fontan
Patch diversion PVR-CAVV, (LSVC, RSVC, and IVC
to the right); modified Fontan.
Intramural tract opened (unroofed) displacing the APVR
entrance to the left. Patch diversion APVR orifice
to CA VV (LSVC, RSVC, and IVC to the right);
modified Fontan.
LSVC exclusion (modified Glenn); patch diversion PVR-RAVV
(RSVC and hepatic veins to the right); modified Fontan
Divided LSVC distal to APVR; proximal end closure
(APVR connected to LA side); distal end anastomosed
to LPA (modified Glenn); patch diversion of LSVC-APVR
entrance to CA VV (lVC to the right); modified Fontan
(atriopulmonary conduit interposed)
LSVC and azygous anastomosed to LPA (modified
Glenn); patch diversion of pulmonary veins to coronary
(RSVC and hepatic veins to right)
Patch diversion PVR-CAVV, CS (and LSVC) to the
right by suturing the patch to the anterior inner aspect
of the CS; modified Fontan. Reoperation 24 yr (PVR
obstruction); baffle replaced (isolating RSVC and
IVC); LSVC excluded (modified Glenn)
Early
Late
Death; SVR obstruction;
conduit compressed by
the sternum
Death; SVR obstruction
Well (2 yr); FC (NYHA)
Well (5 mol; FC
I (NYHA)
Well (2 yr) FC I(NYHA)
Well (4 mol Recatheterized
r /0 right/left
sided ob.
Well (45 days)
peath (LCO)
Continued.
right AV valve (Fig. 1, B). Systemic venous return was
kept on the functional right side of the baffle for direct
connection to the pulmonary artery. Placement of the
atrial baffle varied, according to the various types of
anomalous systemic or pulmonary venous connection.
Anomalous systemic venous connection
Three anomalies of the systemic venous return were
encountered, which necessitated alteration of our standard technique of atriopulmonary anastomotic procedure.
In the first group (Cases 1 to 7), the left SVC drained
directly into the left side of the atrium. Two different
operative approaches have been utilized. In our initial
cases (Nos. 1 to 4), an intraatrial baffle was constructed
to divert all systemic venous return to the right side of
the atrial baffle and to direct all pulmonary venous
return to the AV valve or valves. In three of them,
additional systemic venous drainage from the lower half
of the body (hepatic veins in Case 1 and IVC in Cases 2
and 4) also entered the left side of the atrium. As a
consequence of the venous connections, the patches
necessary to separate pulmonary from systemic venous
return had a complex configuration. Two of the four
patients in this subgroup died (Cases I and 2), both with
low cardiac output caused by obstruction of systemic
venous return. The remaining two patients did well. The
last three patients operated on for left SVC connection
to the left atrium (Cases 5 to 7) have been managed by
division of the left SVC and construction of an endto-side anastomosis between the left SVC and left
pulmonary artery (bidirectional modified Glenn shunt)
(Fig. 2). One of these patients also had total anomalous
pulmonary venous connection to a left SVC, draining
into the left atrium (Case 6). The proximal left SVC
was simply oversewn. All three patients survived.
In the second group, the anomalous left SVC drained
to the coronary sinus. In four patients (Cases 8 to 11),
the atrial baffle separating systemic and pulmonary
venous blood was sewn to the anterior lip of the coronary
sinus to avoid the area of the AV node and to leave the
coronary sinus orifice on the right atrial side of the patch
The Journal of
Thoracic and Cardiovascular
Surgery
526 Vargas et af.
Table I. Cont'd
Patient
Age
Previous
operation
Anomalous venous drainage
Diagnosis
9
22 yr
RBTS
{S,D,D}, straddling RAVV, hypoplastic RV,
D-TGA, PS
BIlateral SVC (LSVC to CS)
10
19 yr
BTS
{S,L,L}, double-inlet SV, LTGA, PS
Bilateral SVC (LSVC to CS)
II
8 yr
PAB
{S,D,D}, CAVY, SV, D-TGA, JLAA
Bilateral SVC (LSVC to CS)
12
16 mo
sp APWR; sp
1AAR
{S,D,D}, LA VV stenosis, HL V, PS
Bilateral SVC (LSVC to CS)
13
7 yr
Septectomy
Asplenia {S,D,D} straddling RA YY, SY,
D-TGA, PS
Bilateral SVC (LSVC to CS)
14
5 yr
sp CoAR; sp
ADKSR
{S,L,L}, LA VV atresia, hypoplastic outlet
chamber
Bilateral SVC (LSVC to CS)
15
5 yr
None
Heterotaxy-{A,D,D}, CAVY, DORV; PS
RSVC, IYC-LA side
16
19 yr
Land RBTS
Asplenia {A,L,L}, common atrium, CAVY,
SV, L-TGA, PS
Total APVR to LSVC to innominate veins;
retrocardiac CPV
17
12 yr
LBTS
Asplenia {A,L,L}, common atrium, CAVY,
SV, L-TGA, PS
Total APVR to RA; no LSYC; CS absent
(Fig, 3). Three patients survived. A fourth patient (Case
8) died with pulmonary venous obstruction. The atrial
septal patch was redundant and could have potentially
limited the pulmonary venous-common AV valve pathway. In the three remaining patients with left SVCcoronary sinus connections (Cases 12 to 14), the potential for a patch passing between the coronary sinus and
the right AV valve anulus to bow to the left (since right
atrial pressure would exceed left atrial pressure),
obstruct the valve orifice, and cause pulmonary venous
obstruction was recognized. In these cases an end-to-side
left SVC-left pulmonary artery anastomosis was done,
and the intraatrial patch was placed well away from the
level of the right AV valve. The coronary sinus orifice
remained on the left (pulmonary venous) side of the
patch. All three patients survived this operation.
One final patient with anomalous systemic venous
connection had an IVC draining to the left side of the
common atrium and a right-sided SVC (Case 15). The
complete atrial baffie required to divert the pulmonary
veins to the common AV valve had to separate both the
right-sided SVC and the left-sided IVC would have
obstructed the pulmonary venous pathway. This patient
was successfully managed by placing an intraatrial tube
graft between the left IVC and right SVC and then
connecting the upper end and the side of the graft to the
right pulmonary artery. The pulmonary veins drained to
the common AV valve around the tube graft (Fig. 4).
Anomalous pulmonary venous connection
Four different types of anomalous pulmonary venous
return were encountered, In the first, total anomalous
pulmonary venous connection drained to a persistent left
vertical vein, which connected to the innominate vein
(Case 16). In this patient, a side-to-side anastomosis was
created between the common pulmonary vein and the
posterior left atrial wall, followed by atrial baffiing of
the newly created pulmonary venous connection to the
common AV valve (Fig. 5). The IVC and right SVC
flows were collected within the right-sided systemic
venous chamber and connected to the right pulmonary
artery by direct anastomosis. In the second case (Case
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Fontan-Kreutzer operation
5 27
Results
Procedure
Patch diversion PVR to RA VV, CS (and LSVC) to
the right by suturing the patch to the anterior inner
aspect of the CS; modified Fontan
Patch diversion PVR to RA VV, CS (and LSVC) to
the right by suturing the patch to the anterior inner
aspect of the CS; modified Fontan
Patch diversion PVR-RA VV, CS (and LSVC) to the
right by suturing the patch to the anterior inner aspect
of the CS; modified Fontan
LSVe exclusion (modified Glenn); patch diversion PVR
to RAVV (RSVC and Ive to the right); es to the
left; modified Fontan
LSVe exclusion (modified Glenn); patch diversion PVR
to RAVV (RSVe and IVC to the right); es to the
left; modified Fontan
LSVe exclusion (modified Glenn); patch diversion PVR to
RAVV (RSVe and Ive to the right); es to the
left; Modified Fontan
Intracardiac tube graft to connect LIVe to RSVC;
RSVC + RA anastomosis to RPA.
CPV-posterior atrial wall anastomosis; LSVC ligated;
patch diversion of anastomotic orifice to CA VV (RS
and CV IVC to the right); modified Fontan
Patch diversion of total APVR to CAVY (RSVC and
Ive to the right); modified Fontan
17), all of the pulmonary venous return entered the
heart at the right atrium-right SVC junction. An
intraatrial baffle was placed, rerouting the pulmonary
venous return to the common AV valve. (IVC and right
SVC were excluded to the right.) The area of potential
obstruction to right SVC flow was subsequently
enlarged during creation of the atriopulmonary anastomosis(Fig. 6). In a third case with associated anomalous
systemic venous connection (Case 4), the pulmonary
veins entered the heart on the right side of a common
atrium at the midatrial level. The pulmonary veins
joined to form a posterior common vein that was
incorporated into the posterior wall of the atrium (distal
to the atrial orifice of the vein). This intramural channel
of the common pulmonary vein was unroofed, which
widely opened the entrance of the anomalous pulmonary
venous connection toward the left side of the atrium.
The pulmonary venous flow was then directed to the
common AV valve by an intraatrial baffle sutured
within the original orifice of the pulmonary veins and
then to the anterior border of the atriotomy. The
Early
Late
Well (18 mol FC
I (NYHA) Late
drainage (pleural eff.)
Well (18 mol FC
I (NYHA)
Well (4 mol FC
I (NYHA)
Well (2 yr 7 mol
Fe I (NYHA)
Well (I yr) Normal
hemodynamics
(recatheterization)
Well (40 days)
Well (2 yr) FC I
Death (third month);
iliac vein; thrombosis;
pulmonary embolus,
2-D echo, rio
R/L-sided obs.
Well (18 mol CF
I (NYHA)
bilateral SVC and IVC flows were thus channeled into
the systemic venous compartment. In a fourth case also
with anomalous systemic venous connection (Case 6),
the pulmonary veins drained to a persistent left SVC,
which entered the left atrium. There was no right SVC.
The left SVC was divided distal to the entrance site of
the pulmonary veins. The atrial end was closed, leaving
the total anomalous pulmonary venous connection
draining to the left side of the atrium (via the proximal
left SVC). The distal left SVC was anastomosed end to
side to the left pulmonary artery (bidirectional cavopulmonary shunt). The stoma of the left SVC into the
atrium (now draining all the pulmonary veins) was then
diverted by an atrial septal patch to the common AV
valve, which isolated the IVC into a systemic venous
chamber (Fig. 7) connected to the left side of the main
pulmonary artery by a conduit.
The above-outlined baffle procedures were followed
by a direct "right" atriopulmonary anastomosis in 15
patients. In the remaining two patients, a systemic
venous atrium-left pulmonary artery conduit had to be
The Journal of
Thoracic and Cardiovascular
Surgery
528 Vargas et al.
Incision
Fig. 1. Surgical technique for intraatrial septation in modified Fontan procedure. A, Incision runs parallel to the
interatrial groove. B, Atrial septal patch diverts the pulmonary
venous flow to the common or right atrioventricular (AV)
valve, separating the systemic venous return into an anterior
chamber. Because the anterior border of the patch is attached
to the anterior lip of the atriotomy (coronary sinus to the left),
the suture lines are placed away from the conduction tissue.
interposed because of {I,L,L} and {A,L,L} configurations. In these two patients, the main pulmonary artery
was divided, its proximal end oversewn, and the distal
stoma was used as a part of the anastomosis between the
atrium and conduit.
Early results
There were three patients who died during hospitalization (17.6%). The first death (Case 1) occurred in a
patient with complex intraatrial anatomy (bilateral
SVC, hepatic veins draining to the left side of the
atrium). The autopsy revealed obstruction of the systemic venous pathways and also external compression of
the nonvalved conduit used to connect the systemic
venous chamber with the left pulmonary artery. The
second hospital death occurred in a patient in whom the
systemic venous return (bilateral SVC, IVC into the left
atrial side) had been excluded with a complex intraatrial
patch. He remained in severe low cardiac output after
the operation, with signs of obstruction to the systemic
venous return. He died 48 hours later (Case 2). The
third hospital death (Case 8) occurred in a patient with
a left SVC connected directly to the coronary sinus. This
patient required reoperation within the first 24 hours
because of obstruction to the pulmonary venous return.
The redundant atrial patch was obstructing the pulmo-
Fig. 2. Surgical technique (Cases 5 to 7). A, Left superior
vena cava (LSVC) draining into the left atrial side. B, The
LSVC was divided, its proximal end suture ligated (arrow),
and the distal end anastomosed end-to-side (arrow) to the left
pulmonary artery (bidirectional cavopulmonary shunt). The
main pulmonary artery was divided, its proximal end suture
ligated, and the distal orifice enlarged toward the right
pulmonary artery. The pulmonary veins were diverted toward
the common or right AV valve by an intraatrial septal patch
attached to the anterior lip of the atriotomy. C, A modified
Fontan procedure was completed.
nary venous return at the coronary sinus entrance site.
The left SVC was divided, and an end-to-side bidirectional cavopulmonary shunt was performed. The original baffle was also replaced by a geometrically simpler
patch, since the left SVC had been diverted to the left
pulmonary artery. However, the patient continued to
have a low cardiac output despite normal right and left
atrial pressures. He died within 24 hours after the
second operation.
The remaining patients did well. In the operating
room right atrial pressures ranged from 12 to 18 em
H 20 (mean, 16 em H 20) and left atrial pressures were
less than 7 em H 20 in all cases. All patients remained in
sinus rhythm postoperatively.
Late results
The mean follow-up period of the 14 survivmg
patients was 21 months, ranging from 40 days to 2 years
and 7 months. There was one late death (5.8%)
attributed to massive pulmonary embolism in a 19year-old patient (Case 16), who was receivingoutpatient
anticoagulant treatment 3 months after the operation
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4
Fig. 3. Surgical technique (Cases 8 to II). A, Left superior
vena cava (LSVCj to the coronary sinus (eS). B, An intraatrial
septal patch, which diverts the pulmonary venous flow to the
common or right AV valve, is sutured to the inner aspect of the
anterior wall of the CS and then to the anterior lip of the
atriotomy. Injury to the conduction tissue has been avoided in
this way. The CS-LSVC flow has been directed into a
right-sided systemic venous chamber. C, A modified Fontan
procedure was completed.
because of late postoperative iliac vein thrombosis. No
postmortem examination was obtained. However, a
two-dimensional echocardiogram obtained shortly
before death ruled out obstruction to systemic or
pulmonary venous pathways. Two patients with persistent pericardial and pleural effusions were recatheterized. Normal "right atrial" pressures without atriopulmonary gradients were demonstrated in both. They were
successfully managed by fashioning of a pericardial
pleural window in one patient and a pleural drainage in
the other patient. All 13 patients are currently in New
York Heart Association Class I.
Discussion
At the beginning of Choussat's and Fontan's" experience with atriopulmonary anastomosis for tricuspid
atresia, patients with abnormal systemic and pulmonary
venous return were excluded. With increasing experi-
Fig. 4. Surgical technique (Case 15). A, Inferior vena cava
(IVCj draining to the left side of the common atrium in a close
relationship with the pulmonary veins. Tailoring of complex
intraatrial septal baffle could result in obstruction to either
systemic or pulmonary pathways. B, An intraatrial circular
graft is sutured inferiorly around the orifice of the left IVC
and superiorly around the entrance of the right superior vena
cava (RSVCj and to the adjacent atrial roof. C, A modified
Fontan procedure was completed.
ence, the indications for a Fontan-Kreutzer operation
have been expanded 16-21 to include younger and older
patients, non-tricuspid atresia anatomy, and patients
with anomalous systemic and pulmonary venous connections." The latter associated anomalies represented 10%
(17 patients) of the total number (170 patients) of
modified Fontan-Kreutzer procedures done at our institution. The coexistence of the anomalous systemic
and/or pulmonary venous connections initially had been
recognized as increasing both operative morbidity and
mortality/- 7.12.13 This was also true in our experience.
However, after an initial disappointing learning curve,
results have been improved. We have learned that
intraatrial baffles constructed to divert a left SVC-Ieft
atrium connection to the right side of the atrium have a
significant potential (two of four cases) to cause either
systemic or pulmonary venous obstruction with consequent mortality.
When the left SVC drains to the coronary sinus, the
baffle can be carried along the inner and anterior aspect
of the coronary sinus, to exclude the coronary sinus-left
SVC, right SVC, and IVC into a right-sided venous
chamber. There was only one death among the four
5 3 0 Vargas et al.
The Journal of
Thoracic and Cardiovascular
Surgery
Fig. 5. Surgical technique (Case 16). A, Total anomalous pulmonary venous return to right superior vena cava
(via left vertical innominate vein). B, The common pulmonary vein was anastomosed with the posterior left atrial
wall (A), and the pulmonary venous return was thus directed to the common AV valve by an intra atrial septal patch
(arrow). The systemic venous return was therefore excluded into an anterior atrial chamber to be connected with
the pulmonary artery (modified Fontan). The left vertical vein was ligated.
Fig. 6. Surgical technique (Case 17). A, Total anomalous
pulmonary venous return (PVR) to the superior (RSVC)
cavoatrial junction. B, The orifice of the entrance was
channeled to the common AV valve (arrow). C, Right
atrium-pulmonary artery (PA) (arrow) anastomosis was completed (modified Fontan).
patients with this anatomy treated in this manner, and
this death was caused by pulmonary venous obstruction.
In patients in whom the right AV valve or common AV
valve is a critical component of the pulmonary venous
pathway, this baffle placement is potentially dangerous.
An extracardiac exclusion of the left SVC by a bidirectional end-to-side left cavopulmonary anastomosis seems
safer in this situation. This modified Glenn shunt can be
performed regardless of the site of drainage of the left
SVC, and it avoids manipulations around the AV node
area. The left SVC-Ieft pulmonary artery anastomosis
can be accomplished without cannulating the distal left
SVC by lowering the body temperature to 25° C,
reducing bypass flow, and occluding the left SVC for 10
to 15 minutes. This modified Glenn anastomosis is done
to facilitate separation of systemic and pulmonary
venous return in these situations of complex venous
anatomy; it is not proposed as a necessary or desirable
adjunct to the routine Fontan-Kreutzer operation.
The presence of an IVC draining into the left atrial
side poses a different problem. The use of an intraatrialtube graft to connect both IVC with the right SVC,
followed by an anastomosis of the upper part of the graft
to the right pulmonary artery (modified Fontan), as
done in Case 15, is an attractive alternative. This
procedure, previously reported at the Mayo Clinic," has
the advantage of being technically simpler than a
complex septation. It also minimizes the risk for venous
pathway obstruction, since the higher-pressure systemic
venous pathway is contained within the tube graft. (The
risk for baffle obstruction of the pulmonary venous
drainage is eliminated in this way.) However, the
long-term outcome of such a prosthetic intraatrial
tubular graft in terms of patency remains to be eluci-
dated."
Anomalous pulmonary venous drainage also can be
satisfactorily managed. The repair of total anomalous
pulmonary venous connection to the left SVC by the use
Volume 93
Number 4
April 1987
of a modified Glenn shunt to exclude the distal left
SVC, as accomplished in Case 6, simplifies the procedure and avoids pulmonary venous obstruction. It can
also be applied for anomalous pulmonary venous connections (partial or total) to the right SVc. A similar
rerouting of anomalous pulmonary venous connections
through the proximal end of a previously transected
right SVC was recently reported for total anomalous
pulmonary venous connections into the upper right
SVC,23 In the other cases, appropriate baffle placement
allowed separation of systemic and pulmonary venous
drainage. Whatever the technique used for rerouting the
anomalous pulmonary venous connection or anomalous
systemic venous connections the patches should be
geometrically simple and without redundancy to avoid
the possibilityof obstructing one or both pathways." To
minimize this possibility, a left atrial retroaortic extension of the atriotomy for the atriopulmonary anastomosis has been reported. 5. 25 We prefer instead to attach the
anterior portion of the patch to the anterior lip of the
atriotomy, which allows for a smaller and straighter
patch. (Fig. 1). In hearts with abnormal AV connections
(AV discordance, straddling, and double-inlet AV
valve), the course of the conduction tissue is usually
abnormal.v" Nevertheless, the AV node always
remains on the atrial side and closely related to the valve
anulus. Consequently, anchoring the atrial baffle to the
atrial incision rather than around the AV valve prevents
injury to the AV node and bundle. With the use of this
technique, we have not observed any AV rhythm
disturbances in these patients.
Preoperative echocardiographic and angiographic
studies are critically important to outline in detail the
pulmonary and systemic venous drainage pattern.
Unrecognized anomalous pulmonary venous connection
has been reported as causing serious hemodynamic
deterioration from pulmonary thrombosis or infarction
during the postoperative period in patients who had an
otherwise uncomplicated Fontan- Kreutzer procedure."
During the past decade, the use of the FontanKreutzer principle has been extended to include more
complex malformations than tricuspid atresia. Patients
with single ventricle and associated anomalous systemic
and/or pulmonary venous connection can now also have
a modified Fontan-Kreutzer procedure at a substantially reduced surgical risk. In cases of persistent left SVC
with or without associated anomalous pulmonary venous
connection, an end-to-side anastomosis between the left
SVC and the left pulmonary artery (modified Glenn)
greatly facilitates the intraatrial rerouting of both systemic and pulmonary venous pathways. Because of the
Fontan-Kreutzer operation
531
Fig. 7. Surgical technique (Case 6). A, Total anomalous
pulmonary venous connection (arrows) to the left superior vena
cava (LSVC). There is no right superior vena cava ({A,L,L}
configuration). B, The LSVC was divided distal to the
entrance of the pulmonary veins, and the proximal end was
closed with a suture (arrow). The anomalous pulmonary
venous return was directed (via the proximal LSVC) to the
common atrial chamber in this way. An intraatrial septal
patch directs the pulmonary venous flow through a common
AV valve into the single ventricle (SV). The inferior vena cava
was excluded into a systemic venous chamber. C, The distal
end of the LSVC was end-to-side anastomosed (white arrow)
to the left pulmonary artery (bidirectional cavopulmonary
shunt). Repair was completed by interposing a conduit (black
arrow) between the systemic venous chamber (containing the
inferior vena caval drainage) and the left pulmonary artery
(modified Fontan).
complexity of the anatomic variables in anomalous
systemic and pulmonary venous connections, each
patient must be carefully studied and the operative
technique planned according to each anatomic variant.
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