Surgical Science, 2013, 4, 509-515
Published Online November 2013 (http://www.scirp.org/journal/ss)
Open Access SS
Complex TAPVC-Experience with Six Patients*
Baskar Ranjith Karthekeyan#, Periyasamy Thangavelu, Jebaraj Rethinasamy, Mahesh Vakamudi,
Siva Muthukumar, Rajeshkumar Kodali, Kamalakannan Sambandham, Sushma Nandipati
Sri Ramachandra Medical College and Research Institute, Chennai, India
Received October 30, 2013; revised November 18, 2013; accepted November 24, 2013
Copyright © 2013 Baskar Ranjith Karthekeyan et al. This is an open access article distributed under the Creative Commons Attribu-
tion License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
Total anomalous pulmonary venous connection becomes a totally different subset when associated with complex con-
genital anomalies. The combination of two separate life-threatening congenital heart defects complicates the manage-
ment of these patients. Six patients with total anomalous pulmonary venous connection associated with complex con-
genital heart disease were studied. There were 2 girls and 4 boys. Three of them were less than 5 kg in weight, and the
other 3 were more than 5 kg in weight. Four patients had severe pulmonary arterial hypertension and 2 patients had
pulmonary stenosis. Three patients had supracardiac type with a right vertical vein, one had drainage to the right atrium
superior vena cava junction, one patient had supra cardiac type but split flow to both the superior vena cava and one
patient had cardiac type. Three patients had double outlet right ventricle. Three patients had atrioventricular canal defect
and 2 patients had preoperative pulmonary vein obstruction. All patients underwent rerouting of pulmonary veins.
Concomitant procedures included intraventricular tunnel repair of ventricular septal defect and infundibular resection in
double outlet right ventricle. Atrioventricular canal rep air was done for Rastelli type A atrioventricular canal. Superior
vena caval plasty, atrioventricular canal repair and pulmonary artery banding were done in unbalanced atrioventricular
septal defect and large double outlet right ventricle. Intracardiac repair through transatrial approach was done for
tetralogy of Fallot. Right ventricle-pulmonary artery conduit was done for truncus arteriosus. Single ventricle repair was
done for corrected transpos ition of great arteries. There were 2 hospital deaths.
Keywords: Total Anomalous; AV Canal; Complex; Congenital; Pulmonary Vein
Total anomalous pulmonary venous connection is char-
acterized by the failed union of the pulmonary veins and
incorporation of it by the developing left atrium in com-
bination with a persistent embryologic connection be-
tween the pulmonary and systemic venous systems. The
impact of the pathology depends on the degree to which
the pulmonary venous drainage is obstructed and the
magnitude of the left-to-right shunt. Accurate assessment
of the anatomy and detailed surgical planning become
essential when total anomalous pulmonary venous con-
nection is associated with other complex congenital ano-
malies. This result in a combination of two separate life-
threatening heart defects needs appropriate management.
Most often the timing of surgery is dictated by the pres-
ence or development of pulmonary venous obstruction
rather than the standardized protocols that apply to the
majority of patients. It is our goal that this analysis will
identify some important principles in the management of
these complex patients.
2. Patients and Methods
Six patients with total anomalous pulmonary venous
connection associated with complex congenital heart
disease were diagnosed in our hospital between 2011 and
2013. Symptoms included delayed milestones, breath-
lessness, cyanosis, excessive sweating, poor weight gain
and recurrent lower respiratory infections. There were 2
girls and 4 boys. Three of them were less than 5 kg in
weight, and the other 3 were more than 5 kg in weight.
Four patients had severe pulmonary arterial hypertension
and 2 patients had pulmonary stenosis. Three patients
had supracardiac total anomalous pulmonary venous
connection with a right vert i cal vein, one had drai nage to
*Conflict of interest: none.
B. R. KARTHEKEYAN ET AL.
the right atrium superior vena cava junction, one patient
had supra cardiac total anomalous pulmonary venous
connection but split flow to both the superior vena cava
and one patient had cardiac total anomalous pulmonary
venous connection. Three patients had double outlet right
ventricle. Three patients had atrioventricular canal defect
and 2 patients had preoperative pulmonary vein obstruc-
tion. Patients’ summary is given in Table 1.
All patients underwent rerouting of total anomalous
pulmonary venous connection. All operations were per-
formed under general anesthesia through a median ster-
notomy. Cardiopulmonary bypass was established with
aortic and bicaval cannulation. Myocardial protection
included cold blood cardioplegia. Deep hypothermic cir-
culatory arrest was required in one patient. All patients
were cooled to 280 celsius. For all types of total anoma-
lous pulmonary venous connection, the anastomosis be-
tween the common pulmonary venous chamber and the
atrium was performed by continuous suture with 7-0
polypropylene. In addition, the area of the anastomotic
orifice was made as large as possible. Vertical vein was
ligated and patent foramen ovale was left open in all
Concomitant procedures included intraventricular
tunnel repair of ventricular sep tal defect and infund ibular
resection in double outlet right ventricle with total
anomalous pulmonary venous connection. Atrioven-
tricular canal repair was done for Rastelli type A atrio-
ventricular canal defect with total anomalous pulmonary
venous connection. Superior vena caval plasty, atrioven-
tricular canal repair and pulmonary artery banding was
done in unbalanced atrioventricular septal defect and
large double outlet right ventricle in which pulmonary
vein was obstructed at the superior vena caval junction.
Intracardiac repair through transatrial approach was done
for tetralogy of Fallot with total anomalous pulmonary
venous connection. Right ventricle-pulmonary artery
conduit was constructed for truncus arteriosus with total
anomalous pulmonary venous connection. Single ventri-
cle repair was done for corrected transposition of great
Table 1. Patient summary.
Patient Age Sex Weight in KgsEchocardiogram Operation
1 2 Years M 8
Situs solitus, double outlet right ventricle with large subaortic
perimembranous ve ntricular septal defect, moderate pulmonary
valve stenosis, unobstructed supracardiac TAPVC to right atrium
superior vena cava junc tion, bilateral superior vena cava with left
superior vena cava draining to unroo fed coronary sinus.
(Figures 1 & 2)
Intraventricular tunnel repair of the
ventricular septal defect,
Infundibular resection, Rerouting
of TAPVC, Rerouting of left superi or
vena cava to right atrium,
Pericardial patch closure of
atrial septal defect.
2 9 Months F 6
Situs ambiguous, ri g ht at rial isomerism, dextrocardia, comm on
atrium, complete atrioventricular septal defect Rastelli type A,
bilateral superior vena cava, supra cardiac TAPVC, 2 ve i ns
entering each of the superior vena cava, small patent ductus
arteriosus, severe pulmonary hypertension. (Figure 3)
Rerouting of TAPVC, Atrioventricular
canal repair. (Patient died on fourth
3 9 Months M 4.5
Situs ambiguous righ t i somerism, p art ia ll y obstructed right
sided supra cardiac TAPVC to rig ht superior vena cava,
unbalanced atrioventricular septal defect with regur g i t at i o n
with small left ventricle, double outlet right ventricle with
severe pulmonary artery hypertension. (Figures 4 & 5)
Rerouting of TAPVC, Right superi o r
vena caval plasty, Atrioventr icular valve
repair, Pulmonar y artery banding.
4 6 Months F 4.5 Situs solitus, tetralogy of Fallot with TAPVC draining to the
coronary sinus. left super ior vena cava draining to coronary
sinus. (Figure 6)
Rerouting of pulmonary veins into left
atrium, Intracardiac repair through
transatrial approach, Rerouting of left
superior vena cava to right atrium.
5 14 Years M 30
Situs ambiguous, levo c ardia, right atrial isomerism, left superior
vena cava to the roof of the left sided atrium, Unbalanced
atrioventricular septal defect with rudim entary left ventricle,
double outlet right ventricle with malposed g reat arteries, severe
pulmonary valvu lar stenosis, unobstructed righ t si ded sup ra
cardiac TAPVC to right brachiocephalic vein. (Figures 7 & 8)
Rerouting of TAPVC with sin g le
stage Fontan surgery.
6 Newborn M 3.2
Situs solitus, Type III-truncusarteriosus, right and left pulmonary
artery arising separately f rom the common trunk, subtruncal
ventricular septal defect, ostiumsecundum atrial septaldefect,
partially obstructed right sided supracardiac TAPVC draining to
right brachio cephalic ve i n , severe pulmonary arteri al
hypertension. (Figure 9)
Rerouting of TAPVC, Tr uncu s repair
with right ventricle-p ulmonary artery
conduit, Ventricular septal defect rerouted
to aorta, Atrial septal defect closure.
(Patient died on third post o pe rative day)
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B. R. KARTHEKEYAN ET AL. 511
Figure 1. Patient no one showing double outlet right ventri-
cle with large subaortic perimembranous ventricular septal
defect, moderate pulmonary valve stenosis with TAPVC.
Procedure done is also shown.
Figure 2. MR Angiogram of patient no one showing
Figure 3. Patient no 2 showing dextrocardia, atrioventricu-
lar septal defect, patent ductus arteriosus, common atrium
Figure 4. Patient no three showing unbalanced atrioven-
tricular septal defect, double outlet right ventricle and ob-
structed TAPVC. Procedure done is also shown.
Figure 5. CT angiogram of patient no 3 showing all four
pulmonary veins and vertical ve in.
Figure 6. Patient no four showing tetralogy of Fallot with
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B. R. KARTHEKEYAN ET AL.
Figure 7. Patient no five showing double outlet right ventri-
cle, atrioventricular septal defect, malposed great arteries
Figure 8. Echocardiogram of patient no five showing un-
balanced atrioventricular septal defect with rudimentary
Figure 9. Patient no six showing truncus arteriosus with
arteries with total anomalous pulmonary venous connec-
tion. There were 2 hospital deaths. Cause of death in both
the patients was pulmonary hypertensive crises.
Complex heart defects involving venous, intracardiac and
arterial pathways can be successfully repaired in a single
stage. Complete repair of the very rare association of
complex congenital anomalies like truncus arteriosus,
tetralogy of Fallot, complete atrioventricular canal and
corrected transposition of great arteries with total ano-
malous pulmonary venous connection is possible and
customized technical solutions are often necessary to
achieve good results . Untreated, the prognosis for this
patients is rather bleak, with half of the patients suc-
cumbing within 1st year of life [2,3]. The relative efficacy
of surgical treatment was a point of debate for several
decades. The subsequent standardization of protocols at
many centers throughout the world has improved the
outlook for these patients with multiple congenital heart
defects substantially .
When total anomalous pulmonary venous connection
is associated with other complex heart defects the risk for
early postoperative death phenomenally increases. Ag-
gressive postoperative management after a very meticu-
lously planned corrective/palliative surgery is needed to
improve the outcome. The 10-year survival rate in het-
erotaxy syndrome has been reported as 39% with total
anomalous pulmonary venous connection and 64%
without total anomalous pulmonary venous connection
. A report of 377 patients undergoing total anomalous
pulmonary venous connection repair found that surgical
outcomes have improved over time due to improvements
in surgical techniques and perioperative management .
The 5-year survival rate after total anomalous pulmonary
venous connection repair in the biventricular heart has
improved to 97% since 2000. However, surgical results
for complex congenital anomalies associated with total
anomalous pulmonary venous connection have not yet
been satisfactory, with a reported 3-year survival rate of
only 47% .
Total anomalous pulmonary venous connection is usu-
ally associated with right isomerism in 30%, other heart
anomalies in 20% and is isolated in more than 50%.
Congenital anomalies associated with total anomalous
pulmonary venous connection include viscerocardiac
situs abnormality, aortocaval juxtapo sition, atrioventricu-
lar septal defect, common atrium, double-outlet right
ventricle, dextro-malposed great arteries, pulmonary
stenosis or atresia, coarctation of aorta, hypoplastic left
heart, parachute mitral valve and straddling mitral valve.
The diagnosis of anomalous pulmonary venous connec-
tions in the setting of heterotaxy should be actively con-
sidered, especially in neonates with right isomerism .
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B. R. KARTHEKEYAN ET AL. 513
In this series of six patients total anomalous pulmonary
venous connection was associated with tetralogy of Fal-
lot, truncus arteriosus, unbalanced AV canal, corrected
transposition of great arteries, double outlet right ventri-
cle and complete AV canal with dextocardia.
The two outcome factors to be considered are mortal-
ity and postoperative recurrent pulmonary vein obstruc-
tion. Recent reports about complex congenital heart dis-
ease and total anomalous pulmonary venous connection
have indicated an improvement in the outcomes over
time. They showed that survival at 5 years after total
anomalous pulmonary venous connection repair was
79% and the risk factors for mortality were yo unger age,
pulmonary atresia, preoperative obstructed total anoma-
lous pulmonary venous connection, body weight less
than 3.5 kg at operation, concomitant systemic pulmo-
nary shunt, postoperative pulmonary vein obstruction and
infracardiac or mixed total anomalous pulmonary venous
Body weight and age at operation was identified as a
risk factor for death. Of 226 patients with repaired total
anomalous pulmonary venous connection in the Society
of Thoracic Surgeons database, the surgical outcome in
body weight ranging from 1 to 2.5 kg at operation was
worse than that in body weight ranging from 2.5 to 4 kg
. Increased bod y weight at operation may also reduce
perioperative complications such as intracranial hemor-
rhage, renal dysfunction, and coagulopathy . In our
series three patients weighed above 5 kgs and three pa-
tients weighed less than 5 kgs. Both the child whom died
was less than 5 kgs. However, waiting for an increase in
body weight before operation may not be advantageous
due to an increase in preoperative morbidities . Age
at operation tends to affect early surgical outcomes .
However in our series all the children were sligh tly older
except the truncus arteriosus patient who was referred to
us in the newborn period.
Nonconfluent pulmonary arteries and pulmonary atre sia
were identified as a risk factor for death in patients oper-
ated for total anomalous pulmonary venous connection
. Because of the varied anatomic variations in the
total anomalous pulmonary venous connection anatomy,
especially when associated with complex congenital
heart malformations, accurate preoperative diagnosis and
determination of the configuration of the pulmonary vein
morphology are essential for planning on surgical treat-
ment of these challenging patients; else the postoperative
course could be complicated . Jenkins et al. reported
that small pulmonary vein confluence was a strong nega-
tive predictor of surv ival in patients with total anomalous
pulmonary venous connection including those with two-
ventricle anatomy. Generally, mixed total anomalous
pulmonary venous connection has smaller pulmonary
vein confluence(s) and this may contribute to its worse
prognosis . Most of the patients in our series had
good pulmonary vein confluence.
It is important to have a regulated pulmonary blood
flow in total anomalous pulmonary venous connection.
However patients requiring manipulation of pulmonary
blood flow are difficult to manage. Concomitant sys-
temic pulmonary shunt or pulmonary artery banding was
identified as an added risk factor for hospital death. In
the management of complex cardiac abnormalities, it is
essential to have an optimal pressure-vo lume relationship
in the heart (prelo ad and after load) to gain better quality
of life . It may be difficult to sustain an adequate
circulation in patients who have had total anomalous
pulmonary venous connection repair with concomitant
systemic pulmonary shunt, especially in patients diag-
nosed with preoperative pulmonary vein obstruction and
functional univentricle physiology with right ventricular
morphology. We consider that intensive management is
important to adjust the pulmonary blood flow in these
complex patients. Among our patients infundibular re-
section was done in two patients in two patients and PA
banding was done in one patient. Vertical vein if present
was ligated and patent foramen ovale was left open in all
Atrioventricular valve regurgitation, which required
surgical intervention, was a significant risk factor for
postoperative recurrent pulmonary vein obstruction.
Volume unloading after the atrioventricular valve repair
reduces the size of the atrium and may stretch or distort
the pulmonary vein(s) . Atrioventricular regurgitation
is associated with systemic ventricular dysfunction and
subsequent pulmonary hypertension. Surgical outcomes
are therefore influenced by the ability to repair atrioven-
tricular valve regurgitation at total anomalous pulmonary
venous connection repair . We had 2 patients with
atrioventricular valve regurgitation. In both the patients
repair was done successfully, however one died. This
patient continued to have persistent pulmonary hyperten-
sion despite a satisfactory anatomic repair. This patient
eventually died from a combination of hypoxemia and
With newer surgical techniques and better manage-
ment strategies, the outcomes of patients with total
anomalous pulmonary venous connection and associated
congenital anomalies have improved dramatically. How-
ever, when complex congenital cardiac malformations
exist in association with total anomalous pulmonary ve-
nous connection, it still remains a clinical challenge es-
pecially if it was obstructed . There is a paucity of
knowledge about this subset of patients. We had 2 cases
of preoperative obstructed total anomalous pulmonary
venous connection of which one child died. Both the
cases were obstructed at the junction with the superior
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B. R. KARTHEKEYAN ET AL.
The repair of total anomalous pulmonary venous con-
nection was performed by a single surgeon, and con-
formed to the technical principles of appropriate (not
excessive) dissection of the pulmonary vein and minimal
manipulation, taking small bites with nonabsorbable su-
tures and cutting off the pectin ate muscle which is proper
to the single right atrium of RAI, if necessary, to mini-
mize the thickness of the anastomosis and precise anas-
tomosis under short and intermittent circulatory arrest, if
necessary. Surgical approaches such as primary suture-
less repair reported by Yun and colleagues may be indi-
cated for patients with mixed total anomalous pulmonary
venous connection, especially when obstructed. However,
it is not clear in the literature if primary sutureless repair
will have any significant impact on the prognosis of
mixed total anomalous pulmonary venous connection.
The practice of cutting off the pectinate muscle is based
on the theory that the thickness of the anastomosis con-
tributes to the occurrence of anastomotic stenosis and
recurrent pulmonary vei n o bstruction [1 6] .
Univentricle or heterotaxy syndrome has been identi-
fied as a risk factor for reoperation in repaired pulmonary
vein stenosis. The study found that 79% of reoperations
for pulmonary vein stenosis occurred within 6 months of
the initial repair . Another report of 20 patients iden-
tified that postoperative pulmonary vein obstruction oc-
curring within 6 months of the operation was a risk factor
for death . Residual pulmonary vein obstruction
might cause subsequent pulmonary hypertension, with
even worse outcomes . Therefore, postoperative pul-
monary vein obstruction should be repaired as soon as
possible, before the pulmonary hypertension becomes
persistent. Another report found that high resistance of
the pulmonary vasculature, caused by postoperative
pulmonary vein obstruction, was the main factor influ-
encing death in 14 autopsies of patients diagnosed with
univentricle and total anomalous pulmonary venous
We think that pulmonary vein wall cannot be normal-
ized immediately and that the various complications
might occur due to the poor general condition of the pa-
tient, even if the pulmonary vein obstruction is relieved.
Recurrent pulmonary vein obstruction occurs due to sev-
eral intrinsic factors and compression from the adjacent
structures like the aorta, atrium, bronchus, or vertebrae.
Conventional total anomalous pulmonary venous con-
nection repair may be associated with later intimal hy-
perplasia localized to the anastomotic site. Improvement
or modification of surgical repair technique may improve
the results. For example, a sutureless technique that
avoids trauma to the pulmonary vein wall and minimizes
the risk of distortion at the anastomosis has been reported
. This technique may be able to prevent postoperative
pulmonary vein obstruction, although long-term surgical
outcomes are unclear. More than the type of total
anomalous pulmonary venous connection it is the post-
operative pulmonary vein obstruction that worsens the
prognosis. With our technical principles, none of the pa-
tients developed anastomotic stenosis.
Improvement or modification of the primary repair tech-
nique is required to avoid postoperative pulmonary vein
obstruction, such as using sutureless technique, espe-
cially in patients of whom total anomalous pulmonary
venous connection is associated with complex congenital
cardiac anomalies. Prevention of postoperative pulmo-
nary vein obstruction is important in this complex group
of patients. Postoperative pulmonary vein obstruction
should be repaired as early as possible, before the pul-
monary hypertension becomes persistent. Careful post-
operative management is required to control the pulmo-
nary blood flow, especially with a concomitant systemic
pulmonary shunt or pulmonary artery banding. Signifi-
cant atrioventricular regurgitatio n should be addressed to
improve postoperative morbidity and mortality. Total
anomalous pulmonary venous conn ection asso ciated with
complex heart disease can be successfully repaired in a
single stage. We therefore consider that complete evalua-
tion of the pulmonary venous anatomy, corrective sur-
gery at the appropriate time, aggressive perioperative
management and close follow-up after hospital discharge
are essential to improve the surgical outcome when total
anomalous pulmonary venous connection is associated
with complex congenital cardiac anomaly.
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