Complex TAPVC-Experience with Six Patients

doi:10.4236/ss.2013.411099

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Surgical Science, 2013, 4, 509-515

Published Online November 2013 (http://www.scirp.org/journal/ss)

http://dx.doi.org/10.4236/ss.2013.411099

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

Email: #[email protected]

Received October 30, 2013; revised November 18, 2013; accepted November 24, 2013

tion License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly

cited.

ABSTRACT

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

1. Introduction

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.

#Corresponding author.

B. R. KARTHEKEYAN ET AL.

510

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

cases.

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

postoperative day)

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

TAPVC.

Figure 3. Patient no 2 showing dextrocardia, atrioventricu-

lar septal defect, patent ductus arteriosus, common atrium

and TAPVC.

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

TAPVC.

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B. R. KARTHEKEYAN ET AL.

512

Figure 7. Patient no five showing double outlet right ventri-

cle, atrioventricular septal defect, malposed great arteries

and TAPVC.

Figure 8. Echocardiogram of patient no five showing un-

balanced atrioventricular septal defect with rudimentary

left ventricle.

Figure 9. Patient no six showing truncus arteriosus with

TAPVC.

arteries with total anomalous pulmonary venous connec-

tion. There were 2 hospital deaths. Cause of death in both

the patients was pulmonary hypertensive crises.

3. Discussion

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 [1]. 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 [4].

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

[5]. 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 [6].

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% [7].

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 [8].

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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

connection [9].

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

[10]. Increased bod y weight at operation may also reduce

perioperative complications such as intracranial hemor-

rhage, renal dysfunction, and coagulopathy [10]. 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 [11]. Age

at operation tends to affect early surgical outcomes [11].

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

[12]. 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 [13]. 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 [14]. 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 [15]. 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

patients.

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) [9]. 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 [15]. 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

respiratory failure.

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 [9]. 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

vena cava.

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B. R. KARTHEKEYAN ET AL.

514

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 [17]. 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 [18]. Residual pulmonary vein obstruction

might cause subsequent pulmonary hypertension, with

even worse outcomes [19]. 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

connection [20].

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

[20]. 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.

4. Conclusion

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.

5. Acknowledgements

The authors like to acknowledge the help of Dr. Ramya,

Dr. Ramkumar and Dr. Rammohan.

REFERENCES

[1] S. Conte, T. Jensen, J. R. Jacobsen, F. S. Joyce, P. Laur id-

sen and G. Pettersson, “One-Stage Repair of Truncus Ar-

teriosus, CAVC and Total Anomalous Pulmonary Venous

Connection,” Annals of Thoracic Surgery, Vol. 63, No. 6,

1997, pp. 1781-1783.

http://dx.doi.org/10.1016/S0003-4975(97)83863-5

[2] R. D. Mainwaring, V. M. Reddy, O. Reinhartz, R. Punn,

T. Tacy and F. L. Hanley, “Surgical Results in Patients

With Pulmonary Atresia-Major Aortopulmonary Collat-

erals in Association with Total Anomalous Pulmonary

Venous Connection,” Annals of Thoracic Surgery, Vol.

92, No. 5, 2011, pp. 1756-1760.

http://dx.doi.org/10.1016/j.athoracsur.2011.06.020

[3] H. Leonard, G. Derrick, J. O’Sullivan and C. Wren, “Natural

and Unnatural History of Pulmonary Atresia,” Heart, Vol.

84, No. 5, 2000, pp. 499-503.

http://dx.doi.org/10.1136/heart.84.5.499

Open Access SS

B. R. KARTHEKEYAN ET AL.

Open Access SS

515

[4] A. Carotti, S. B. Albanese, S. Filleppelli, et al., “Deter-

minants of Outcome after Surgical Treatment of Pulmo-

nary Atresia with Ventricular Septal Defect and Major

Aortopulmonary Collateral Arteries,” Journal of Thoracic

and Cardiovascular Surgery, Vol. 140, No. 5, 2010, pp.

1092-1103. http://dx.doi.org/10.1016/j.jtcvs.2010.07.087

[5] S. R. Foerster, K. Gauvreau, D. B. McElhinney and T.

Geva, “Importance of Totally Anomalous Pulmonary Ve-

nous Connection and Postoperative Pulmonary Vein

Stenosis in Outcomes of Heterotaxy Syndrome,” Pediat-

ric Cardiology, Vol. 29, No. 3, 2008, pp. 536-544.

http://dx.doi.org/10.1007/s00246-007-9128-5

[6] T. Karamlou, R. Gurofsky, E. Al Sukhni, et al., “Factors

Associated with Mortality and Reoperation in 377 Chil-

dren with Total Anomalous Pulmonary Venous Connec-

tion,” Circulation, Vol. 115, 2007, pp. 1591-1598.

http://dx.doi.org/10.1161/CIRCULATIONAHA.106.6354

[7] C. L. Hanco ck Friese n, D. Zurakowski, R. R. Thiagarajan,

et al., “Total Anomalous Pulmonary Venous Connection:

An Analysis of Current Management Strategies in a Sin-

gle Institution,” Annals of Thoracic Surgery, Vol. 79, No.

2, 2005, pp. 596-606.

http://dx.doi.org/10.1016/j.athoracsur.2004.07.005

[8] C. R. Patel, J. R. Lane , M. L. Spector, P. C. Smith and S.

S. Crane, “Totally Anomalous Pulmonary Venous Con-

nection and Complex Congenital Heart Disease Prenatal

Echocardiographic Diagnosis and Prognosis,” Journal of

Ultrasound in Medicine, Vol. 24, No. 9, 2005, pp. 1191-

1198.

[9] T. Nakata, Y. Fujimoto, K. Hirose and M. Osaki, “Func-

tional Single Ventricle with Extracardiac Total Anoma-

lous Pulmonary Venous Connection,” European Journal

of Cardio-Thoracic Surgery, Vol. 36, No. 1, 2009, pp. 49-

56. http://dx.doi.org/10.1016/j.ejcts.2009.02.060

[10] C. L. Curzon, S. Milford-Beland, J. S. Li, et al., “Cardiac

Surgery in Infants with Low Birth Weight Is Associated

with Increased Mortality: Analysis of the Society of Tho-

racic Surgeons Congenital Heart Database,” Journal of

Thoracic and Cardiovascular Surgery, Vol. 135, No. 3,

2008, pp. 546-551.

http://dx.doi.org/10.1016/j.jtcvs.2007.09.068

[11] V. M. Reddy, D. B. McElhinney, T. Sagrado, A. J. Parry,

D. F. Teitel and F. L. Hanley, “Results of 102 Cases of

Complete Repair of Congenital Heart Defects in Patients

Weighting 700 to 2500 Grams,” Journal of Thoracic and

Cardiovascular Surgery, Vol. 117, No. 2, 1999, pp. 324-

331. http://dx.doi.org/10.1016/S0022-5223(99)70430-7

[12] K. Takeu chi, A. Murakami, Y. Hirata, K. Kitahori, Y. Doi

and S. Takamoto, “Surgical Outcome of Heterotaxy Syn-

drome in a Single Institution,” Asian Cardiovascular and

Thoracic Annals, Vol. 14, No. 6, 2006, pp. 489-494.

http://dx.doi.org/10.1177/021849230601400610

[13] U. K. Chowdhury, B. Airan, A. Malhotra, A. K. Bisoi, A.

Saxena, S. S. Kothari, M. Kalaivani and P. Venugopal,

“Mixed Total Anomalous Pulmonary Venous Connection:

Anatomic Variations, Surgical Approach, Techniques, and

Results,” Journal of Thoracic and Cardiovascular Sur-

gery, Vol. 135, No. 1, 2008, pp. 106-116.

http://dx.doi.org/10.1016/j.jtcvs.2007.08.028

[14] K. J. Jenkins, S. P. Sanders, E. J. Orav, E. A. Coleman, J.

E. Mayer Jr and S. D. Colan, “Individual Pulmonary Vein

Size and Survival in Infants with Totally Anomalous

Pulmonary Venous Connection,” Journal of the American

College of Cardiology, Vol. 22, No. 1, 1993, pp. 201-206.

http://dx.doi.org/10.1016/0735-1097(93)90835-O

[15] Y. Nakayama, T. Hiramatsu, Y. Iwata, T. Okamura and T.

Konuma, “Surgical Results for Functional Univentricular

Heart with Total Anomalous Pulmonary Venous Connec-

tion over a 25-Year Experience,” Annals of Thoracic

Surgery, Vol. 93, No. 2, 2012, pp. 606-613.

http://dx.doi.org/10.1016/j.athoracsur.2011.09.038

[16] T. J. Yun, O. O. Al-Radi, I. Adatia, C. A. Caldarone, J. G.

Coles, W. G. Williams, J. Smallhorn and G. S. Van Ars-

dell, “Contemporary Management of Right Atrial Isom-

erism: Effect of Evolving Therapeutic Strategies,” Jour-

nal of Thoracic and Cardiovascular Surgery, Vol. 131,

No. 5, 2006, pp. 1108-1113.

http://dx.doi.org/10.1016/j.jtcvs.2005.11.036

[17] A. M. Kelle, C. L. Backer, J. G. Gossett, S. Kaushal and

C. Mavroudis, “Total Anomalous Pulmonary Venous Con-

nection: Results of Surgical Repair of 100 Patients at a

Single Institution,” Journal of Thoracic and Cardiovas-

cular Surgery, Vol. 139, No. 6, 2010, pp. 1387-1394.

http://dx.doi.org/10.1016/j.jtcvs.2010.02.024

[18] M. Ricci, M. Elliott, G. A. Cohen, et al., “Management of

Pulmonary Venous Obstruction after Correction of Total

Anomalous Pulmonary Venous Connection: Risk Factors

for Adverse Outcome,” European Journal of Cardio-

Thoracic Surgery, Vol. 24, No. 1, 2003, pp. 28-36.

http://dx.doi.org/10.1016/S1010-7940(03)00180-5

[19] F. Lacour-Gayet, J. Zoghbi, A. E. Serraf, et al., “Surgical

Management of Progressive Pulmonary Venous Obstruc-

tion after Repair of Total Anomalous Pulmonary Venous

Connection,” Journal of Thoracic and Cardiovascular

Surgery, Vol. 117, No. 4, 1999, pp. 679-687.

http://dx.doi.org/10.1016/S0022-5223(99)70287-4

[20] J. W. Gaynor, M. H. Collins, R. Rychik, J. P. Gaughan

and T. L. Spray, “Long-Term Outcome of Infants with

Single Ventricle and Total Anomalous Pulmonary Ve-

nous Connection,” Journal of Thoracic and Cardiovas-

cular Surgery, Vol. 117, No. 3, 1999, pp. 506-514.

http://dx.doi.org/10.1016/S0022-5223(99)70330-2