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\begin{document}

\title{Double drainage of total anomalous pulmonary venous connection revealed
after surgical repair of a supracardiac total anomalous pulmonary venous
connection}



\author[1]{Hiroyuki Nagao}%
\author[2]{Kenta Tominaga}%
\author[2]{Naoya Kamei}%
\author[2]{Toshikatsu  Tanaka}%
\affil[1]{National Cerebral and Cardiovascular Center}%
\affil[2]{Hyogo Prefectural Kobe Children's Hospital}%


\vspace{-1em}



  
  \date{May 18, 2024}


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\selectlanguage{english}
\begin{abstract}
Total anomalous pulmonary venous connection is an anomaly where the
pulmonary veins are directly connected to one of the systemic veins or
to the right atrium. The most common classification of this condition is
by Craig, Darling and Rothney, which is based on the anatomic site of
abnormal connection%
\end{abstract}%



\sloppy


Abstract

Total anomalous pulmonary venous connection (TAPVC) is an anomaly where
the pulmonary veins are directly connected to one of the systemic veins
or to the right atrium. The most common classification of this condition
is by Craig, Darling and Rothney, which is based on the anatomic site of
the abnormal connection. A few variants are reported which do not fit
into these classic forms.

We report 2 cases that previously underwent surgical repair of
supracardiac TAPVC, where the pulmonary veins drained into the
innominate vein through an ascending vertical vein. However these cases
did not fit into the classic forms, because in both cases, a second
vertical vein was detected incidentally during a follow-up diagnostic
catheterization 1 year after the initial surgery. The second veins
originated proximal to the ligated vertical vein and drained into the
superior vena cava through the accessory hemiazygos vein and azygos
vein.

In Case 1, which was did not provoke right heart volume overload and
general condition is good. the patient is followed up carefully, even in
the absence of any intervention or surgical intervention.

In Case 2, we confirmed right heart volume overload, and then performed
coil embolization of the veno-venous shunt (VV shunt) of the second
vertical vein.

This report describes a successful intervention by catheterization in
the treatment of a VV shunt of TAPVC.

\textbf{Introduction}

Total anomalous pulmonary venous connection (TAPVC) constitutes
1\%--1.5\% of congenital heart diseases in children. Four types have
been identified based on where the pulmonary vein connects abnormally:
supracardiac, cardiac, infracardiac, and mixed, where the anomalous
connection occurs at 2 or more of the above levels. {[}1{]} {[}2{]}

In the 2 cases discussed in this report, a second vertical vein was
noticed incidentally during routine follow-up catheterization 1 year
after the initial surgery.

It originated proximally from the ligated vertical vein and drained into
the superior vena cava through the accessory hemiazygos vein.

It seemed that blood perfusion to a second vertical vein had increased
due to disappear of dominant vertical vein blood perfusion after the
initial surgery.

In this report we discuss the management of this rare case. Furthermore,
this is the first reported case of a transcatheter closure of a second
vertical vein in a mixed TAPVC.

\textbf{Case Report}

\textbf{Case 1}

A 9 year-old boy underwent surgical repair of supracardiac TAPVC when he
was 2 months old and weighed 2.2 Kg. {[}Figure1{]}

During the operation, the vertical vein was ligated. One year after the
surgical repair, we performed follow up diagnostic catheterization that
showed obvious azygos vein enlargement. Contrast enhanced CT was
performed, and it showed a veno-venous shunt (VV shunt) that originated
proximal to the ligated vertical vein and drained into the superior vena
cava through the accessary hemiazygos vein-azygos vein. {[}Figure2{]}

These findings showed there was a second vertical vein causing TAPVC.
Therefore, we corrected the diagnosis from a supracardiac TAPVC to a
double drainage of TAPVC, a rare variant of the mixed type. The
left-right shunting, which occurred as a result of this development, did
not provoke right heart volume overload, and the general condition of
the patient was good. His right heart function is under close monitoring
in outpatient.

\textbf{Case 2}

A 2 year-old infant, weighing 9 kg, with a diagnosis of supracardiac
TAPVC underwent surgical correction at 1 month. Preoperative
echocardiography and contrast CT showed that the pulmonary veins formed
a confluence and drained into the innominate vein through the vertical
vein. We diagnosed supracardiac TAPVC without another cardiac anomaly.

During surgery, the vertical vein was ligated on the innominate vein
side. Postoperatively, recovery was good and pulmonary venous stenosis
(PVS) did not arise. Furthermore, using contrast CT, we confirmed that
the connection of the common chamber and left atrium was not restrictive
before the patient was discharged. A year after the surgical repair, we
performed a follow up catheter examination to check the patients'
cardiac condition. It showed that central venous pressure was 5 mmHg,
right ventricular pressure was 23 mmHg, mean pulmonary capillary wedge
pressure 9 mmHg, left ventricular pressure was 63 mmHg, left ventricular
end-diastolic volume was 104\% of normal, and right ventricular
end-diastolic volume 103 \% of normal.

Although the patient was hemodynamically stable with no pulmonary vein
stenosis (PVS) , a second vertical vein was incidentally noticed that
originated from the proximal ligated vertical vein through the accessary
hemiazygos vein, which drained into the superior vena cava (Figure 3).

On initial assessment, it appeared as though the second vertical vein
originated from the same location as the first vein. However, the first
vertical vein was obviously dominant; therefore, the second vessel would
have had much less perfusion and would have been difficult to be
identified before surgery. After surgery, it became visible as it had
not spontaneously atrophied but rather had increased perfusion. At the
time of discovery, it appeared that the RV volume load was tolerable and
would be managed by careful follow-up. However, at the next check-up,
tricuspid regurgitation had gradually increased. The RV volume load
appeared to be worsening, and we decided to perform occlusion of the
second vertical vein.

Next year of first catheter, we performed the treatment. The right
femoral vein was accessed with a 5Fr venous sheath, and the accessary
hemiazygos veins were accessed from the SVC using a 4 Fr/100 cm
GlidecathII catheter with a Berenstein tip (Terumo). A diagnostic
contrast angiography using a 2.2 Fr
Progreat\selectlanguage{greek}β\selectlanguage{english}\textsuperscript{3}microcatheter (Terumo) was also performed in
the SVC and left pulmonary with occlusion 4Fr wedge in the azygos vein.
The left pulmonary artery (LPA) mean pressure measured 22 mmHg, showing
an increase from the previous year. We carefully performed coil
embolization to avoid occlusion of the pulmonary vein. The first coil
(8mm x 250mm MICRUSFRAME\selectlanguage{ngerman}®C coil; Johnson \& Johnson) was introduced in
close proximity to the origin of the VV shunt to avoid occlusion of the
pulmonary vein, and angiography showed it was well positioned.
Additional coils (6mm x 250mm,5mm x 200mm, 4 x 150mm DELTAFILL® ;
Johnson \& Johnson) and a final one (3mm x 100mm AZUR®\selectlanguage{english}[?] ; Terumo) were
placed to ensure complete occlusion (Figure 4). Repeat angiography
showed disappearance of the residual flow from the VV shunt. Mean left
pulmonary artery pressure measurements revealed the same result before
and after the procedure, and the patient was discharged from the
hospital 2 days later.

\textbf{Discussion}

Double drainage of TAPVC is a rare variant of a mixed type TAPVC, which
occurs when all the pulmonary veins form a confluence and then drain to
both the coronary sinus and the left innominate vein. {[}3{]}. {[}4{]}

Recently however, other variants of TAPVCs with double drainage have
been reported. {[}5{]}. {[}6{]}

In our 2 cases, the pulmonary veins formed a confluence and drained into
the systemic venous system via two vertical veins. The first of the
vertical veins drained through the innominate vein, and the second
originated from the proximal side of the ascending vertical vein.

Preoperative identification of TAPVC with double drainage has important
surgical implications. Although in both cases we performed
echocardiography and contrast CT before the initial surgery so as not to
miss the diagnosis, we did not detect this anomaly.

Because the first ascending vertical vein was more prominent and blood
flowed without obstruction, the second vertical vein had less perfusion
and a smaller vessel diameter. However, after the initial surgery,
perfusion to the dominant vertical vein decreased, causing an increase
in blood flow to the second vertical vein. Pulmonary venous obstruction,
with or without confluence stenosis, is a well-known complication
occurring in approximately 8--15\% of patients after surgical correction
of TAPVC. {[}7{]}

It was reported that with smaller left-sided chambers and a noncompliant
left atrium, an un-ligated vertical vein may improve survival by
preventing a pulmonary hypertensive (PH) crisis. {[}8{]}

An un-ligated vertical vein has been reported to atrophy spontaneously.

However, if it remains patent, it may cause right cardiac failure due to
left-to-right shunting. {[}9{]}

PVS did not occur after surgery in either of the cases, and on
follow-up, using chest X-rays and echocardiography for diagnostic
imaging, this complication was not detected. In our institution, a
follow-up diagnostic catheterization for post TAPVC surgery patients is
performed 1 year after the operation routinely. If this catheterization
is not performed, this anomaly can go undetected. While echocardiography
is sufficient for diagnosing most TAPVC cases, cardiac catheterization
is essential in a mixed variety to adequately assess drainage and
possible obstruction of all 4 pulmonary veins. {[}10{]}

We performed angiography and contrast CT again in another patient who
underwent repair of a supracardiac TAPVC 10 years ago in our
institution; this patient did not present with the same anomaly. The
left-to-right shunting persisted, and we did not identify right heart
volume overload early as was done in Case 1. The hemodynamics in this
patient however mimicked an atrial septal defect, and the right heart
volume load gradually increased as he became an adult.

On the other hand, in Case 2, there was significant right heart volume
overload and it caused an exacerbation of the tricuspid regurgitation,
resulting in an increase of the mean pulmonary artery pressure. Coil
embolization of the second vertical vein was therefore appropriate in
this case.

Previously, it was recommended that the vertical vein was deliberately
not operated on in order to prevent a PH crisis, with embolization with
a coil or plug to be performed if the right heart volume load increased.
{[}11{]}, {[}12{]}

If we could ligate vertical vein near the pulmonary vein, these results
might not be occur.

To our knowledge, this is the first case of a coil embolization for a
vessel that originated from the proximal ligated vertical vein and
drained into the superior vena cava through an accessory hemiazygos
vein-azygos vein after a TAPVC repair.

TAPVC of mixed type is often misdiagnosed if left-to-right shunting is
present like in our case. This procedure proved to be safe and provided
an alternative noninvasive treatment that did not involve surgery.

\textbf{Conclusion}

We encountered two rare cases of mixed type TAPVC with double drainage,
where the second vertical vein enlarged rather than atrophied after
surgery. Echocardiography is a useful diagnostic tool in the follow-up
of PVS, however, contrast enhanced CT or diagnostic catheterization
should be performed to exclude a second veno-venous shunt post-surgery.
Cases of left-to-right shunting that is not recognized before surgery,
it can be treated with catheterization as demonstrated by our case.

\textbf{Author contribution}

Hiroyuki Nagao: Data curation; Formal analysis; Investigation; Software;
Visualization; Writing---original draft. Kenta Tominaga: Investigation;
Methodology; Resources. Naoya Kamei: Investigation; Resources;
Validation. Toshikatsu Tanaka: Conceptualization; Project
administration; Supervision; Validation.

\textbf{Reference}

1) Kanter KR. Surgical repair of total anomalous pulmonary venous
connection. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu.
2006;9:40--44.

2) Darling RC, Rothney WB, Craig JM. Total pulmonary venous drainage
into the right side of the heart; report of 17 autopsied cases not
associated with other major cardiovascular anomalies. Lab Invest
1957;6:44-64.

3) Arciprete P, Mckay R, Watson GH, Hamilton DI, Wikinson JL, Arnold RM.
Double connection in total anomalous pulmonary venous connection. J.
Thorac. Cardiovasc. Surg. 1986; 92: 146--152.

4) Lee ML, Wang JK, Wu MH, Chu SH, Lue HC. Unusual form of total
anomalous pulmonary venous connection with double drainage. Pediatr.
Cardiol. 1995; 16: 301--303.

5)Tanabe S, Nakasato M , Suzuki H, Ishikawa A, Fukasawa M, Hayasaka K. A
new form of total anomalous pulmonary venous connection with double
drainage. Pediatr Int. 2000 ;42:369-371.

6) Krishna MR, Gnanappa GK, Mervis J, Forsey J, Ayer JG, Orr Y. Double
drainage of total anomalous pulmonary venous connection: A rare variant
of mixed total anomalous pulmonary venous connection. Ann Pediatr
Cardiol. 2020 ;13:100-101

7) van de Wal HJ, Hamilton DI, Godman MJ, Harinck E, Lacquet LK, van
Oort A. Pulmonary venous obstruction following correction for total
anomalous pulmonary venous drainage: a challenge. Eur J Cardiothorac
Surg. 1992;6:545--549.

8)Chowdhury UK, Subramaniam KG, Joshi K, Varshney S, Kumar G, Singh R,
Venugopal P. Rechanneling of total anomalous pulmonary venous connection
with or without vertical vein ligation: results and guidelines for
candidate selection. J Thorac Cardiovasc Surg 2007;133:1286-1294.

9) Amoozgar H, Ahmadipoor M, Amirghofran AA

Transcatheter Closure of Partially Ligated Vertical Vein after Surgical
Correction of Supracardiac Total Anomalous Pulmonary Venous Connection.
J Tehran Heart Cent. 2015;10:152-155

10) Chowdhury UK, Airan B, Malhotra A, Bisoi AK, Saxena A, Kothari SS,
Kalaivani M, Venugopal P. Mixed total anomalous pulmonary venous
connection: anatomic variations, surgical approach, techniques, and
results. J Thorac Cardiovasc Surg. 2008;135:106-116, 116.e1-5.

11) Narula N, Wilson N, Kumar RS. Transcatheter closure of persistent
unligated vertical vein after TAPVC surgery using the Amplatzer PDA
device. Catheter Cardiovasc Interv. 2007;70:117--119.

12) Kobayashi D, Forbes TJ, Delius RE, Aggarwal S. Amplatzer vascular
plug for transcatheter closure of persistent unligated vertical vein
after repair of infracardiac total anomalous pulmonary venous
connection. Catheter Cardiovasc Interv. 2012;80:192--198.

Figure Legends

Figure 1 : Contrast CT before surgery repair (behind view). White arrow
indicate vertical vein(common PV to innominate vein)

Figure 2 : Contrast CT after surgery repair (behind view). White arrow
indicate second vertical vein(originated from proximal ligated vertical
vein and drain into superior vena cava )

Figure 3a : Contrast CT after surgery repair (behaind view). White arrow
indicate second vertical vein(originated from proximal ligated vertical
vein and drain into superior vena cava)

Figure 3b : White arrow indicate accessory hemiazygos vein

Figure 3c : left pulmonary angiogram. White arrow indicate second
vertical vein into superior vena cava

Figure 4: left pulmonary angiogram after coil embolization\selectlanguage{english}
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