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\begin{document}

\title{Surgical repair of an obstructed mixed-type Total Anomalous Pulmonary
Venous Connection}



\author[1]{Reem   Shammout}%
\author[1]{Doaa Alkhadraa}%
\author[1]{Sidra  Alkadi}%
\author[1]{Alwaleed Al-Dairy}%
\affil[1]{Damascus University Faculty of Medicine}%


\vspace{-1em}



  
  \date{June 01, 2024}


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\selectlanguage{english}
\begin{abstract}
Total anomalous pulmonary venous connection is a rare congenital
anomaly, and has four anatomical subtypes of which the mixed type
represents diagnostic and therapeutic challenge. Herein, we present a
rare case of obstructed mixed type total anomalous pulmonary venous
connection with successful surgical repair%
\end{abstract}%



\sloppy


\textbf{Surgical repair of an obstructed mixed-type Total Anomalous
Pulmonary Venous Connection}

Reem Shammout \textsuperscript{1}, Doaa Alkhadraa \textsuperscript{1},
Sidra Alkadi \textsuperscript{1}, Alwaleed Al-Dairy \textsuperscript{2*}

\textsuperscript{1} Medical student at Faculty of Medicine, Damascus
University, \textsuperscript{2} Assistant Professor in Cardiac Surgery
at Faculty of Medicine, Damascus University.

\textsuperscript{\textbf{*}}\textbf{Correspondence to} : Alwaleed
Al-Dairy: Assistant Professor in Cardiac Surgery at Faculty of Medicine,
Damascus University, and pediatric cardiac surgeon at Children
University Hospital, Damascus, Syria.

Email: alwaleedaldairy.aa@gmail.com; Tel+963112133545, Fax +9632129437.

ORCID: Alwaleed Al-Dairy (0000-0002-2239-646X).

\textbf{Abstract:}

Total anomalous pulmonary venous connection is a rare congenital
anomaly, and has four anatomical subtypes of which the mixed type
represents diagnostic and therapeutic challenge. When associated with
obstruction however; urgent surgical repair is needed. Herein, we
present a rare case of obstructed mixed type total anomalous pulmonary
venous connection with successful surgical repair.

\textbf{Key words:}

Patent foramen ovale, Pulmonary venous obstruction, Total anomalous
pulmonary venous connection, Ventricular septal defect.

\textbf{Key Clinical Message:}

Total anomalous pulmonary venous connection has four main anatomical
types. When associated with obstruction however; it needs urgent
surgical repair. Accurate preoperative evaluation is critical for
achieving favorable outcomes.

\textbf{Introduction:}

Total anomalous pulmonary venous connection (TAPVC) is one of the rarest
congenital cardiac diseases with 7 per 100000 neonates' approximate
incidence (1,2). It accounts for about 1\% of all congenital heart
diseases (3). In this anomaly, all the pulmonary veins drain into right
atrium (or one of its tributaries) instead of the normal drainage into
left atrium (4). Anatomically, TAPVC can be divided into 4 major types
depending on the level of pulmonary veins' drainage: supracardiac (into
the innominate vein or right superior vena cava), cardiac (into the
coronary sinus or directly to the right atrium), infracardiac (most
commonly into the portal vein or into the ductus venosus, hepatic vein,
or directly into the infradiaphragmatic inferior vena cava), and mixed
(in which there is a combination between types above) (1-4). The
separation of both pulmonary and systemic circulations in TAPVC makes
the presence of right-to-left shunt obligatory for survival, and any
restriction in this communication represents an ''obstruction''. The
obstruction may be at any level of the drainage, and is encountered in
about 25\% to 50\% of TAPVC patients. The degree and level of
obstruction are remarkable factors affecting the pathophysiology and
presentation (5, 6). In the presence of obstruction, the patient is
usually symptomatic in the neonatal period, symptoms progressively
worsen, and the neonate may present with profound acidosis and hypoxemia
despite the aggressive management and oxygen supplement (4). Herein, we
report a rare case of an obstructed mixed-type TAPVC in a neonate in
association with large VSD in whom successful surgical repair was
performed.

\textbf{Case presentation:}

A 7-day male neonate presented to our hospital with cyanosis, tachypnea
since birth.

Transthoracic Echocardiography (TTE) revealed mixed type TAPVC, small
patent foramen ovale (PFO), a large VSD (10 mm), a large patent ductus
arteriosus (PDA) with a right to left shunt, and severe pulmonary
hypertension (PH). For more anatomical details , computed tomographic
angiography (CTA) was performed, and confirmed the diagnosis of a mixed
type TAPVC (the common pulmonary confluent was draining both into the
coronary sinus and the left innominate vein through a vertical vein)
with an obstruction. The obstruction level was at the connection of the
vertical vein with the left innominate vein. On the 10th day of life,
the neonate developed a sudden cardiac arrest and underwent
cardiopulmonary resuscitation with rapid response without the need for
mechanical ventilation. The patient was scheduled for urgent surgical
repair. The operation was performed through median sternotomy. A large
vertical vein was seen on the left side outside the pericardium and
connecting with a very small vein to the left innominate vein
(representing the site of obstruction) (Figure 1). The vertical vein was
dissected and controlled. The pericardium was opened, and a large PDA
was controlled and closed by metal clips. Complete cardiopulmonary
bypass (CPB) was prepared with bicaval cannulation, the vertical vein
was closed at its junction with the left innominate vein, and the heart
was arrested by antegrade cold blood cardioplegic solution. The common
pulmonary confluent was seen behind the heart. The right atrium was
opened parallel to the right atrioventricular groove. A small PFO was
seen (representing another site of obstruction). The coronary sinus
orifice was cut through to unroof the coronary sinus and establish a
wide communication with the left atrial cavity after resecting the
atrial septum. A large fresh autologous pericardial patch was used to
baffle the created cavity toward the left atrial cavity. A large VSD was
closed by a bovine pericardium patch with interrupted sutures. The
remainder of the operation was completed uneventfully and the patient
was weaned off the CPB easily. The patient suffered from atrial
arrhythmias in the intensive care unit (ICU) on the second postoperative
day, and was managed appropriately. After one week on mechanical
ventilation, the patient was extubated, and on the
15\textsuperscript{th} postoperative day was discharged from the ICU.
The patient was followed-up for six months and was in very good general
condition with significant improvement and weight gain.

\textbf{Discussion:}

The diagnosis of mixed type TAPVC and its surgical repair are
challenging, however; it is the least common type of TAPVC, accounting
for about 5\% of cases (3). Our patient had mixed type of TAPVC (cardiac
and supracardiac) associated with VSD. There was two cites of
obstruction in our patient, the first was at the connection of the
vertical vein with the left innominate vein which was through a very
small vein branch (Figure 1), and the second was due to the very small
PFO. The majority of TAPVC cases are diagnosed by TTE which is the
preferred diagnostic tool, but sometimes additional images modalities
are required such as CTA, angiography via cardiac catheterization or MRI
(7, 8). The main management of TAPVC patients is primary surgical
repair, however; a transcatheter palliative shunt may be considered in
patients with other important comorbidities such as prematurity, low
birth weight, multisystem organ dysfunction, multiple congenital
anomalies (9, 10). Our patient was managed surgically and did not need
primary transcatheter palliation. It has been reported that the
mortality of obstructed TAPVC had decreased over the eras from 42.1\% in
the seventies to 7.4\% after 2010. The worst prognosis is predicted in
patients with pulmonary obstruction and suprasystemic PH preoperatively
in which the patient might have been intubated. Patients who underwent
emergent surgery with significant pulmonary vein obstruction still have
the worst scenario despite the progress in the field of TAPVC surgery
(11).

\textbf{Conclusion:}

Mixed type TAPVC represents diagnostic and therapeutic challenge in the
field of congenital heart surgery. The best surgical outcomes in
obstructed TAPVC cases need accurate preoperative evaluation and prompt
intervention.

\textbf{Figure legends:}

Figure 1: Intraoperative image showing the vertical vein with its
connection to the left innominate vein. 1: Right atrium, 2: vertical
vein, 3: The small branch that was connecting the vertical vein with the
left innominate vein.

\textbf{Author Contribution}

\textbf{Reem Shammout} : Participated in writing the report and approved
the final version

\textbf{Doaa Alkhadraa} : wrote and reviewed the successive versions and
participated in their revisions

\textbf{Sidra Alkadi} : wrote and reviewed the successive versions and
participated in their revisions

\textbf{Alwaleed Al-Dairy} : Planned and performed the work leading to
the report. Wrote and reviewed successive versions and participated in
their revisions.

\textbf{Author's Statement:}

\textbf{Consent:} Written informed consent was obtained from the
patient's parents to publish this report in accordance with the
journal's patient-consent policy\textbf{.}

\textbf{Author's Declaration:}

None of the authors listed on the manuscript are employed by a
government agency that has a primary function other than research and/or
education. Moreover, none of the authors are submitting this manuscript
as an official representative or on behalf of the government.

\textbf{Conflict of interest:}

The authors have no conflict of interest

\textbf{Acknowledgement:} None

\textbf{Ethics statement:}

The manuscript was approved by ethics committee at Damascus University.

\textbf{Funding statement:}

There is no funding resources for writing this manuscript.

\textbf{Data availability statement:}

The data that support the findings of this study are available from the
corresponding author, {[}A.A{]}, upon reasonable request.

\textbf{References:}

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\tightlist
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\end{enumerate}\selectlanguage{english}
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