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\begin{document}

\title{Misdiagnosis of persistent left superior vena cava with unroofed
coronary sinus as a coronary sinus-type atrial septal defect}



\author[1]{Alwaleed Al-Dairy}%
\author[1]{Reem Ahmad}%
\author[1]{Rawan Hasan}%
\affil[1]{Damascus University Faculty of Medicine}%


\vspace{-1em}



  
  \date{May 17, 2024}


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\selectlanguage{english}
\begin{abstract}
Unroofed coronary sinus syndrome may be associated with persistent left
superior vena cava (PLSVC). Herein, we present a case of a 2-year-old
patient who underwent an operation for repair of a coronary sinus-type
ASD; however, PLSVC was detected intraoperatively. total repair was
performed by creating an intra-atrial tunnel.%
\end{abstract}%



\sloppy


\textbf{Misdiagnosis of persistent left superior vena cava with unroofed
coronary sinus as a coronary sinus-type atrial septal defect}

Alwaleed Al-Dairy\textsuperscript{1,[?]}, MD, Reem
Ahmad\textsuperscript{2}, Rawan Hasan \textsuperscript{2}.

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

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

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

\textbf{Abstract}

Unroofed coronary sinus syndrome is a rare congenital heart defect
representing less than 1\% of all atrial septal defect types, and may be
associated with persistent left superior vena cava which may be missed
during preoperative diagnosis. Herein, we present a case of a 2-year-old
patient who underwent an operation for repair of a coronary sinus-type
ASD; however, PLSVC was detected intraoperatively. An antra-atrial
tunnel was created to divert the flow of PLSVC into the right atrium
along with the repair of the atrial septal defect.

\textbf{Keywords} : persistent left superior vena cava, unroofed
coronary sinus, intra-atrial tunnel.

\textbf{Key Clinical Message:}

The association of UCSS with PLSVC must be kept in mind during
operation. Creating an intra-atrial tunnel to divert the flow of PLSVC
into RA, without obstructing the mitral valve or the pulmonary veins, is
a safe surgical approach.

\textbf{Introduction} :

Persistent left superior vena cava (PLSVC) is the most common thoracic
venous anomaly, with an incidence of 0.2\% in normal neonates (1).
However; it is more common among patients with congenital heart diseases
(CHD) (1.4\%), and recognition of its presence by preoperative diagnosis
is of a great importance during congenital heart surgery (1-4).
Commonly, PLSVC drains into the right atrium (RA) via the coronary sinus
(CS), and when isolated, it is usually asymptomatic, and may be detected
incidentally (1). Unroofed coronary sinus syndrome (UCSS) is an uncommon
CHD which leads to a left to right shunt at the atrial level, and
comprises \textless{}1\% of all atrial septal defect (ASD) types (2, 5).
When UCSS is associated with PLSVC, it is difficult to detect the
diagnosis by transthoracic echocardiography (TTE) (1). Herein, we
present a case of a 2-year-old patient with preoperative diagnosis of
coronary sinus-type ASD; however, PLSVC draining into the left atrium
(LA) was detected during surgery.

\textbf{Case presentation:}

A 2-year-old girl was presented to our hospital when her parents noticed
that she had suffered from moderate tachypnea recently. Physical
examination revealed a 3/6 systolic murmur on the left sternal border
and there was not any cyanosis. TTE showed an ASD of coronary sinus type
of about 1.5 cm with dilated right heart cavities. The patient was
scheduled for surgical closure of the ASD. Upon anaesthesia, oxygen
saturation (SaO2) was 100\%, and the operation was performed through
median sternotomy. The pericardium was opened, and PLSVC was noticed.
Complete cardiopulmonary bypass (CPB) was prepared without cannulating
the PLSVC. The heart was arrested by antegrade cold blood cardioplegic
solution. The right atrium (RA) was opened with an incision parallel to
the right atrioventricular groove as usual. On inspection, we found that
the PLSVC was draining into the roof of the LA, and a venous cannula was
directed through the ASD towards its orifice to drain it (Figure 1). The
pulmonary veins drainage and the mitral valve were inspected. A fresh
autologous pericardial patch was used to construct a tunnel that drains
the PLSVC into RA (Figure 2). The ASD was closed by another fresh
autologous pericardial patch and thus the PLSVC will drain into RA
(Figure 3). The remainder of the operation was completed uneventfully.

\textbf{Discussion:}

UCSS is a rare CHD representing less than 1\% of all ASD types (1). Its
clinical manifestation is atypical, and may be associated with varied
CHDs (6). Preoperative diagnosis by TTE is very difficult when
associated with PLSVC (2, 6). In our patient, the diagnosis of
associated PLSVC was missed preoperatively, and was detected
intraoperatively, and this had led to change in the surgical plan. Our
surgical approach was to create an intra-atrial tunnel to divert the
flow of PLSVC into RA with great attention to the pulmonary veins and
the mitral valve, and repair the ASD with fresh autologous pericardial
patch . Other operative options include: ligating PLSVC and repairing
the ASD; constructing a baffle to guide PLSVC towards RA (6). We confirm
the importance of precise preoperative diagnosis to avoid any
complications from such an incidental intraoperative finding.

\textbf{Conclusion:}

UCSS is a rare CHD; however, its association with PLSVC must be kept in
mind. Creating an intra-atrial tunnel to divert the flow of PLSVC into
RA, without obstructing the mitral valve or the pulmonary veins, is a
safe surgical approach.

\textbf{Figure legends:}

Figure-1: Intraoperative image showing the venous cannula placed through
the ASD towards PLSVC orifice. 1: The opened RA, 2: LA, 3: the venous
cannula placed into PLSVC orifice.

Figure-2: Intraoperative image showing the intra-atrial tunnel. 1: the
pericardial patch used to construct the tunnel.

Figure-3: Intraoperative image showing the final anatomy after closing
the ASD. 1: the pericardial patch used for ASD closure, 2: the venous
cannula placed in the intra-atrial tunnel to divert PLSVC flow into RA.

\textbf{Author Contribution}

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

\textbf{Reem Ahmad} : wrote and reviewed the successive versions and
participated in their revisions

\textbf{Rawan Hasan} : Participated in writing the report and approved
the final version

\textbf{Author's Statement:}

\textbf{Consent:} Written informed consent was obtained from the
patient's parents for publication of this report and the images.

\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}

1- Azizova A, Onder O, Hazirolan T, Persistent left superior vena
cava:clinical importance and differential diagnoses . Insight Imaging
.2021 Dec;12:49. 2- Chen J\_l, YU C\_G, Chen H\_B. Misdiagnosis of
unroofed coronary sinus syndrome as ostium primum artrial septal defect
by echocardiograghy. World J clin Cases. 2022 Feb 16; 10(5):1592\_1597

3- Perles Z, Nir A, Gavri S et al (2013) Prevalence of persistent
superior vena cava and association with congenital heart anomalies. Am J
Cardiol 112(8):1214--1218.

4- Nagasawa H, Kuwabara N, Goto H, et al. Incidence of Persistent Left
Superior Vena Cava in the Normal Population and in Patients with
Congenital Heart Diseases Detected Using Echocardiography.~Pediatr
Cardiol.~2017;39(3):484--490.

5- Velamakanni SM, Khadkikar G, Panchal SS, Patel A, Shah BR. Unroofed
Coronary Sinus in a Patient With Prior Surgical Closure of Secundum
Atrial Septal Defect. Cureus. 2021 Nov 8;13(11):e19350.

6- Liang J, Liu W, Liu J. {[}Unroofed coronary sinus syndrome{]}.
Zhonghua Wai Ke Za Zhi. 1996 Sep;34(9):546-8. Chinese. PMID: 9594159.

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