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\begin{document}

\title{Intracardiac defects and vascular ring from aberrant subclavian artery:
single-incision approach.}



\author[1]{Jineel Raythatha}%
\author[2]{Himanshu Choudhury}%
\author[2]{Bharat Dalvi}%
\author[2]{krishnanaik shivaprakasha}%
\affil[1]{The University of Sydney Sydney Medical School}%
\affil[2]{Sir HN Reliance Foundation Hospital and Research Centre}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
We present a rare case of atrial septal defect and ventricular septal
defect with a vascular ring. The ring was formed by a right-sided aortic
arch with an aberrant left subclavian artery that gave rise to a patent
ductus arteriosus connecting to the main pulmonary artery. We performed
a single-stage repair of the intra-cardiac defects and division of
vascular ring with a novel sternotomy approach instead of the
traditionally practised dual approach. Our novel approach included
implantation of the aberrant left subclavian artery to the left carotid
artery after transection. We could perform single-stage division of
vascular ring along with the closure of both septal defects.%
\end{abstract}%



\sloppy


\emph{Case Report:}

A 2-year-old girl was referred for management of restrictive
perimembranous ventricular septal defect (VSD), moderate sized secundum
atrial septal defect (ASD) and right aortic arch with a left aberrant
subclavian artery (ASA). The left ASA ran a retrooesophageal and
retrotracheal course with indentation of the oesophagus and trachea.
Additionally, a large patent ductus arteriosus (PDA) connecting the main
pulmonary artery (MPA) to left ASA completed a rare case of vascular
ring (Figure 1).

\emph{Steps of surgery:}

The child underwent surgery through mid-sternotomy employing
cardio-pulmonary bypass (CPB) under general anaesthesia. After
sternotomy, the thymus was excised completely to expose and loop the
innominate vein. That exposed the right carotid artery and left carotid
artery on either side of the trachea. This was followed by further
diligent dissection into the uppermost aspect of the posterior
mediastinum between the trachea, left carotid artery and ascending aorta
to trace entire length of the ASA and loop it so as to complete the
mobilisation (Figure 2A).

After establishing the cardio-pulmonary bypass (CPB), the ductus was
divided and sutured. Under mild hypothermia, the ASA was mobilised
proximally as well as distally and was transected near its aortic origin
while the subclavian end was brought anteriorly and was anastomosed with
the left carotid artery in an end to side fashion using 7/0 prolene
continuous sutures (Figure 2C). The removal of clamps revealed good
pulsatile flow with satisfactory distention of the ASA.

Thereafter, under cardioplegic arrest, the VSD (autologus pretreated
pericardial patch) and ASD (direct closure) were closed from right
atrium. Patient was rewarmed and cross clamp was released. All the steps
of open-heart surgery were along the standard lines. Child was
discharged on the seventh day with good pulsations of the left radial
artery. The child remains symptom-free and has well felt pulsations in
left radial artery 3 months after surgery.

\emph{\textbf{Discussion}:}

A right aortic arch with a left aberrant subclavian artery, as seen in
this patient, is the second most common form of vascular ring anomaly
after the double aortic arch\textsuperscript{1}. In our case, the ring
was completed by a PDA running between MPA and ASA which is unusual
(Figure 2B).

The conventional approach is division of the ligamentum arteriosum or
PDA to relieve compression caused by the vascular ring allowing the
structures to retract away from the tracheo-oesophageal
complex\textsuperscript{2}. This can be also achieved with minimally
invasive methods but with a higher chance of recurrence of symptoms
requiring re-intervention\textsuperscript{3}. Translocation of the ASA
to the carotid artery via lateral thoracotomy has better outcomes than
the above approaches\textsuperscript{2,3} but this technique applies to
isolated vascular rings without coexisting intracardiac lesions and our
child had large septal defects that also required closure in the same
sitting. A single approach to treat vascular rings with intra-cardiac
defects that has a lower risk of re-intervention remains undescribed.

In our patient, a lateral thoracotomy approach was not considered in
view of co-existing intra-cardiac shunts. A dual incision approach
comprising of supraclavicular access and a sternotomy had obvious
disadvantages of increase in operative time with attendant morbidities
and 2 visible scars. Moreover, complete translocation of the ASA without
a CPB from sternotomy was risky as the ASA was posterior to the trachea
and oesophagus with chances of injuring the oesophagus during dissection
and mobilisation. In view of these, it was thought that a midline
approach using CPB was ideal because it gave access to the vascular
ring, increased safety and also provided the option of closing the
intracardiac defects. Besides, it also provided better access to
mobilise the ASA after exit from its tracheo-oesophageal relation.

\emph{Conclusion:} To conclude, our single-stage and single-incision
approach has several additional advantages and early result have been
encouraging.

\begin{enumerate}
\tightlist
\item
  It enabled total correction of vascular ring and also closure of
  septal defects in a single stage and single incision.
\item
  Complete removal of pulsatile blood flow posterior to the trachea and
  oesophagus without injuring them.
\item
  Minimal damage to the second and third part of the subclavian artery
  which is associated with increased risk of vascular
  compromises\textsuperscript{4}.
\item
  It was quicker, cosmetically superior and less morbid as compared to
  other approaches\textsuperscript{5}.
\item
  It eliminated chances of the recurrence of symptoms due to transection
  and reimplantation of ASA.
\end{enumerate}

\emph{Conflicts of interest:}

Conflict of interest: None declared

\emph{Author contribution statement:}

Jineel Raythatha -- Conceptualization; Formal analysis; Investigation;
Methodology; Project administration; Visualization; Writing -- original
draft; Writing -- review \& editing

Himanshu Choudhury -- Conceptualization; Formal analysis; Investigation;
Visualization

Bharat Vinayak Dalvi -- Methodology; Supervision; Validation;
Visualization; Writing -- review \& editing

Krishnanaik Shivaprakasha -- Conceptualization; Formal analysis;
Methodology; Supervision; Validation; Visualization; Writing -- review
\& editing

\emph{\textbf{References}:}

\begin{enumerate}
\tightlist
\item
  Hanneman K, Newman B, Chan F. Congenital Variants and Anomalies of the
  Aortic Arch. Radiographics: a review publication of the Radiological
  Society of North America, Inc. 2017;37(1):32-51.
  doi:10.1148/rg.2017160033
\item
  Bidar E, Arrigoni SC, Accord RE. Resection of Kommerell Diverticulum
  and Reimplantation of Aberrant Left Subclavian Artery in Right Aortic
  Arch Vascular Ring. Seminars in Thoracic and Cardiovascular Surgery.
  2019;31(3):561-563. doi:10.1053/j.semtcvs.2018.11.015
\item
  Shinkawa T, Greenberg SB, Jaquiss RDB, Imamura M. Primary
  Translocation of Aberrant Left Subclavian Artery for Children With
  Symptomatic Vascular Ring. The Annals of Thoracic Surgery.
  2012;93(4):1262-1265. doi:10.1016/j.athoracsur.2011.12.030
\item
  Hodge JC. Iatrogenic Vertebrobasilar Insufficiency after Surgery of
  the Subclavian or Brachial Artery: Review of Three Cases.
  Neurosurgery. 1998;43(6):1458-1458.
  doi:10.1097/00006123-199812000-00113
\item
  Doenst T, Diab M, Sponholz C, Bauer M, F\selectlanguage{ngerman}ärber G. The Opportunities and
  Limitations of Minimally Invasive Cardiac Surgery. Deutsches
  Arzteblatt international. 2017;114(46):777-784.
  doi:10.3238/arztebl.2017.0777
\end{enumerate}

\emph{Figure legends}

\emph{\textbf{Figure 1:} \textbf{Computer Tomography}}

A: Sagittal section showing TBC

B: Axial view of TBC by the vascular ring

\emph{\textbf{Figure 2:} \textbf{Pre and post operation photographs and
illustrations}}

\textbf{A:} Pre-operative photograph. Aorta and right ventricle (RV) are
visualised on right. LCC is retracted to reveal posteriorly located ASA
L represents lateral.

\textbf{B:} Illustration of TBC by VR.

\textbf{C} : Post-operative photograph. End-side anastomosis bulb of ASA
to LCC seen.

\textbf{D} : Illustration of Interruption of ASA at origin from aorta,
distal transection and anastomosis to LCC, and PDA ligation.

\textbf{Keys for figures:} AO = Aorta; ASA = Left aberrant subclavian
artery; CCA = Common Carotid Artery; LCC = Left Common Carotid artery;
MPA = Main Pulmonary Artery; O = Oesophagus; PDA = Patent Ductus
Arteriosus; SCA = Subclavian Artery; TBC= tracheo-bronchial compression;
T = Trachea; VR = Vascular Ring.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-1-V2---Vascular-Ring-500ppi/Figure-1-V2---Vascular-Ring-500ppi}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Figure-2-V3---Vascular-Ring-400ppi-compressed/Figure-2-V3---Vascular-Ring-400ppi-compressed}

\end{center}
\end{figure}

\selectlanguage{english}
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