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\begin{document}

\title{THORACOSCOPIC ABLATION IN SITUS INVERSUS DEXTROCARDIA WITH INTERRUPTED
INFERIOR VENA CAVA CONTINUATION IN AZYGOS VEIN}



\author[1]{Fabrizio Rosati}%
\author[1]{Rattenni F}%
\author[1]{Di Bacco L}%
\author[1]{Michele D'Alonzo}%
\author[2]{Antonio Curnis}%
\author[1]{Claudio Muneretto}%
\author[1]{Benussi S.}%
\affil[1]{Universita degli Studi di Brescia}%
\affil[2]{Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia}%


\vspace{-1em}



  
  \date{May 26, 2024}


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\selectlanguage{english}
\begin{abstract}
INTRODUCTION Situs inversus totalis, dextrocardia with interrupted
inferior vena cava and azygos vein continuation concomitant with
symptomatic atrial fibrillation requiring ablation. This case was deemed
not suitable for percutaneous ablation due to anatomic variations and
the lack of case reports in literature. METHODS AND RESULTS We performed
bilateral thoracoscopic epicardial ablation and epicardial left atrial
appendage exclusion. The direct vision allowed for a complete box lesion
set with bipolar~radiofrequency device. Patient remained in sinus rhythm
at 12-months follow-up. CONCLUSION Surgical thoracoscopic epicardial
ablation is safe and effective also in congenital defects.
Multidisciplinary expertise can offer minimally invasive ablation
treatments.%
\end{abstract}%



\sloppy


\textbf{THORACOSCOPIC ABLATION IN SITUS INVERSUS DEXTROCARDIA WITH
INTERRUPTED INFERIOR VENA CAVA CONTINUATION IN AZYGOS VEIN}

Rosati F, Rattenni F, Di Bacco L, D'Alonzo M, Curnis A, Muneretto C,
Benussi S.

Division of Cardiac Surgery, Spedali Civili di Brescia, University of
Brescia, Brescia, Italy Division of Electrophysiology, Spedali Civili di
Brescia, University of Brescia, Brescia, Italy

\emph{Corresponding Author:}

\textbf{Benussi Stefano, MD PhD}

Division of Cardiac Surgery - Spedali Civili di Brescia

University of Brescia Medical School

P.le Spedali Civili 1,

25123 Brescia, Italy

Phone: +39 030 399 6401

Fax: +39 030 399 5004

Email Address:\emph{stefano.benussi@unibs.it}

Stefano Benussi is consultant/proctor for Atricure, Allergan, and
Cryolife.

Other authors: No conflict of interest to disclose.

Funding: (None)

\textbf{ABSTRACT} INTRODUCTION

Situs inversus totalis, dextrocardia with interrupted inferior vena cava
and azygos vein continuation concomitant with symptomatic atrial
fibrillation requiring ablation. This case was deemed not suitable for
percutaneous ablation due to anatomic variations and the lack of case
reports in literature.

METHODS AND RESULTS

We performed bilateral thoracoscopic epicardial ablation and epicardial
left atrial appendage exclusion. The direct vision allowed for a
complete box lesion set with bipolar~radiofrequency device. Patient
remained in sinus rhythm at 12-months follow-up.

CONCLUSION

Surgical thoracoscopic epicardial ablation is safe and effective also in
congenital defects. Multidisciplinary expertise can offer minimally
invasive ablation treatments.

KEYWORDS

Atrial fibrillation, radiofrequency ablation, surgical thoracoscopic
ablation, minimally invasive, left atrial appendage, left atrial
appendage exclusion, situs inversus totalis, dextrocardia, interrupted
inferior vena cava

INTRODUCTION Concomitant long-persistent atrial fibrillation in a rare
congenital aberration: situs inversus totalis, dextrocardia with
interrupted inferior vena cava and azygos vein continuation. This case
was deemed not suitable for percutaneous ablation due to anatomic
variations and the lack of case reports in literature. METHODS AND
RESULTS We performed surgical thoracoscopic epicardial ablation and
concomitant left atrial appendage exclusion. Thanks to direct vision,
the complete box lesion was performed with radiofrequency. At twelve
months patient remained in sinus rhythm. CONCLUSION Surgical
thoracoscopic epicardial ablation is safe and effective also in
congenital defects. Multidisciplinary expertise can offer minimally
invasive ablation treatments.

KEYWORDS: Atrial fibrillation, radiofrequency ablation, surgical
thoracoscopic ablation, left atrial appendage, left atrial appendage
exclusion, congenital defects, situs inversus totalis, dextrocardia,
interrupted inferior vena cava

\textbf{INTRODUCTION}

Intrahepatic interruption of the inferior vena cava (IVC) with azygos
vein continuation is a rare congenital aberration, occurring in 0.6\% of
patients with other cardiac defects as situs abnormalities and
dextrocardia.\textsuperscript{1} In most instances, patients are
asymptomatic. They might be noticed as incidental findings following
imaging investigations. We present a case of situs inversus totalis and
dextrocardia associated with interrupted inferior vena cava (IVC) and
azygos vein continuation in a patient with concomitant long-persistent
atrial fibrillation. The treatment of atrial fibrillation (AF) has been
considered at high risk for percutaneous interventions and thus referred
to our institute for surgical bilateral thoracoscopic epicardial
ablation and left atrial appendage (LAA) exclusion.

The patient is a 45-years old male with symptomatic long-standing
persistent atrial fibrillation (LsP-AF) who received multiple electrical
cardioversions for AF recurrences since 2003. Class I antiarrhythmic
drugs failed to restore sinus rhythm thus, according to the current ESC
2020 Guidelines for AF treatment \textsuperscript{2}, the patient was
scheduled for transcatheter pulmonary veins (PV)
isolation.\textsuperscript{3}Pre-operative chest X-RAY showed the
presence of a complete situs inversus dextrocardia (SID). (Figure 1A)

Then, a thoraco-abdominal CT scan was performed to rule out other
anatomical abnormalities. A concomitant intrahepatic interruption of the
IVC was described with the renal veins draining into the azygos vein
which was directly collecting blood from the lower body and draining
posteriorly into the superior vena cava (SVC). In adjunct, the hepatic
veins were draining directly into the right atrium (RA). Nonetheless,
regular anatomy of the left atrium (LA) with two right and two left PVs
was depicted (Figure 1B, 1C).

The patient was deemed not suitable for percutaneous ablation (PA)
because of the complex anatomy and was then referred to our Institution
for surgical thoracoscopic ablation. Transesophageal echocardiogram was
performed to rule out thrombus in the LAA prior to surgery.

\textbf{METHODS}

Thoracoscopic procedure was performed starting from the chest side in
which LAA was promptly approachable: the right side. Two 10-mm ports and
one 5-mm port were placed along the right anterior axillary (3th and 5th
intercostal space) and mid-axillary line (4th intercostal space) as
previously described for classic anatomy
procedures.\textsuperscript{3,4} Briefly, once the pericardium was
opened, the LAA was visualised as reference. A blunt dissection at the
level of the roof of the LA was performed to allow the right curved
bipolar ablation device (Isolator Synergy Clamp, EMR2; AtriCure Inc,
Mason, OH USA) to encircle the PV on the right side (Figure 2A). The
device followed the route previously prepared by means of a track-light
dissector (Lumitip, AtriCure Inc, Mason, OH USA). Connecting lesions
were then performed by using a linear bipolar radiofrequency device
(Coolrail linear pen, AtriCure Inc, Mason, OH USA) at the level of the
inferior side of the Box Lesion (Figure 2B). LAA occlusion was performed
by means of AtriClip Pro 2 device 40 mm (AtriCure Inc, Mason, OH USA).
We then moved ports to the contralateral side in order to complete the
thoracoscopic surgical ablation (Figure 2C).

A blunt dissection of the pericardial reflection below the confluence of
the two sovra-hepatic veins (draining into the RA) was performed in
order to gain access to the oblique sinus (Figure 3A). The access to the
transverse sinus was obtained by gently dissecting the pericardial
reflection below the SVC. PVs at the left side were encircled and RF
energy was delivered by means of left curved bipolar ablation RF device
(Isolator Synergy Clamp, EML2 AtriCure Inc, Mason, OH USA) (Figure 3B).
Once completed, connecting lesions between right and left PVs were
performed at the level of the roof of the LA (Coolrail, AtriCure Inc,
Mason, OH USA). Exit block was confirmed at the end of the procedure
with a total surgical time of 200 minutes.

\textbf{RESULTS} Intra-operatively and during hospitalization no
complications occurred. The patient was discharged in sinus rhythm after
four days. At three and twelve months follow-up, the patient was in
stable sinus rhythm without experiencing recurrent symptomatic episodes
of AF, as confirmed by means of holter-ECG evaluation.

\textbf{DISCUSSION}

The incidence of dextrocardia with situs inversus totalis is
1/10,0000--50,000 births.(6) In such patients, the IVC might be
interrupted or stenotic in 8-18\% of patients, with azygos continuation
present in only 0,6\% of cases.\textsuperscript{7} Unless severe
concomitant congenital defects occur, patients with SID showed life
expectancy similar to that of the general population.\textsuperscript{8}
In literature only few case reports described ablation strategies in
this specific subset of patients, in which percutaneous PVs isolation
can be extremely challenging. The major limitations are considered:
small calibre of the entrance vessel, often complicated by tortuosity
especially at the level of the azygos vein;\textsuperscript{9}
difficulty in obtaining trans-septal puncture; limited manoeuvrability
of the ablation/mapping system.\textsuperscript{7} For these reasons,
``unconventional methods'' were explored and described in
literature:\textsuperscript{10,11} so far, three different PA approaches
have been reported: trans-septal puncture via trans-jugular approach
through the SVC\textsuperscript{9}; transaortic retrograde
approach\textsuperscript{10} and percutaneous transhepatic vein
approach.\textsuperscript{11}

Trans-septal puncture via internal jugular/subclavian vein and SVC was
described by Masumoto et al.\textsuperscript{9} PVs isolation was
effectively performed under 3D navigation system. However, the procedure
was reported as extremely long despite authors simplified the procedure
by omitting electrophysiological mapping of the LA (over 360 minutes of
procedural time with more than 60 minutes of PVI time). Major concerns
raised mainly while performing the trans-septal puncture with a
procedure generally defined as ``not smooth''. Of note, authors avoided
to approaching the IVC because of the tortuosity.\textsuperscript{9}

An intriguing trans-aortic solution was reported by Okajima and
colleagues \textsuperscript{10} in this specific subset of patients. Via
the left femoral artery authors gained access to the LA in a retrograde
fashion and under magnetic navigation system. However, authors were
unable to perform a complete isolation of the four PVs: the right
inferior PV isolation was aborted since the ablation catheter repeatedly
dropped into the left ventricle because of an unfavourable angle and
short distance between mitral annulus and PV orifice. Moreover, in order
to simplify a such complex procedure, authors avoided to evaluate
entrance/exit block as well as the electro-anatomical mapping.
\textsuperscript{9,10}

A percutaneous trans-hepatic vein approach was described by Tandon et
al.\textsuperscript{11} in a patient with dextrocardia and IVC
continuation in the azygos vein. LA access was gained via hepatic vein
puncture under ultrasound guidance and fluoroscopy. The procedure was
reported as safe, however, only addressed the LAA. \textsuperscript{11}

Non-invasive percutaneous solutions provided a stable restoration of the
sinus rhythm, however they were reported as particularly challenging. In
most instances, the major drawbacks were the necessity to use different
types of catheters, the complex catheter guidance and manoeuvrability,
thus leading to exceedingly long and often incomplete procedures.

Surgical thoracoscopic AF ablation had the advantage of a direct vision
of the complex anatomy of the patient, allowing the surgeons to promptly
recognize cardiac structures despite the complete SID. Effective lesions
were safely performed and confirmed by testing the presence of the exit
block, thus without affecting the completeness of the ablation lesion
set and the procedural time. Moreover, the LAA was successfully excluded
by epicardial access with no additional risk.

\textbf{CONCLUSION} In conclusion, patients with complex anatomy should
be carefully evaluated with a multidisciplinary approach in specialistic
facilities with a wide expertise in minimally invasive arrhythmia
surgery. A patient-tailored approach was guaranteed once risks and
benefits of the surgical procedure over percutaneous strategy were
wisely balanced, thus providing the best option in terms of safety,
efficacy and patient satisfaction.

\textbf{REFERENCES}

1. Lev M, Liberthson RR, Eckner FA, Arcilla RA. Pathologic anatomy of
dextrocardia and its clinical implications. Circulation.
1968;37(6):979--99.

2. Hindricks G, Potpara T, Dagres N, et al. 2020 ESC Guidelines for the
diagnosis and management of atrial fibrillation developed in
collaboration with the European Association for Cardio-Thoracic Surgery
(EACTS). Eur Heart J. 2021;42(5):373--498.

3. Muneretto C, Bisleri G, et al. European prospective multicentre study
of hybrid thoracoscopic and transcatheter ablation of persistent atrial
fibrillation: the HISTORIC-AF trial. Eur J Cardiothorac Surg.
2017;52(4):740--5.

4. Meelad IH, Theo J, Putte V, Bart P, Massimo A. Totally thoracoscopic
pulmonary vein isolation a simplified technique. Innovations (Phila)
2017;12:493--5.

5. Vos LM, Kotecha D, Geuzebroek GSC, Hofman FN, Boven WJP Van, Kelder
J, et al. Totally thoracoscopic ablation for atrial fibrillation: a
systematic safety analysis. EP Europace.2018;20(11);1790--7.

6. Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia:
An analysis of cardiac structures in 125 patients. Int J Cardiol.
2003;88(2--3):143--55.

7. Anderson RC, Adams P BB. Anomalous inferior vena cava with azygos
continuation (infrahepatic interruption of the inferior vena cava):
Report of 15 new cases. J Pediatr. 1961;59:370--83.

8. Zhao X, Su X, Long DY, Sang CH, Bai R, Tang RB, et al. Catheter
ablation of atrial fibrillation in situs inversus dextrocardia:
Challenge, improved procedure, outcomes, and literature review. PACE -
Pacing Clin Electrophysiol. 2021;44(2):293--305.

9. Masumoto A, Kobori A, Sasaki Y, Pak M, Furukawa Y. Successful
catheter ablation of persistent atrial fibrillation and common atrial
flutter in a patient with dextrocardia, situs inversus, and interrupted
inferior vena cava with azygos continuation. Hear Case Reports.
2021;7(6):403--7.

10. Okajima K, Nakanishi T, Ichibori H, Shirai T, Kadotani M, Shimizu H,
et al. Trans-aortic pulmonary vein isolation using magnetic navigation
system for paroxysmal atrial fibrillation in a patient with
dextrocardia, situs inversus, and inferior vena cava continuity with
azygos vein. J Arrhythmia. 2018;34(5):583--5.

11. Tandon TS, Agarwal HM, Alla VM, Abuissa HS. Left atrial appendage
closure in a patient with situs inversus totalis and interrupted
inferior vena cava -- A transhepatic approach. Hear Case Reports.
2020;6(11):871--4.

\textbf{FIGURE LEGEND}

Figure 1 - A: chest X-Ray showing complete situs inversus dextrocardia.
B: Left-sided view howing azygos vein (white asterisk) continuation
draining into the posterior aspect of the superior vena cava (white
dot). C: Posterior view of the azygos vein (white asterisk) crossing the
midline.

Figure 2 -- A: Right curved bipolar ablation device encircling the
right-sided pulmonary veins (white arrow: right superior pulmonary vein;
white asterisk: left atrial appendage). B: Linear radiofrequency device
performing connecting lesion at the level of the floor of the box lesion
(white arrow: left inferior pulmonary vein; white asterisk: confluence
of the hepatic veins). C: Left atrial appendage exclusion device
positioning.

Figure 3 -- A: Left anatomy after pericardial opening. B: Left curved
bipolar ablation device encircling the left sided pulmonary vein. (white
arrrow: left pulmonary vein; white asterisk: superior vena cava).\selectlanguage{english}
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