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CARDIOVASCULAR SYSTEM
Zdrav Vestn | November – December 2021 | Volume 90 | https://doi.org/10.6016/ZdravVestn.3180
Copyright (c) 2021 Slovenian Medical Journal. This work is licensed under a
Creative Commons Attribution-NonCommercial 4.0 International License.
The first successful double switch operation for congenitally 
corrected transposition of the great arteries in Slovenia
Prva popolnoma uspešna poprava pri prirojeni popravljeni transpoziciji velikih arterij 
v Sloveniji
Miha Weiss,1 Anže Djordjević,2 Roman Gebauer,3 Miroslav Elek,4 Mirko Topalović,4 Teja Senekovič,4 Andraž 
Zupan,5 Matija Jelenc,1 Mirta Koželj4,6
Abstract
Congenitally corrected transposition of the great arteries is a rare congenital heart defect characterized by atrioventricular 
and ventriculoarterial discordance and can be potentially associated with several other concomitant anomalies, such as 
ventricular septal defect or congenital complete atrioventricular block. Different surgical options are used for treating the 
condition. Anatomic repair yields the best long-term outcomes; however, it is technically the most challenging. We present 
a case of a 3-year old female patient who was electively admitted for complete repair after pulmonary artery banding and 
pacemaker implantation soon after birth. Subsequently, an anatomic repair (double switch) was performed with an unevent-
ful postoperative course and excellent mid-term outcomes.
Izvleček
Prirojeno popravljena transpozicija velikih arterij je redka prirojena srčna napaka, pri kateri gre za neskladje med odno-
si preddvorov in prekatov ter prekatov in izstopajočih velikih arterij (aorta, pljučna arterija). Pridružene so lahko tudi dru-
ge napake, kot sta okvara medprekatnega pretina ali prirojeni popolni preddvorno-prekatni blok. Obstaja več možnosti 
Slovenian Medical Journallovenian Medical Journal
1 Department of Cardiovascular Surgery, Divison of Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
2 Department of Cardiac Surgery, University Medical Centre Maribor, Maribor, Slovenia
3 Division of Surgery, Children’s Heart Center, 2nd Faculty of Medicine, Charles University in Prague and Motol University,Prague, Czech 
   Republic
4 Department of Paediatric Cardiology, University Medical Centre Children’s Hospital Ljubljana, Ljubljana, Slovenia
5 Institute of Radiology, University Medical Centre Ljubljana, Ljubljana, Slovenia
6 Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Correspondence / Korespondenca: Anže Djordjević, e: anze.djordjevic@ukc-mb.si
Key words: congenitally corrected transposition of the great arteries; ventricular septal defect; congenital complete atrioventricular block; 
double switch procedure; congenital heart surgery
Ključne besede: prirojeno popravljena transpozicija velikih arterij; okvara medprekatnega pretina; prirojeni popolni preddvorno-prekatni 
blok; popolno kirurško popravilo; otroška srčna kirurgija
Received / Prispelo: 16. 10. 2020 | Accepted / Sprejeto: 13. 11. 2020
Cite as / Citirajte kot: Weiss M, Djordjević A, Gebauer R, Elek M, Topalović M, Senekovič T, et al.. The first successful double switch 
operation for congenitally corrected transposition of the great arteries in Slovenia. Zdrav Vestn. 2021;90(11–12):624–7. DOI: https://doi.
org/10.6016/ZdravVestn.3180
eng slo element
en article-lang
10.6016/ZdravVestn.3180 doi
16.10.2020 date-received
13.11.2020 date-accepted
Cardiovascular system Srce in obtočila discipline
Case report Klinični primer article-type
The first successful double switch operation 
for congenitally corrected transposition of the 
great arteries in Slovenia
Prva popolnoma uspešna poprava pri prirojeni 
popravljeni transpoziciji velikih arterij v Sloveniji article-title
The first successful double switch operation 
for congenitally corrected transposition of the 
great arteries in Slovenia
Prva popolnoma uspešna poprava pri prirojeni 
popravljeni transpoziciji velikih arterij v Sloveniji alt-title
congenitally corrected transposition of the 
great arteries, ventricular septal defect, 
congenital complete atrioventricular block, 
double switch procedure, congenital heart 
surgery
prirojeno popravljena transpozicija velikih arterij, 
okvara medprekatnega pretina, prirojeni popol-
ni preddvorno-prekatni blok, popolno kirurško 
popravilo, otroška srčna kirurgija
kwd-group
The authors declare that there are no conflicts 
of interest present.
Avtorji so izjavili, da ne obstajajo nobeni 
konkurenčni interesi. conflict
year volume first month last month first page last page
2021 90 11 12 624 627
name surname aff email
Anže Djordjević 2 anze.djordjevic@ukc-mb.si
name surname aff
Miha Weiss 1
Roman Gebauer 3
Miroslav Elek 4
Mirko Topalović 4
Teja Senekovič 4
Andraž Zupan 5
Matija Jelenc 1
Mirta Koželj 6
eng slo aff-id
Department of Cardiovascular 
Surgery, Division of Surgery, 
University Medical Centre 
Ljubljana, Ljubljana, Slovenia
Klinični oddelek za kirurgijo 
srca in ožilja, Kirurška klinika, 
Univerzitetni klinični center 
Ljubljana, Ljubljana, Slovenija 
1
Department of Cardiac Surgery, 
University Medical Centre 
Maribor, Maribor, Slovenia
Oddelek za kardiokirurgijo, 
Univerzitetni klinični center 
Maribor, Maribor, Slovenija 
2
Division of Surgery, Children’s 
Heart Center, 2nd Faculty of 
Medicine, Charles University 
in Prague and Motol 
University,Prague, Czech 
Republic
Division of Surgery, Children’s 
Heart Center, 2nd Faculty of 
Medicine, Charles University in 
Prague and Motol University, 
Praga, Češka republika
3
Department of Paediatric 
Cardiology, University Medical 
Centre Children’s Hospital 
Ljubljana, Ljubljana, Slovenia
Oddelek za otroško kardiologijo, 
Univerzitetni klinični center 
Ljubljana, Ljubljana, Slovenija
4
Institute of Radiology, 
University Medical Centre 
Ljubljana, Ljubljana, Slovenia
Klinični inštitut za radiologijo, 
Univerzitetni klinični center 
Ljubljana, Ljubljana, Slovenija
5
Faculty of Medicine, University 
of Ljubljana, Ljubljana, Slovenia
Medicinska fakulteta, Univerza v 
Ljubljani, Ljubljana, Slovenija 6
625
CASE REPORT
The first successful double switch operation for congenitally corrected transposition of the great arteries in Slovenia
1 Introduction
Congenitally corrected transposition of the great ar-
teries (ccTGA) is a combination of discordant atrioven-
tricular (AV) and ventriculoarterial connections (1). 
With an incidence of 1/33.000 live births ccTGA accounts 
for 0.05% of congenital cardiac malformations (2). Since 
both ventricles and great arteries (aorta and pulmonary 
artery (PA)) are transposed, normal physiology of blood 
flow is observed (3). Deoxygenated blood returns to the 
right atrium (RA) and through the mitral valve (MV) into 
the left ventricle (LV), pumping it through the pulmonary 
valve into the PA. Oxygenated blood returns to the left 
atrium (LA) and through the tricuspid valve (TV) into 
the right ventricle (RV), which in turn pumps it against 
systemic pressure through the aortic valve into the aorta. 
RV failure and/or TV regurgitation are the most common 
indications for surgical repair, followed by concomitant 
anomalies, such as ventricular septal defect (VSD), con-
genital complete AV block or LV outflow tract (LVOT) 
obstruction (4). Presenting with AV and ventriculoar-
terial discordance, it is plausible that these patients live 
without surgical corrections into adulthood due to almost 
physiological blood flow. Individuals with ccTGA present 
a broad spectrum of clinical presentations, from asymp-
tomatic to heart failure or even sudden cardiac deaths (5). 
Transthoracic echocardiography with computed tomog-
raphy or magnetic resonance angiography adding valu-
able information regarding coronary artery anatomy re-
mains the golden diagnostic standard. VSDs are common 
in patients with ccTGA with reported incidence of 60% 
of all affected individuals (6). A dysplastic TV is found in 
75% of cases with VSDs (3). The congenital AV block is 
the consequence of atrial and ventricular septal malalign-
ment and extensive membranous septum filling the gap, 
thus preventing the AV node to lie appropriately in the 
triangle of Koch (3). Here, we present the first Slovenian 
successfully treated case of ccTGA managed at the Uni-
versity Medical Centre Ljubljana, Slovenia.
2 Case presentation
A 3-year old Caucasian female child with diagnosed 
ccTGA after PA banding (performed 2 days after birth) 
intended for LV pre-conditioning and a single chamber 
VVI pacemaker (PM) implantation (in order to spare 
kirurškega zdravljenja. Popolno anatomsko popravilo prinaša najboljše dolgoročne rezultate, vendar je tehnično najzahtev-
nejše. Prikazujemo primer triletne deklice, ki je imela prvo operacijo kmalu po rojstvu (bandažiranje pljučne arterije, vstavitev 
srčnega spodbujevalnika), zdaj pa je bilo uspešno opravljeno popolno popravilo z odličnim srednjeročnim izidom zdravljenja.
the atrial tissue until final correction) due to a congenital 
complete AV block was admitted to the Department of 
Paediatric Cardiology for definite treatment. Preopera-
tive transthoracic echocardiogram showed an 8-mm large 
non-restrictive perimembranous VSD with a left-to-right 
shunt, a good functioning LV without obstruction in the 
LVOT, a hypertrophic RV with an ejection fraction of 65% 
and without obstruction in the RVOT, a dysplastic TV 
with moderate to severe regurgitation (indexed vena con-
tracta 6 mm) and without stenosis (Figure 1). Abnormal 
coronary artery anatomy with a common origin of both 
coronaries from the left coronary sinus was confirmed on 
a computed tomography scan (Figure 2). There was no 
need to perform invasive coronary angiography (7). At 
surgery, a standardized double switch operation was per-
formed (8). Standard median re-sternotomy with distal 
ascending aorta and bicaval cannulation was performed. 
Under mild hypothermia, the aorta was cross-clamped 
and the heart was arrested using antegrade cardioplegia. 
With the right transatrial approach, the perimembranous 
VSD was closed using a Dacron (a condensation polymer 
of ethylene glycol and terephthalic acid) patch. The MV 
was functioning well and did not require any surgical re-
pair. Then, the aorta and PA were separated and a single 
coronary button was excised. A pericardial patch was used 
to reconstruct the neo-PA. The PA band was removed and 
Figure 1: Preoperative transthoracic echocardiogram 
image in four-chamber view depicting atrioventricular and 
ventriculoarterial discordance.
Legend: LA – left atrium, RA – right atrium, LV – morphologically 
left ventricle, RV – morphologically right ventricle.
Image is from authors’ own archive.
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CARDIOVASCULAR SYSTEM
Zdrav Vestn | November – December 2021 | Volume 90 | https://doi.org/10.6016/ZdravVestn.3180
the coronary button was reimplanted into the neo-aorta. 
Both the neo-PA and neo-aorta were switched (without 
the Lecompte manoeuvre) and anastomosed to their re-
spective counterparts. Finally, the Senning procedure or 
the atrial switch was performed. With the Senning sur-
gical repair, a baffle is created within the atria, which re-
routes deoxygenated blood from both caval veins to the 
MV and onwards to the pulmonary circulation. The baf-
fle was constructed using RA and interatrial septal tissue 
as well as bovine pericardium. The right atriotomy was 
closed using in situ pericardium in order to prevent RA 
reduction. After de-airing, the cross-clamp was removed 
and the heart spontaneously reanimated. New atrial and 
ventricular epicardial PM leads were implanted. After 
weaning from cardiopulmonary bypass, haemostasis 
was achieved and the sternal wound closed in layers. The 
patient was extubated on the second postoperative day 
(POD) and discharged from the hospital on POD 12. 
Postoperative transthoracic echocardiography showed 
no obstruction in the atrial reconstruction, mild TV 
regurgitation, no other valvular pathology, appropriate 
biventricular function, no residual VSD, and no pericar-
dial effusion (Figures 3 and 4). The anatomic restoration 
of AV and ventriculoarterial connections was achieved. 
Due to coronary artery reinsertion into the neo-aorta, 
acetylsalicylic acid at a dose of 1mg/kg was added to her 
therapy for 6 months.
3 Discussion
ccTGA is a rare clinical entity with unique charac-
teristics. Adequate preoperative diagnostics (i.e. trans-
thoracic echocardiography, computed tomography) is 
crucial for successful timing of the surgery and good 
long-term outcomes. During surgery, either biventricular 
Figure 2: Computed tomography angiography image 
depicting a single origin of both coronary arteries with left 
anterior descending (LAD) arising from the right coronary 
artery.
Legend: LCX – left circumflex artery, RCA – right coronary 
artery, LCA  – left coronary artery, AA –  ascending aorta.
Image is from authors’ own archive.
Figure 3: Postoperative transthoracic echocardiography 
image in four-chamber view with colour flow Doppler 
depicting pulmonary (left arrow) and systemic venous 
baffle (right arrow).
Legend: LV – morphologically left ventricle, RV – 
morphologically right ventricle.
Image is from authors’ own archive.
Figure 4: Postoperative transthoracic echocardiography 
image in four-chamber view with colour flow Doppler 
depicting ventriculoarterial concordance.
Legend: LV – morphologically left ventricle, Ao – aorta.
Image is from authors’ own archive.
627
CASE REPORT
The first successful double switch operation for congenitally corrected transposition of the great arteries in Slovenia
or univentricular surgical correction is indicated. Biven-
tricular correction can be either physiological, meaning 
that only associated anomalies are corrected (VSD repair, 
TV replacement), and anatomical, which aims to restore 
anatomic relations, focusing especially on removing the 
RV from pumping into the systemic circulation (1). If 
an anatomic repair is not technically feasible, the Fontan 
palliative procedure is advocated with comparable mid-
term results. Factors, precluding anatomic repair, are ab-
errant coronary artery anatomy, AV valves’ straddling, 
dextrocardia or a very large area of septation required 
for VSD closure (9). We opted for an anatomic repair 
despite the coronary anomaly (single origin of both cor-
onary arteries), partially because of favourable position 
regarding the neo-aorta (origin from the left coronary 
sinus). Anatomic repair or double switch is a challeng-
ing surgical procedure, which requires an experienced 
team of surgeons, paediatricians, anaesthesiologists and 
intensivists. Due to small numbers of children with con-
genital heart defects in Slovenia (approximately 120 per 
year); double switch was not yet performed at our centre. 
These children were offered either physiologic or pallia-
tive repair or were transferred to a high-volume centre. 
With this case, we offer a new therapeutic option for  pa-
tients diagnosed with ccTGA in Slovenia. With encour-
aging mid-term results, we are aiming to achieve similar 
long-term results comparable to high-volume centres, 
where the survival rate is 80% 15 years after the double 
switch procedure (10,11).
4 Conclusion
To conclude, we have presented a case of a 3 years old 
female child with ccTGA and several associated defects 
(VSD, coronary artery anomaly, congenital complete 
AV block and dysplastic TV with significant regurgita-
tion), which required a multidisciplinary approach and 
two surgical interventions before a complete correction 
of the anomaly. PA banding was performed and a PM 
implanted in the neonatal period. Limitation in physical 
activity, a VSD and TV regurgitation were indications 
for surgery. After accurate non-invasive imaging diag-
nostics only, a standardized double switch operation was 
successfully performed. Anatomic repair in ccTGA is 
the preferred surgical method; however, it requires an 
experienced surgical team with good support from oth-
er modalities (paediatric cardiology, anaesthesiology, 
intensive care unit, radiology). This is the first successful 
case of a double switch surgical repair for complex ccT-
GA with several associated heart anomalies in Slovenia 
with excellent mid-term outcomes.
Conflict of interest
None declared.
Informed consent of the patient
The parent of a case patient signed the informed con-
sent form for the publication.
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