Journal of Cardiology Cases 22 (2020) 136–139

Contents lists available at ScienceDirect

Journal of Cardiology Cases

journal homepage: www.elsevier.com/locate/jccase

Case Report

Three-dimensional visualization of the left atrium by intracardiac

echocardiography facilitates trans-septal catheterization and atrial
fibrillation catheter ablation in cor triatriatum sinister: A case report
and literature review
Itsuro Morishima (MD, PhD)*, Yasunori Kanzaki (MD), Koichi Furui (MD),
Ryota Yamauchi (MD), Yasuhiro Morita (MD), Hideyuki Tsuboi (MD, PhD, FJCC)
Department of Cardiology, Ogaki Municipal Hospital, Ogaki, Japan

A R T I C L E I N F O A B S T R A C T

Article history: A key to the success of catheter ablation in complex congenital heart disease is an accurate delineation of
Received 8 March 2020 the anatomy. Here we describe the efficiency of intracardiac echocardiogram in guiding the catheter
Received in revised form 12 May 2020 ablation of persistent atrial fibrillation in a 55-year-old Japanese male with cor triatriatum sinister. Echo
Accepted 16 May 2020
imaging provided a detailed three-dimensional anatomy of the whole left atrium and identified an ideal
trans-septal puncture site that allowed catheter access to both the accessory and main chambers of the
Keywords: left atrium. We review similar cases from the literature.
Atrial fibrillation
<Learning objective: Cor triatriatum sinister is a rare cardiac anomaly wherein a fibromuscular
Catheter ablation
Cor triatriatum sinister
membrane divides the left atrium into two parts. An accurate delineation of the entire left atrial anatomy
Intracardiac echocardiography including the membrane by intracardiac echocardiography may facilitate the catheter ablation procedure
Review of atrial fibrillation, especially when deciding the optimal trans-septal catheterization site to map both
left atrial chambers.>
© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Introduction Case report

Cor triatriatum sinister is a rare cardiac anomaly with an
incidence of 0.1–0.4% of the children with congenital heart disease
[1,2]. The condition is characterized by the presence of a
fibromuscular membrane that divides the left atrium (LA) into a
postero-superiorly located accessory chamber (AC) which receives
the pulmonary veins (PVs) and an antero-inferiorly located main
chamber (MC) which contains the LA appendage and mitral
annulus. When LA mapping is performed in a patient with cor
triatriatum sinister, the fibromuscular membrane limits the
manipulation of a catheter depending on the site of the trans-
septal catheterization (TSC) [3,4]. Our patient's case provides an
example of this.
* Corresponding author at: Department of Cardiology, Ogaki Municipal Hospital,
4-86 Minaminokawa-cho, Ogaki 503-8502, Japan.
E-mail address: morishima-i@muc.biglobe.ne.jp (I. Morishima).
A 55-year-old Japanese male with persistent atrial fibrillation
(AF) was referred for catheter ablation. Three-dimensional (3D)
computed tomography detected the diaphragm on the left side of
the interatrial septum separating the LA into two parts (Fig. 1A and
B), which we diagnosed as cor triatriatum sinister. Prior to the TSC,
an intra-cardiac echo probe (SOUNDSTAR, Biosense Webster,
Irvine, CA, USA) was advanced to the right atrium. The endocardial
contours of the LA, PVs, and intra-atrial membrane were obtained
with the CARTOSoundTM system (Biosense Webster) to generate a
detailed 3D reconstruction of the LA (Fig. 1C–I). The images
indicated that the entire fossa ovalis faced the AC and that the
access to the MC would be impossible due to the membranous
structure if TSC was performed in the posterior region of the fossa
ovalis (Fig. 1C–E). Accordingly, the TSC was performed anteriorly
(Fig. 1F). Three sheaths were advanced without difficulty through
the single trans-septal puncture site with two sheaths in the AC
and one sheath in the MC (Fig. 2A). The 3D geometry of both LA
chambers was created by the direct contact of an electrode
catheter to the LA wall (Fig. 2B and C).

https://doi.org/10.1016/j.jccase.2020.06.002
1878-5409/© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
 I. Morishima et al. / Journal of Cardiology Cases 22 (2020) 136–139 137

The left atrium of our patient with cor triatriatum sinister, reconstructed by cardiac computed tomography (CT) (A,B) and intracardiac echocardiography (C–I). (A,
B) The CT images distinguished a membranous structure (orange arrows) dividing the left atrium (LA) into two parts: a postero-superior accessory chamber (AC)
and an antero-inferior main chamber (MC). The serial cross-sectional echo images (C–G) correspond to the directions of the red arrows shown on the cranial view
Fig. 1. of the 3D reconstruction of the LA (H). The fenestration (dotted lines in F,G) was detected in the antero-inferior portion of the membrane (orange arrows). (I) The
semi-transparent 3D LA images clearly demonstrate the membrane structure and the fenestration. The trans-septal catheterization was performed aiming at the
anterior portion of the fossa ovalis so that the catheter can cross the fenestration to enter the MC (F). LAA, left atrial appendage; LIPV, left inferior pulmonary vein;
LSPV, left superior pulmonary vein; RIPV, right inferior pulmonary vein; RSPV, right superior pulmonary vein.

After the patient's AF was converted to sinus rhythm by
electrical cardioversion, no spontaneous triggering of premature
atrial contractions appeared to induce AF. We performed radio-
frequency catheter ablation in order to achieve extensive encircling
PV isolation with an irrigation catheter (THERMOCOOL SMART-
TOUCH, Biosense Webster), by using an SL1 sheath. The procedure
was completed without any difficulty in manipulating the catheter
within the AC. Intravenous adenosine triphosphate did not
provoke any dormant PV reconnections. In addition, the superior
vena cava was empirically isolated. No atrial tachycardia was
induced by rapid atrial pacing, and the session was closed. The
patient has been off anti-arrhythmic drugs and free from AF
recurrence for the subsequent 3 years.
Discussion
Our patient's case highlights the importance of the 3D
visualization of the LA in a patient with cor triatriatum sinister
undergoing LA ablation. An accurate delineation of the patient's
entire LA anatomy, which is a key to the success of anatomically
based ablation procedures for AF, was fully obtainable. An ideal TSC
site that allowed catheter access to both the AC and MC was
precisely determined.
In cor triatriatum sinister, communication between the two LA
chambers usually occurs through the fenestration in the fibro-
muscular membrane [1,2,5]. The opening in the membrane varies
in diameter and location depending on the individual patient. The
hemodynamic consequences and symptoms of cor triatriatum
sinister resemble those of mitral stenosis, and the symptoms are
related to the degree of obstruction across the membrane, which is
classified as follows: type 1 is no opening, type 2 is a small opening,
and type 3 is a large communication through the membrane [5]. In
general, patients without any associated cardiac anomalies are
symptom-free when the diameter of the fenestration is >1 cm.
However, later in life, these patients may develop AF [6], and this
was the situation for our patient.
Isolation of the PV in a patient with cor triatriatum sinister was
first reported in 2008 [3]; TSC was performed in that patient under
fluoroscopic guidance to enter the MC directly, which required the
catheter to cross the membrane inferiorly back to the AC to ablate
PVs. Due to recurrence, the patient underwent a second session in
which TSC was done posteriorly into the AC. The catheter
138 I. Morishima et al. / Journal of Cardiology Cases 22 (2020) 136–139

The left atriography (A) and the 3D geometry created by the direct contact of an electrode catheter (B,C) illustrating the accessory chamber (AC) and the main
chamber (MC). Three sheaths were advanced through the single trans-septal puncture site with two in the AC and one in the MC. The 3D geometry demonstrates
Fig. 2.
an optimal access to both left atrial chambers. Orange arrows indicate the LA membranous structure. LAA, left atrial appendage; LSPV, left superior pulmonary
vein; RAO, right anterior oblique projection; RSPV, right superior pulmonary vein.

manipulation in the AC was much easier compared to the index
session [4], demonstrating the importance of directing the TSC to
the target chamber.
There have since been an additional five case reports
describing AF ablation in patients with cor triatriatum sinister
[7–11] (Table 1). TSC was guided by intracardiac echography in
four of the patients [7,9–11] and by transesophageal echocardi-
ography in one patient [8] to directly enter the AC. PV isolation
was successful in all five patients. However, access to the MC is a
prerequisite if the ablation of an AF substrate and non-PV foci is
necessary in areas such as an LA appendage, mitral annulus, or the
anterior wall of the LA.

Table 1 Catheter ablation of atrial fibrillation in patients with cor triatriatum sinister.

Case Authors/year Age/gender Type Associated AF TSC Procedure Outcome

congenital
heart defect

1 Yamada T/2008 [3] 66/M 3 None Paroxysmal Fluoroscopy-guided PVI AF recur. >3 mos.
2 Yamada T/2009 [4]a 66/M 3 None Paroxysmal Fluoroscopy-guided PVI (re-do) AF-free, off AAD at 6 mos.
3 Bhatia NL/2010 [7] 72/M 3 None Paroxysmal ICE-guided PVI + CTI AF-free, off AAD at 6 mos.
4 Gavin A/2011 [8] 64/M 3 None Long-standing TEE-guided PVI + LA AF-free, off AAD at 7 mos.
persistent roof + MI + CFAE
5 Fukumoto K/2012 [9] 58/M 3 None Paroxysmal ICE-guided PVI AF-free, off AAD at 24 mos.
6 Tokuda M/2016 [10] 57/M 3 None Persistent ICE-guided PVI + LAPWI AF-free, off AAD, at 12 mos.
7 Borne RT/2016 [11] 51/M 3 None Paroxysmal ICE-guided PVI + LA AF-free, off AAD, at 12 mos.
roof + MI + CTI
8 Present case 56/M 3 None Persistent ICE-guided PVI + SVCI AF-free, off AAD, at 36 mos.
a
The repeated session of case 1.
AAD, anti-arrhythmic drugs; AF, atrial fibrillation; CFAE, complex fractionated electrogram; CTI, cavo-tricuspid isthmus; ICE, intra-cardiac echocardiography; LA, left atrial;
LAPWI, left atrial posterior wall isolation; MI, mitral isthmus; PVI, pulmonary vein isolation; SVCI, superior vena cava isolation; TEE, trans-esophageal echocardiography; TSC,
trans-septal catheterization.
 I. Morishima et al. / Journal of Cardiology Cases 22 (2020) 136–139
In our patient's case, additional ablations should have been
technically possible via the single TSC site. The access to the MC
was secured as shown by the 3D geometry created by direct
catheter contact to the LA wall (Fig. 2B and C), and the detailed LA
structure including the membrane was reconstructed by the
intracardiac echocardiogram, which provided anatomical guidance
within both the MC and AC of the LA.
It should also be noted that none of the patients in the case
series including the present patient had AF recurrence without
anti-arrhythmic medications at follow-up, although one
patient required a repeated procedure [4] (Table 1). The
patients share the following characteristics: (1) They were
middle-aged or post middle-aged men, (2) they had no
associated congenital heart defects, (3) they had type III,
and (4) they developed AF at an advanced age. Cather ablation
may be a reasonable option in this type of patients with cor
triatriatum sinister.
In conclusion, in patients with cor triatriatum sinister, a
detailed delineation of the entire LA anatomy including the
membrane by intracardiac echocardiography may facilitate the
catheter ablation of AF, especially when deciding the optimal TSC
site to map both LA chambers.
Conflict of interest
The authors declare that there is no conflict of interest.
139
References
[1] Niwayama G. Cor triatriatum. Am Heart J 1960;59:291–317.
[2] Jorgensen CR, Ferlic RM, Varco RL, Lillehei CW, Eliot RS. Cor triatriatum. Review
of the surgical aspects with a follow-up report on the first patient successfully
treated with surgery. Circulation 1967;36:101–7.
[3] Yamada T, Tabereaux PB, McElderry HT, Kay GN. Successful catheter ablation
of atrial fibrillation in a patient with cor triatriatum sinister. Heart Rhythm
2008;5:903–4.
[4] Yamada T, Tabereaux PB, McElderry HT, Doppalapudi H, Kay GN. Transseptal
catheterization in the catheter ablation of atrial fibrillation in a patient with
cor triatriatum sinister. J Interv Card Electrophysiol 2009;25:79–82.
[5] Loeffler E. Unusual malformation of the left atrium: pulmonary sinus. Arch
Pathol Lab Med 1949;48:371–6.
[6] Modi KA, Annamali S, Ernest K, Pratep CR. Diagnosis and surgical correction of cor
triatriatum in an adult: Combined use of transesophageal and contrast echocar-
diography, and a review of literature. Echocardiography 2006;23:506–9.
[7] Bhatia NL, Humphries J, Chandrasekaran K, Srivathsan K. Atrial fibrillation
ablation in cor triatriatum: value of intracardiac echocardiography. J Interv
Card Electrophysiol 2010;28:153–5.
[8] Gavin A, Singleton CB, McGavigan AD. Successful multi-chamber catheter
ablation of persistent atrial fibrillation in cor triatriatum sinister. Indian Pacing
Electrophysiol J 2011;11:50–5.
[9] Fukumoto K, Takatsuki S, Miyoshi S, Tanimoto K, Nishiyama N, Aizawa Y, et al.
Cor triatriatum sinister: an incidental finding in a patient with paroxysmal
atrial fibrillation. Herz 2012;37:217–8.
[10] Tokuda M, Yamane T, Tokutake K, Yokoyama K, Hioki M, Narui R, et al. Catheter
ablation of persistent atrial fibrillation in a patient with cor triatriatum sinister
demonstrating a total common trunk of the pulmonary vein. Heart Vessels
2016;31:261–4.
[11] Borne RT, Gonzalez J, Khanna A, Sauer WH, Thai Nguyen D. Getting to the right
left atrium: catheter ablation of atrial fibrillation and mitral annular flutter in
cor triatriatum. Heart Rhythm Case Rep 2016;2:502–5.