Journal of Cardiology Cases 22 (2020) 136–139

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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 A 55-year-old Japanese male with persistent atrial fibrillation
incidence of 0.1–0.4% of the children with congenital heart disease (AF) was referred for catheter ablation. Three-dimensional (3D)
[1,2]. The condition is characterized by the presence of a computed tomography detected the diaphragm on the left side of
fibromuscular membrane that divides the left atrium (LA) into a the interatrial septum separating the LA into two parts (Fig. 1A and
postero-superiorly located accessory chamber (AC) which receives B), which we diagnosed as cor triatriatum sinister. Prior to the TSC,
the pulmonary veins (PVs) and an antero-inferiorly located main an intra-cardiac echo probe (SOUNDSTAR, Biosense Webster,
chamber (MC) which contains the LA appendage and mitral Irvine, CA, USA) was advanced to the right atrium. The endocardial
annulus. When LA mapping is performed in a patient with cor contours of the LA, PVs, and intra-atrial membrane were obtained
triatriatum sinister, the fibromuscular membrane limits the with the CARTOSoundTM system (Biosense Webster) to generate a
manipulation of a catheter depending on the site of the trans- detailed 3D reconstruction of the LA (Fig. 1C–I). The images
septal catheterization (TSC) [3,4]. Our patient's case provides an indicated that the entire fossa ovalis faced the AC and that the
example of this. 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
* Corresponding author at: Department of Cardiology, Ogaki Municipal Hospital,
and one sheath in the MC (Fig. 2A). The 3D geometry of both LA
4-86 Minaminokawa-cho, Ogaki 503-8502, Japan. chambers was created by the direct contact of an electrode
E-mail address: morishima-i@muc.biglobe.ne.jp (I. Morishima). 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 site that allowed catheter access to both the AC and MC was
electrical cardioversion, no spontaneous triggering of premature precisely determined.
atrial contractions appeared to induce AF. We performed radio- In cor triatriatum sinister, communication between the two LA
frequency catheter ablation in order to achieve extensive encircling chambers usually occurs through the fenestration in the fibro-
PV isolation with an irrigation catheter (THERMOCOOL SMART- muscular membrane [1,2,5]. The opening in the membrane varies
TOUCH, Biosense Webster), by using an SL1 sheath. The procedure in diameter and location depending on the individual patient. The
was completed without any difficulty in manipulating the catheter hemodynamic consequences and symptoms of cor triatriatum
within the AC. Intravenous adenosine triphosphate did not sinister resemble those of mitral stenosis, and the symptoms are
provoke any dormant PV reconnections. In addition, the superior related to the degree of obstruction across the membrane, which is
vena cava was empirically isolated. No atrial tachycardia was classified as follows: type 1 is no opening, type 2 is a small opening,
induced by rapid atrial pacing, and the session was closed. The and type 3 is a large communication through the membrane [5]. In
patient has been off anti-arrhythmic drugs and free from AF general, patients without any associated cardiac anomalies are
recurrence for the subsequent 3 years. symptom-free when the diameter of the fenestration is >1 cm.
However, later in life, these patients may develop AF [6], and this
Discussion was the situation for our patient.
Isolation of the PV in a patient with cor triatriatum sinister was
Our patient's case highlights the importance of the 3D first reported in 2008 [3]; TSC was performed in that patient under
visualization of the LA in a patient with cor triatriatum sinister fluoroscopic guidance to enter the MC directly, which required the
undergoing LA ablation. An accurate delineation of the patient's catheter to cross the membrane inferiorly back to the AC to ablate
entire LA anatomy, which is a key to the success of anatomically PVs. Due to recurrence, the patient underwent a second session in
based ablation procedures for AF, was fully obtainable. An ideal TSC 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 four of the patients [7,9–11] and by transesophageal echocardi-
session [4], demonstrating the importance of directing the TSC to ography in one patient [8] to directly enter the AC. PV isolation
the target chamber. was successful in all five patients. However, access to the MC is a
There have since been an additional five case reports prerequisite if the ablation of an AF substrate and non-PV foci is
describing AF ablation in patients with cor triatriatum sinister necessary in areas such as an LA appendage, mitral annulus, or the
[7–11] (Table 1). TSC was guided by intracardiac echography in 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) 136–139 139

In our patient's case, additional ablations should have been References

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Conflict of interest [11] Borne RT, Gonzalez J, Khanna A, Sauer WH, Thai Nguyen D. Getting to the right
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The authors declare that there is no conflict of interest.