© 2013, Wiley Periodicals, Inc.
DOI: 10.1111/echo.12490

Echocardiography

Cor Triatriatum Sinistrum Presenting as Cardioembolic
Stroke: An Unusual Cause of Adolescent Hemiparesis
Anil Minocha, M.B.B.S., D.N.B., M.N.A.M.S., F.N.I.C., F.T.E.E.,* Sanjeev Gera, M.B.B.S., M.D., D.N.B.,*
Nishith Chandra, M.B.B.S., M.D., D.M.,* Atampreet Singh, M.B.B.S., M.D., D.M.,† and Sanjay Saxena,
M.B.B.S., M.D., D.M.†
*Department of Cardiology, Fortis Escorts Heart Institute, Fortis Hospital Noida, Noida, India; and
†Department of Neurology, Fortis Hospital, Noida, India

Cor triatriatum sinistrum is a rare congenital cardiac malformation, in which the left atrium (LA) is
divided into two distinct chambers by a fibromuscular membrane. Classically, the proximal (upper or
superior) chamber of the LA receives pulmonary venous connections, whereas the distal (lower or inferior) chamber contains LA appendage and true atrial septum containing fossa ovalis. The distal chamber
is in continuity with the atrioventricular valve, while the two chambers communicate through a defect
in the membrane. The hemodynamics of cor triatriatum sinistrum are similar to that of mitral stenosis
due to obstructive property of membrane. The majority of reported cases of cor triatriatum occur in
infants with symptoms of pulmonary venous obstruction, with adult cases being rare. Herein, we
describe an unusual case of cor triatriatum in a 17-year-old boy who presented for the first time with
embolic cerebral infarction with left hemiparesis. (Echocardiography 2014;31:E120–E123)
Key words: cor triatriatum
Case Summary:
A 17-year-old previously healthy male who was
admitted in the department of neurology with
sudden onset left hemiparesis and dysarthria was
referred to the cardiology department for further
investigation of suspected cardioembolic stroke.
Computed tomography (CT) (GE Medical Systems, light speed, VCT, 64-slice, Waukesha, WI.,
USA) and magnetic resonance imaging (MRI)
(Signa Excite 1.5 Tesla, GE Medical Systems) of
brain had revealed infarction of right middle
cerebral artery territory of brain (Fig. 1). He had
uneventful medical history and had no significant
risk factors for stroke, nor any congenital disorder
of coagulation and no significant family history
of cerebrovascular disease. There was no previous
history of heart disease although he confirmed
occasional shortness of breath on exertion with
palpitations for last 1 year. Blood pressure was
normal at the time of presentation and during
the hospital stay. Positive findings on physical
examination included an irregularly irregular
pulse and a faint holodiastolic murmur at the cardiac apex. Electrocardiogram (ECG) (Sanborn
Series, Philips Medical Systems, Andover, MA,
Address for correspondence and reprint requests: Anil Minocha,
Department of Cardiology, Fortis Escorts Heart Institute,
Fortis Hospital Noida, Noida, India 201301. Fax: +91-1202403222; E-mail: dr_anilminocha@yahoo.com

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USA) showed atrial fibrillation with a fast ventricular rate. Chest radiograph showed a dilated
atrial shadow. Laboratory data including risk factors for hypercoagulopathy (which included antithrombin III, protein C, protein S, and
antiphospholipid antibodies) were not remarkable and bilateral carotid Doppler ultrasound was
free of any atheromatous plaque.
A two-dimensional transthoracic echocardiography (TTE) (HD-11XE, Philips Ultrasound,
Bothell, WA, USA) revealed a large left atrium
(LA) which was divided into two by a membrane
like echo dense structure (movie clip 1). This was
attached medially to the interatrial septum (IAS)
and laterally to the lateral wall of LA adjacent to
the left atrial appendage (LAA), giving rise to a
proximal small postero-superior chamber, and a
distal large anterior-inferior chamber of LA, suggesting a cor triatriatum (Figs. 2 and 3). The
membrane showed pronounced movement during the cardiac cycle (movie clip 2.). The transesophageal echocardiography (TEE) (HD-11XE)
was done to compliment TTE findings (Fig. 4)
(movie clip 3) and to search for embolic source.
On TEE, thick spontaneous echo contrast (SEC)
was seen in the distal chamber and a soft clot
was seen in LAA, which was presumed to be the
cardioembolic source (Fig. 5). There was no significant pressure gradient observed within the LA
using color flow Doppler imaging. The IAS was

Cor Triatriatum Sinistrum

Figure 1. Diffusion-weighted magnetic resonance imaging of the brain shows high signal intensity in the right cerebral hemisphere suggestive of an acute ischemic infraction in the territory of the right middle cerebral artery (arrows).

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Minocha, et al.

Figure 2. Transthoracic modified four-chamber (4C) echocardiographic view showing membrane (arrow) crossing left
atrium (LA) and dividing it into upper and lower chamber.
Note lower chamber containing left atrial appendage (LAA) is
full of spontaneous echo contrast (SEC). Left ventricle appears
obliterated due to modified 4C view.

Figure 5. Modified transesophageal echocardiographic
(TEE) view shows left atrial appendage containing clot
(arrow).

intact and all pulmonary veins drained into the
proximal superior chamber. The distal inferior
chamber, which contained the LAA, communicated with the left ventricle through a morphologically normal mitral valve. There was no other
associated abnormality seen on echocardiography.
The final diagnosis rendered in the adolescent
was embolic stroke associated with cor triatriatum sinister, atrial fibrillation, and LAA clot. The
patient was managed conservatively with anticoagulation therapy which was tolerated well. His
hemiparesis and dysarthria gradually improved
and to date he has not shown any evidence of
recurrence.
Figure 3. Another transthoracic modified echocardiographic
view showing fibromuscular membrane (arrow) crossing left
atrium (LA).

Figure 4. Transesophageal echocardiographic (TEE) view
showing fibromuscular membrane attached to left atrial
appendage (LAA) (arrow). Interatrial septum (IAS) is seen on
left (double arrow).

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Discussion:
Cor triatriatum is a rare congenital defect first
reported by Church in 1868.1 It is found in only
0.1% of cases of congenital heart diseases.2 Cor
triatriatum sinister results from the persistence of
a fibromuscular membrane in the LA, dividing it
into a proximal posterosuperior chamber receiving pulmonary veins and a distal anteroinferior
chamber containing the LAA and communicating
with the mitral orifice. Embryologically, this condition is thought to arise from the failure of inclusion of the pulmonary veins into the left posterior
atrial wall.
Cor triatriatum dextrum is extremely rare and
results from the complete persistence of the right
sinus valve of the embryonic heart. The membrane divides the right atrium into upper and
lower chambers. The upper chamber receives the
venous blood from both vena cavae and the
lower chamber is in contact with the tricuspid
valve and the right atrial appendage.
The majority of reported cases of cor triatriatum
sinister occur in infants with symptoms of pulmo-

Cor Triatriatum Sinistrum

nary venous obstruction. Although, It is frequently
an isolated finding in adults,3,4 cor triatriatum has
been reported in association with ostium secundum atrial septal defect (ASD), dilated coronary
sinus due to persistent left superior vena cava, and
bicuspid aortic valve in adults.5 Diagnosis in adults
is most commonly the result of dyspnea or arrhythmias and often it mimics mitral stenosis.6 The natural history of cor triatriatum depends on the
effective size of the orifice(s) in the diaphragm and
the presence and location of the ASD. When small,
it usually presents in infancy with reduced cardiac
output, pulmonary venous hypertension, and cardiac failure. In patients with large communications, the diagnosis may present only in adulthood
with development of fibrosis and calcification in
the orifice of the separating membrane, along with
development of mitral regurgitation and atrial
fibrillation.3 The embolic presentation is extremely
rare both in children and adults.
The management of cor triatriatum sinister
depends on the degree of obstruction between
the two LA chambers. Surgical resection of the
accessory membrane is recommended for
patients presenting with obstruction, but no specific treatment is recommended for patients presenting with nonobstructive cor triatriatum.7 In
the setting of an embolism, anticoagulation therapy is a conservative treatment for the prevention of future embolic events.8 A search of
existing literature revealed only 9 previously
reported cases of adult cor triatriatum discovered
in the setting of embolism and no more than 7
published cases of adult cor triatriatum associated with cerebral infarction9 and none in an
adolescent. In 2009, Park, et al.10 compared the
risk factors and treatments in 5 of these cases.
Four of the 5 presented with stroke, with one
patient presenting with a systemic infarction of
multiple sites. Treatment of those patients
included anticoagulation therapy, resection of
the membrane, medical therapy (anticoagulation), and thrombectomy. 10
Conclusion:
In conclusion, this report demonstrates the
exceptionally rare finding of a cor triatriatum sinister in an adolescent presenting as embolic
stroke with hemiparesis, underscoring the impor-

tance of careful evaluation for structural abnormalities of the heart in patients with systemic
embolization. The natural history and complications of cor triatriatum sinister have been poorly
documented, which relates primarily to the small
numbers available for review. Our work adds further to the published literature available on this
rare congenital anomaly.
References
1. Church WS: Congenital malformation of the heart:
Abnormal septum in the left auricle. Trans Pathol Soc Lond
1868;19:188–190.
2. Van Son JAM, Danielson GK, Schaff HV, et al: Cor triatriatum: Diagnosis, operative approach and late results.
Mayo Clin Proc 1993;68:854–859.
3. Chen Q, Guhathakurta S, Vadalapali G, et al: Cor triatriatum in adults: Three new cases and a brief review. Tex
Heart Inst J 1999;26:206–210.
4. Slight RD, Nzewi OC, Mankad PS: Echocardiographic
diagnosis of cor triatriatum sinister in the adult. Heart
2004;90:63.
5. Hamdan R, Mirochnik N, Celermajer D, et al: Cor Triatriatum Sinister diagnosed in adult life with three dimensional
transesophageal
echocardiography.
BMC
Cardiovasc Disord 2010;10:54.
6. Baweja G, Nanda NC, Kirklin JK: Definitive diagnosis of
cor triatriatum with common atrium by three-dimensional transesophageal echocardiography in an adult.
Echocardiography 2004;21:303–306.
7. O’Murchu B, Seward JB: Images in cardiovascular medicine. Adult congenital heart disease. Obstructive and
nonobstructive cor triatriatum. Circulation 1995;92:3574.
8. Rodefeld MD, Brown JW, Heimansohn DA, et al: Cor triatriatum: Clinical presentation and surgical results in 12
patients. Ann Thorac Surg 1990;50:562–568.
9. Patrascu A, Libner A, Terres W: Adult cor triatriatum sinistrum as an unusual cause of embolic stroke Clinical case
portal. 11 Feb 2013.
10. Park KJ, Park IK, Sir JJ, et al: Adult cor triatriatum presenting as cardioembolic stroke. Intern Med 2009;48:1149–
1152.

Supporting Information
Additional Supporting Information may be found
in the online version of this article:
Movie clip S1. Modified TTE 4C view.
Movie clip S2. Modified TTE 4C view with
anterior angulation. Pronounced movement of
fibromuscular membrane is seen. Dense SEC seen
in anterior-inferior chamber.
Movie clip S3. Modified TEE view showing
fibromuscular membrane, SEC in LA and soft clot
in LAA.

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