Case Report

Ischemic Stroke as an Uncommon Complication of Cor
Triatriatum
Konstantinos Spengos, MD, PhD, FESO, Elias Gialafos, MD, PhD,
and Sofia Vassilopoulou, MD, PhD, FESO

Cor triatriatum (CT) is a rare congenital heart disease characterized by a fibromuscular membrane that divides the left atrium into two distinct chambers and mostly manifests during infancy mimicking mitral valve stenosis. We present the uncommon
case of cardioembolic ischemic stroke in an adult man on the grounds of an otherwise
asymptomatic CT with intra-atrial thrombus formation, which was established by
means of transesophageal echocardiography and cardiac magnetic resonance imaging. Because exact diagnosis and surgical treatment is feasible, we suggest CT as
a rare, but potentially treatable source of cerebral embolism in adult patients with
stroke and dilatated left atrium. Key Words: Cor triatriatum—cardioembolic stroke.
Ó 2008 by National Stroke Association

Cor triatriatum (CT) is a rare congenital heart disease
characterized by a fibromuscular membrane that divides
the left atrium into two distinct chambers.1 Whereas most
cases present during infancy, some others remain asymptomatic until late adulthood and their diagnosis might be
missed.2,3 Clinically, both infant and adult patients present mostly with symptoms mimicking mitral stenosis as
a result of the obstructive function of the intra-atrial membrane.4,5 There are only a few reports of cerebral embolism related to CT.6-10 Atrial fibrillation (AF) is a quiet
frequent accompanying feature that makes the identification of the true cause of cerebral embolism rather challenging.4,9,10

From the Department of Neurology, University of Athens School of
Medicine, Eginition Hospital, Athens, Greece.
Received May 9, 2008; revision received June 26, 2008; accepted July
1, 2008.
Address correspondence to Konstantinos Spengos, MD, PhD,
FESO, Department of Neurology, University of Athens School of
Medicine, Eginition Hospital, Vas. Sofias 74-11528 Athens, Greece.
E-mail: Spengos@hol.gr.
1052-3057/$—see front matter
Ó 2008 by National Stroke Association
doi:10.1016/j.jstrokecerebrovasdis.2008.07.007

436

A 55-year-old man presented with severe hemiparesis
and aphasia. He had a history of hypertension, hyperlipidemia, and AF, treated with warfarin. Repeated transthoracic echocardiograms demonstrated a significant
dilatation of the left atrium (55 mm) and a slight concentric
hypertrophy of the left ventricle. Admission international
normalized ratio was 1.9 allowing the initial assumption of
an acute cardioembolic infarction (Fig 1, A) on the grounds
of AF. Transesophageal echocardiography was performed
to exclude intracardial thrombus formation. A hyperechoic membrane dividing the left atrium into two communicating chambers was visualized and the diagnosis of CT
was set. No thrombotic material and normal flow velocities were found in the normal-sized auricula. However,
a 3.5- 3 1.3-cm large thrombus was attached to the basis
of the anterior cusp of the mitral valve and the free wall
of the left atrium. It was identified as an obvious source
of cerebral embolism (Fig 1, B). Within the following weeks
the patient improved significantly. At this point cardiac
magnetic resonance imaging clearly demonstrated CT,
but showed no intra-atrial thrombus (Fig 1, C) allowing
the assumption of clot disruption under effective anticoagulation (international normalized ratio . 2.5). Follow-up
transesophageal echocardiography after cardiosurgical

Journal of Stroke and Cerebrovascular Diseases, Vol. 17, No. 6 (November-December), 2008: pp 436-438

ISCHEMIC STROKE CAUSED BY COR TRIATRIATUM

437

Figure 1. (A) Diffusion-weighted
magnetic resonance imaging (MRI)
shows an acute ischemic lesion in the
territory of the left middle cerebral
artery. (B) Transesophageal echocardiography reveals a membrane (arrowheads) and thrombotic material (small
arrows) in the enlarged left cardial
atrium establishing intra-atrial thrombus formation in a case of CT. (C)
MRI in fast white blood technique demonstrates a membrane (white arrows)
that separates a left atrium into two communicating chambers supporting the
diagnosis of CT.

intervention with total membrane excision revealed no
membrane rests, nor thrombotic material within the left
atrium. However, AF persisted.

Conclusion
This constellation of findings suggests the direct association between stroke and this rare congenital heart disease
as a result of the obstructing effect of the membrane that reduces blood flow between both chambers of the left atrium.
The fact that, in addition to AF and atrial dilatation, our patient presented no signs of pulmonary hypertension or
overloading of the right ventricle and remained cardiologically asymptomatic make the differential diagnostic approach of this case intriguing. Currently, diagnosis of CT

can be easily achieved by means of transesophageal echocardiography or cardiac magnetic resonance imaging and
surgical treatment is feasible. It should, therefore, be considered as a rare, but potentially treatable source of embolism in adult patients with stroke and a dilatated left
atrium.

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