Cor Triatriatum: Clinical Presentation of 18 Cases

Ryszard W Lupinski, Sriram Shankar, Keng Yean Wong, Yoke Hwee Chan, Susan M
Vosloo and Jacek J Moll
Asian Cardiovasc Thorac Ann 2001;9:106-110

This information is current as of January 23, 2012

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
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The Asian Cardiovascular & Thoracic Annals is the official journal of The Asian Society for
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Lupinski
ORIGINAL CONTRIBUTION

COR TRIATRIATUM: CLINICAL PRESENTATION OF 18 CASES

COR TRIATRIATUM: CLINICAL

PRESENTATION OF 18 CASES
Ryszard W Lupinski, MD, PhD,
Sriram Shankar, MBBS, Wong Keng Yean, MBBS1,
Chan Yoke Hwee, MBBS2,
3
Susan M Vosloo, MBChB , Jacek J Moll, MD, PhD4
Department of Pediatric Cardiothoracic Surgery
1Department of Pediatric Cardiology Service
2Department of Pediatric Medicine
Kandang Kerbau Womens and Childrens Hospital
Singapore, Republic of Singapore
3Department of Cardiothoracic Surgery
Red Cross Memorial Childrens Hospital
Cape Town, South Africa
4Department of Pediatric Cardiovascular Surgery
WOK Hospital
Zabrze, Poland

ABSTRACT
From 1985 to 1999, 18 patients with cor triatriatum underwent surgical correction.
Their ages ranged from 3 months to 9 years (mean, 32.5 months). Eight patients
were cyanosed; the other 10 presented with congestive heart failure. Recurrent
chest infection was observed in 10 cases, and failure to thrive in 7. One patient
had isolated cor triatriatum, and 17 had an associated atrial septal defect. Other
associated anomalies included patent ductus arteriosus, ventricular septal defect,
and partial and total anomalous pulmonary venous connection. Two deaths occurred
perioperatively in patients with associated severe heart defects. Follow-up ranged
from 1 month to 10 years. No late events occurred among the survivors, and all
were in New York Heart Association functional class I. Their most recent
echocardiograms showed no residual obstruction or shunt and good development
of the left cardiac chambers. Echocardiography is recommended as the diagnostic
modality of choice. Cor triatriatum can be corrected surgically with low mortality
and good long-term results.
(Asian Cardiovasc Thorac Ann 2001;9:10610)

INTRODUCTION
Cor triatriatum is a rare cardiac malformation accounting
for only 0.1% of all cardiac defects. Clinical presentation
depends on the degree of restriction of blood flow from
the upper chamber to the lower chamber of the left atrium
through a fibromuscular membrane containing one or
more fenestrations.1,2 The aim of this study was to describe
the clinical presentation of cor triatriatum sinistrum,

surgical and anatomical findings, and outcome after

surgical correction.

PATIENTS AND METHODS

Of 18 patients with cor triatriatum, 8 were treated in
South Africa, 9 in Poland, and 1 in Singapore, between
1985 and 1999. Their case notes were reviewed retrospectively. Demographic features, clinical presentation, results

For reprint information contact:

Ryszard W Lupinski, MD, PhD Tel: 65 394 1132 Fax: 65 291 0161 email: richardl@kkh.com.sg
Department of Pediatric Cardiothoracic Surgery, Kandang Kerbau Womens and Childrens Hospital, 100 Bukit Timah Road,
Singapore 229899, Republic of Singapore.
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COR TRIATRIATUM: CLINICAL PRESENTATION OF 18 CASES

of investigations (chest radiographs, electrocardiograms),

and surgical or autopsy findings were studied. Echocardiography and cardiac catheterization were performed
to assess the heart defect. The demographic variables are
listed in Table 1. There were 5 females and 13 males;
their mean age was 32.5 months (range, 3 months to 9
years), and 6 patients were younger than one year. Ten

patients had a history of recurrent chest infection and 7

presented with failure to thrive. The clinical findings are
summarized in Table 2. Eight children were cyanosed and
10 presented with congestive heart failure. Right
ventricular hypertrophy on an electrocardiogram was
observed in all cases. On chest radiography, cardiomegaly
was observed in 17 patients and plethora in 10.

Table 1. Demographics and Surgery of Patients With Cor Triatriatum

Patient No.
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18

Sex

Age

Male
Male
Male
Male
Female
Female
Male
Male
Male
Male
Male
Female
Male
Female
Male
Male
Female
Male

4
7
9
5
15
4
5
18
8
14
3
4
7
30
4
3
3
5

Type of Cor Triatrium

Surgical Approach

I
II
II
III
IV
IV
II
II
IV
II
IV
IV
II
II
IV
II
III
III

RA, LA
RA
RA
RA
RA
RA
RA
RA
RA
RA
RA
RA
RA
RA
RA, LA
RA, LA
RA
RA

years
months
years
months
months
years
months
months
years
months
months
years
months
months
years
years
months
years

Associated Lesion
None
ASD,
ASD,
ASD,
ASD
ASD,
ASD,
ASD,
ASD
ASD
ASD,
ASD,
ASD,
ASD
ASD,
ASD
ASD,
ASD,

PDA
PAPVD
PDA
PAPVD, PS
DORV, VSD, CoA
TAPVD

PAPVD
PS
VSD, PS
TAPVD
TAPVD, PDA
PAPVD

ASD = atrial septal defect, CoA = coarctation of the aorta, DORV = double-outlet right ventricle, LA = left atrium, PAPVD = partial pulmonary
venous drainage, PDA = patent ductus arteriosus, PS = pulmonary stenosis, RA = right atrium, TAPVD = total pulmonary venous drainage,
VSD = ventricular septal defect.

Table 2. Clinical Presentation of Patients With Cor Triatriatum

Patient No.
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18

Congestive
Heart Failure

Yes
Yes
Yes

Cyanosis

Yes
Yes

Yes
Yes

Yes

Yes
Yes
Yes
Yes
Yes

Yes
Yes

Yes
Yes

Yes
Yes
Yes
Yes

Failure to Thrive

Yes
Yes
Yes
Yes

Yes
Yes
Yes

Recurrent
Chest Infection

Yes
Yes

Yes
Yes
Yes
Yes

Yes

Chest Radiograph
CM, plethora
CM, plethora
CM
CM, plethora
CM
CM
CM
CM, plethora
CM
CM
CM, plethora
CM
CM, plethora
CM, plethora
CM, plethora
Plethora
CM, plethora
CM

Electrocardiogram
RVH,
RVH,
RVH
RVH,
RVH
RVH
RVH
RVH,
RVH
RVH
RVH
RVH,
RVH
RVH
RVH
RVH
RVH,
RVH

IRBBB
RAD
RAD

RAD

RAD

RAD

CM = cardiomegaly, IRBBB = intermittent right bundle branch block, RAD = right atrial dilatation, RVH = right ventricular hypertrophy.

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COR TRIATRIATUM: CLINICAL PRESENTATION OF 18 CASES

Excision of the cor triatriatum membrane was accomplished through a right atrial approach (Figure 1),
with the aid of cardiopulmonary bypass in all patients
except one in whom an additional left atrial incision was
made to clarify anatomy. One patient had cor triatriatum
alone. In 17 patients, there was an associated atrial septal
defect (ASD). Other associated defects are listed in
Table 1.

RESULTS
All patients required inotropic support in the perioperative
period. Two deaths occurred postoperatively due to low

cardiac output in patients with associated complex heart

defects. Other complications included reoperation for left
atrial-to-inferior vena cava fistula, and left hemiparesis
due to unrelated pathology. Follow-up ranged from 1
month to 10 years. No late events occurred among the
survivors, and all were in New York Heart Association
functional class I. Their most recent echocardiograms
showed no residual obstruction or shunt and good
development of the left cardiac chambers.
Depending on the presence and localization of an ASD,
there are 4 types of cor triatriatum (Figure 2). Type I

Figure 1. Surgery for cor triatriatum. (A) Surgical forceps inside the obstructing membrane. (B) Left atrium after the obstructing membrane has
been excised (arrow).

Type I

Type III

Type II

Type IV

Figure 2. Anatomical types of cor triatriatum. LV = left ventricle, RA = right atrium, RV = right ventricle.

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COR TRIATRIATUM: CLINICAL PRESENTATION OF 18 CASES

(intact interatrial septum) was found in 1 patient, type II

(ASD to upper left atrial chamber) was observed in 8,
type III (ASD to lower left atrial chamber) was noted in
3, and 6 had type IV (ASD to both chambers).

DISCUSSION
In cor triatriatum, the remnants of the common pulmonary
vein (the upper left atrial chamber) and primitive left
atrium (the lower left atrial chamber) are separated by a
fibromuscular diaphragm containing one or more
fenestrations that allow some passage of blood from the
upper to the lower chamber. The left atrial appendage
usually arises from the lower chamber.1 Patients with this
anomaly are usually symptomatic from birth.1,2 A few
reports have described adults diagnosed with cor
triatriatum.3,4 Clinical manifestations depend on the size
of the opening and the degree of obstruction of venous
return. When this is severe, signs and symptoms occur.1,2
Congestive heart failure, recurrent chest infections, failure
to thrive, and cyanosis are typical.1,2 Pulmonary hypertension is usually present.1,5 Electrocardiographic features
include right atrial dilation and right ventricular hypertrophy. Radiographically, the heart is moderately enlarged
and the lungs may reveal some degree of congestion with
fluid.1,2 The diagnosis is usually confirmed by echocardiography.3,610 M-mode and 2-dimensional echocardiography demonstrate a membrane in the mid left atrium.
Doppler echocardiography is useful to assess the degree
of flow obstruction within the left atrium. 8,1113
Cineangiography usually delineates clearly the membrane
and associated lesions.
An ASD or foramen ovale connects the right atrium to the
lower chamber in approximately 80% of cases. Often,
additional malformations may be associated.2 Absent
communication between the left and right atria has seldom
been reported, but it was found in one of the patients in
this study.11,12 Associated anomalies observed previously
include patent ductus arteriosus, ventricular septal defect,
total and partial anomalous pulmonary venous connection,
coarctation of the aorta, pulmonary stenosis, atrioventricular septal defect, and double-outlet right ventricle.6,1416
Rare associations of cor triatriatum with tetralogy of
Fallot, Ebsteins anomaly, and transposition of the great
arteries have also been reported.1719
Up to 50% of affected patients may die within the first
2 years of life, but successful surgical correction can be
carried out.2,20 Excision of the cor triatriatum membrane
is usually accomplished via a right atrial approach, but an
additional left atrial incision is needed sometimes to clarify
the anatomy.11,12 This happened in one of the cases in this
study. All associated anomalies should be corrected
simultaneously. Death occurs mainly from low cardiac
output in patients with associated complex anomalies.4
Late postoperative echocardiography in survivors usually
reveals no flow obstruction within the left atrium and
good development of the left cardiac chambers.
2001, V OL . 9, N O. 2

It was concluded from this study that echocardiography

is the diagnostic modality of choice for cor triatriatum,
and this rare defect can be corrected surgically with low
mortality and good long-term results. Cor triatriatum
seldom appears as a solitary heart defect. Of the various
associated lesions, the most common is an atrial septal
defect.
Presented at the 8th Annual Meeting of The Asian Society
for Cardiovascular Surgery, Fukuoka, Japan, September
68, 2000.

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Cor Triatriatum: Clinical Presentation of 18 Cases

Ryszard W Lupinski, Sriram Shankar, Keng Yean Wong, Yoke Hwee Chan, Susan M
Vosloo and Jacek J Moll
Asian Cardiovasc Thorac Ann 2001;9:106-110
This information is current as of January 23, 2012
Updated Information
& Services

including high-resolution figures, can be found at:

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References

This article cites 17 articles, 5 of which you can access for free at:
http://asianannals.ctsnetjournals.org/cgi/content/full/9/2/106#BIBL

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