Acta Cardiol Sin 2016;32:758-761
Case Report
doi: 10.6515/ACS20160129A
A Very Rare Case of Co-Existence of Cor Triatriatum
Sinister and Left Pulmonary Vein Atresia
Mustafa Aparci, Murat Yalcin, Zafer Isilak, Mehmet Dogan and Ejder Kardesoglu
Cor triatriatum sinister (CTS) is a rare congenital abnormality. Clinical presentation of patients with CTS mainly
depends on the anatomic features of membrane and may vary from mild or moderate symptoms mimicking mitral
stenosis to more severe and complicated cardioembolic stroke or a new onset heart failure. We herein have
reported on a young male who presented with the signs and symptoms of mitral stenosis and was diagnosed as CTS
with gradient on the orifice of the membrane after transthrocacic echocardiography. Computerized tomographic
angiography revealed that the patient had unilateral, left sided pulmonary arterial hypoplasia and pulmonary vein
atresia, with only the right pulmonary veins draining into the left atrial chamber. Further cardiac imaging by either
computed tomography or magnetic resonance imaging is necessary in order to seek accompanying cardiac and
vascular abnormalities. Patients with CTS have improved short and long term survival rates if CTS and accompanying
abnormalities are surgically treated before the disease is complicated with heart failure, pulmonary hypertension,
stroke and etc.
Key Words:
Cor triatriatum sinister · Mitral stenosis · Pulmonary artery hypoplasia · Pulmonary vein atresia
INTRODUCTION
the pulmonary venous blood to the systemic circulation
through anatomical left atrium and to the mitral valves.
Either CTS or its coexistence with pulmonary vein atresia and also pulmonary artery hypoplasia are very rare
congenital abnormalities. Clinical signs and symptoms of
CTS depend on the obstruction of the hole on the membrane and accompanying congenital abnormalities of
the central and pulmonary vasculatures. Treatment of
CTS is most effectively achieved through surgical excision of this membrane; however, in a limited number of
cases, percutaneous balloon dilatation of the orifice (as
in mitral stenosis) has been reported.2
Cor triatriatum sinister (CTS) is generally recognized
to be a rare congenital abnormality. Clinical presentation of patients with CTS may vary from mild or moderate symptoms mimicking mitral stenosis to more severe
and complicated cardioembolic stroke or a new onset
heart failure.1,2
A membrane separates the left atrium into two
chambers: the proximal or superior chambers with pulmonary venous blood flow, and the distal inferior chamber-true atrium is surrounded by the mitral valve, atrial
appendage, and interatrial septum. An orifice on the
membrane connects those two chambers and transfers
CASE REPORT
A 19-year-old male presented with recent onset dyspnea on exercise. Upon physical examination, the patient’s blood pressure was 110/65 mm Hg, heart rate
was 85 beats/min, and a 1/4 grade diastolic rumble is
auscultated at the cardiac apex. Following transthoracic
Received: November 2, 2015 Accepted: January 29, 2016
Haydarpasa Training Hospital, Department of Cardiology, *Mevki
Military Hospital, Department of Cardiology.
Address correspondence and reprint requests to: Dr. Mehmet Dogan,
Irfan Bastug Street, Altindag, Ankara, Turkey. Tel: +905305928277;
E-mail: [email protected]
Acta Cardiol Sin 2016;32:758-761
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An Interesting Cor Triatriatum Sinister Case
the left atrium. There were only right pulmonary veins
draining to the left atrium, and increased vasculature
and enlargement of right sided pulmonary vessels. Additionally, cardiac shifting to the left pulmonary space
with reduced vasculature was remarkable (Figure 2).
The patient decided to be treated with surgical resection of the membrane lying within the left atrium.
echocardiography examination, apical four chamber
view exactly demonstrated the presence of membrane
separating the left atrium into proximal and distal chambers. Interpretation of the color flow Doppler ascertained the location of the orifice on the membrane,
communicating the chambers and continuous wave with
Doppler echocardiography, and measured a 20 mmHg
maximum peak gradient on the orifice (Figure 1). Since
the orifices of pulmonary veins draining into the left
atrium could not be observed on transthoracic echocardiography, computed tomography (CT) angiography
was planned to evaluate the pulmonary arterial tree and
pulmonary venous return to the left atrium.
CT angiography documented that the left pulmonary artery was hypoplastic, and the left pulmonary
veins were atresic, with no pulmonary venous return to
A
DISCUSSION
Clinical manifestations of patients with CTS vary
from dyspnea, recurrent syncope to stroke, heart failure, pulmonary edema, severe pulmonary arterial hypertension, and atrial fibrillation.3 The signs and symptoms of CTS are mainly associated with the pulmonary
B
C
Figure 1. (A) Chest x-ray shows left-sided cardiac displacement and the engorgement of right sided pulmonary hilus. (B) Transthoracic echocardiography indicates the membrane which divides the left atrium. (C) Continuous wave Doppler image shows 20 mmHg maximum peak gradient
on the orifice. LA, left atrium; PG, peak gradient; RA, right atrium.
A
B
C
Figure 2. CT angiography revealed that the left pulmonary artery was hypoplastic (A), atresic left pulmonary veins with no pulmonary venous return to the left atrium (B) and increased vasculature and enlargement of right sided pulmonary vessels (C). LA, left atrium; LPV, left pulmonary vein;
RPV, right pulmonary vein.
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Acta Cardiol Sin 2016;32:758-761
Mustafa Aparci et al.
congestion due to obstruction of pulmonary venous return
from the proximal atrial chamber to true left atrium and
late onset pulmonary arterial hypertension, which mimic
the clinical spectrum of mitral stenosis. Slight et al. reviewed all of the cases presented in the English-language
medical literature, and concluded that almost all the patients had manifested with the signs and symptoms of mitral stenosis, increased pulmonary capillary wedge pressure and mean pressure gradient, and also an increased
prevalence with aging.4 As in our case, continuous wave
Doppler echocardiography can demonstrate the increased
gradient on either diastolic or systolic phase of flow
through the orifice of the membrane opening to the true
left atrium (Figure 1C). We found that the max and mean
peak gradients were measured on the orifice of membrane
as 20 mmHg and 14 mmHg, respectively.
The patient’s chest x-ray revealed left-sided cardiac
displacement and the engorgement of right sided pulmonary hilus (Figure 1A). Although echocardiography is
the best way to initially diagnose CTS, it cannot definitively demonstrate all the congenital abnormalities accompanying CTS. A high quality image of membrane in
the left atrium on parasternal long axis view should be
evaluated from the apical views and by means of color
flow Doppler echocardiography. Orifices of pulmonary
veins should be sought in the proximal and distal-true
left atrium. Nevertheless, further cardiac imaging techniques should be performed in order to identify and describe the exact anatomy. Furthermore, 2- or 3-dimensional transesophagial echocardiography may also reveal the anatomy and structure of the membrane and
pulmonary venous return. CT angiography or magnetic
resonance imaging can detect all the components of
cardiac abnormalities related to the left atrium, pulmonary venous return and pulmonary arterial tree, and
also pulmonary vasculature. We observed that only the
right pulmonary veins return to the left atrium, and left
pulmonary veins were absent. Since the left pulmonary
artery was hypoplastic, drainage of left-sided pulmonary
venous return had likely been achieved by mediastinal
or bronchial venous collaterals.
Cor triatriatum sinistra may be observed alone or in
association with other anomalies. In adults, the most
common accompanying anomalies are mitral regurgitation, secundum-type atrial septal defect, persistent left
superior vena cava, and anomalies of PV return.5,6 Less
Acta Cardiol Sin 2016;32:758-761
frequent concomitant anomalies are reported to be
hypoplastic left heart syndrome, patent ductus arteriosus, cor triatriatum dexter, aortic regurgitation, bicuspid aortic valve and tetralogy of Fallot.7,8 Several theories
have been suggested to better understand the formation
mechanism of CTS.8 Although there is no consensus on
such proposed mechanisms, the most accepted pathophysiologic mechanism is failure of resorption of the
common PV in the intrauterin life.9 Proposed mechanisms may provide a partial explanation for pulmonary
vein anomalies, but as in our case the pathophysiological
mechanisms that explain the relationship between CTS
and pulmonary artery atresia are not yet available.
This case is rare due to the co-existence of CTS with
gradient on its orifice, and the fact that it was accompanied by left-sided pulmonary arterial hypoplasia and
pulmonary vein atresia and only with right sided pulmonary veins draining into the left atrium. Accurate diagnosis and detection of all abnormalities accompanying
CTS is clinically significant in the management and follow up of patients with CTS. Additionally, CTS and coexisting congenital abnormalities could be corrected
and treated by surgery, with high short-term and longterm survival rates reaching 96% and 88%, respectively.10
CONCLUSIONS
Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the
anatomic features membrane and the components of
the accompanying cardiac abnormalities. Further cardiac imaging by computerized tomography or magnetic
resonance imaging is necessitated to identify the coexisting cardiac and vascular abnormalities and to more
efficaciously guide the surgical treatment.
CONFLICT OF INTEREST
The authors declared no conflicts of interest.
REFERENCES
1. Minocha A, Gera S, Chandra N, et al. Cor Triatriatum sinistrum
760
An Interesting Cor Triatriatum Sinister Case
2.
3.
4.
5.
presenting as cardioembolic stroke: an unusual cause of adolescent hemiparesis. Echocardiography 2014;31:E120-3.
Mendez AB, Colchero T, Garcia-Picart J, et al. Unusual case of
new-onset heart failure due to cor triatriatum sinister. Eur J
Heart Fail 2013;15:237-9.
D'Aloia A, Vizzardi E, Caretta G, et al. Diagnosis of cor triatriatum
sinister in patient with pulmonary edema and severe pulmonary
arterial hypertension: assessment by three-dimensional transesophageal echocardiography. Echocardiography 2011;28:E198201.
Slight RD, Nzewi OC, Buell R, Mankad PS. Cor-triatriatum sinister
presenting in the adult as mitral stenosis: an analysis of factors
which may be relevant in late presentation. Heart Lung and Circulation 2005;14:8-12.
Eichholz JL, Hodroge SS, Crook JJ 2nd, et al. Cor triatriatum sinister in a 43-year-old man with syncope. Tex Heart Inst J 2013;
40:602-5.
6. McMaster KS, Tandon A, Schussler JM. Renal infarction secondary to cor triatriatum sinister. Am J Cardiovasc Dis 2012;2:331-3.
7. Khan MA, Almoukirish AS, Das K, Galal MO. Hypoplastic left heart
syndrome, cor triatriatum and partial anomalous pulmonary
venous connection: imaging of a very rare association. J Saudi
Heart Assoc 2012;24:137-40.
8. Zepeda IA, Morcos P, Castellanos LR. Cor triatriatum sinister
identified after new onset atrial fibrillation in an elderly man.
Case Rep Med 2014;2014:674018.
9. Ilhan E, Ergelen M, Soylu O, et al. Severe right heart failure and
pulmonary hypertension because of cor triatriatum sinister in a
54 year-old patient. Int J Cardiol 2011;151:e29-31.
10. Alphonso N, Nørgaard MA, Newcomb A, et al. Cor triatriatum:
presentation, diagnosis and long-term surgical results. Ann
Thorac Surg 2005;80:1666-71.
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