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 Table of Contents  
INTERESTING CASE REPORT
Year : 2020  |  Volume : 4  |  Issue : 1  |  Page : 45-48

Cor Triatriatum Sinister


Heartline Cardiac Care Centre, Allahabad, Uttar Pradesh, India

Date of Submission30-Apr-2019
Date of Decision01-Jun-2019
Date of Acceptance16-Jun-2019
Date of Web Publication11-Apr-2020

Correspondence Address:
Dr. Ishita Banerji
Heartline Cardiac Care Centre, 14/18 Elgin Road, Civil Lines, Allahabad - 211 001, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_20_19

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  Abstract 

A 10-year-old boy presented with complaints of poor weight gain, progressively increasing dyspnea since early childhood, and orthopnea. Echocardiogram was suggestive of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dextrum) is divided into two compartments by an abnormal fibromuscular septum usually with an opening between the two chambers associated with varying degrees of obstruction of the pulmonary veins. The pulmonary venous return is followed by pulmonary arterial hypertension. Cor triatriatum literally means a heart with three atria (triatrial heart), namely, the superior chamber, the inferior chamber or the true atrium, and the atrial appendage. This anomaly occurs in 0.4% of patients with congenital heart defects, with a slight male predominance. The present case of cor triatriatum sinister is being reported in view of the fact that the child remained undiagnosed till the age of 10 years, despite being “sick since a little boy” requiring multiple hospital admissions for chest infections. He had been receiving treatment for “chest problem” and had been on bronchodilators. The classical echocardiographic findings would be of academic interest as well.

Keywords: Cor triatriatum, echocardiography, pulmonary venous obstruction


How to cite this article:
Banerji I. Cor Triatriatum Sinister. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:45-8

How to cite this URL:
Banerji I. Cor Triatriatum Sinister. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2020 Sep 29];4:45-8. Available from: http://www.jiaecho.org/text.asp?2020/4/1/45/282197




  Introduction Top


Cor triatriatum is a rare congenital abnormality. Although it was first described by Church in 1868, as a left atrium divided by an abnormal septum, the name “cor triatriatum” was given by Borst in 1905.[1] Cor triatriatum is a treatable cardiac malformation whose diagnosis can easily be missed on “routine” echocardiographic studies. The anomaly may be associated with other congenital cardiac lesions in up to 80% of cases in pediatric population, most frequently with ostium secundum atrial septal defect and anomalous pulmonary vein return.[2] The atrium is divided into two distinct chambers, usually by a thick fibromuscular septum. The proximal or superior chamber drains the pulmonary venous blood, whereas the distal inferior chamber (or true atrium) is in contact with the atrioventricular valve and contains the atrial appendage and the true atrial septum.[3]

The simplest classification was given by Loeffler in 1949,[4] based on the number and size of fenestrations in the fibromuscular membrane and classified cor triatriatum into three groups as follows: Group 1: there is absence of connection between the two chambers, the accessory chamber might connect with the right atrium, or some of the pulmonary veins might drain in an anomalous fashion; Group 2: there are one or few small openings in the intra-atrial membrane; and Group 3: the accessory chamber communicates widely with the true atrium by a large single opening.

While Group 3 is mostly found in the adult population, the first two groups are usually diagnosed in highly symptomatic infants and children and are associated with increased mortality at a younger age.

The clinical presentations of cor triatriatum are directly related to the size of the orifice between the accessory and the true atrial chamber. When the foramen is small, the obstruction is sufficient to create a pressure gradient within the atria, mimicking mitral stenosis and presenting with dyspnea, orthopnea, and hemoptysis. Adults having the disease are usually asymptomatic, due to the presence of a large foramen with no intra-atrial pressure gradient, often revealed by atrial arrhythmias and cerebral and systemic embolic events, due to thrombus in an enlarged accessory atrial chamber. In infants and newborns, the manifestations of the disease are secondary to a relatively narrow opening with a subsequent rise in proximal left atrial pressure and pulmonary congestion.[5]


  Clinical Presentation Top


A 10-year-old malnourished boy, born to nonconsanguineous parents, presented with progressively increasing dyspnea and poor weight gain with a history of recurrent chest infection requiring multiple hospitalizations since early childhood. He was referred for cardiac evaluation, on failure to respond to bronchodilators. On auscultation, a loud second heart sound was heard. No distinctly audible murmur was appreciable in the “noisy chest.” Auscultation of the chest revealed bilateral crepitations and rhonchi, suggestive of pulmonary congestion.

On echocardiographic study, an abnormal, dense linear echo was seen consistently in every view, traversing the left atrial cavity obliquely, attaching to the posterior aortic wall on one end and lateral wall of the left atrium on the other, suggestive of cor triatriatum [Figure 1], [Figure 2], [Figure 3], [Figure 4]. The upper pulmonary veins were seen draining into the superior chamber, whereas the lower pulmonary veins drained into the lower chamber or the true left atrium, with no communication seen between the two chambers [Figure 5]. However, a connection was seen between the superior chamber and the right atrium through a restricted orifice. A turbulent flow was seen across the restricted orifice [Figure 6] and [Figure 7], with maximal transmembrane pressure gradient measuring 11 mmHg [Figure 8]. Thus, pulmonary venous pressure of the upper pulmonary veins was high, behaving like a partial anomalous pulmonary venous drainage with pulmonary venous obstruction, resulting in raised pulmonary venous pressure leading to pulmonary arterial hypertension as evaluated by the tricuspid regurgitation (TR) gradient on apical four-chamber view [Figure 9]. Besides, persistent left superior vena cava (PLSVC) was seen with a dilated coronary sinus. The mitral annulus was found to be good sized [Figure 10] at 19 mm, whereas the tricuspid annulus measured 14 mm.
Figure 1: Apical four-chamber view

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Figure 2: Apical four-chamber color flow

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Figure 3: Parasternal long-axis view

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Figure 4: Foreshortened biatrial view

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Figure 5: Upper pulmonary venous confluence draining into the superior chamber

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Figure 6: Size of the restricted orifice

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Figure 7: Turbulent flow across the restricted orifice

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Figure 8: Maximal Doppler gradient at the restricted orifice

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Figure 9: Tricuspid regurgitation gradient

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Figure 10: Good sized mitral annulus

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  Discussion Top


Echocardiographic evaluation of cor triatriatum is a noninvasive tool that can accurately delineate the morphology of the extramembrane and the associated cardiac lesions.[6] The measurements of the mean and maximal transmembrane pressure gradients and maximal velocity, in addition to the measurement of the pulmonary arterial (PA) pressure, are evaluated by spectral Doppler. It has been reported that severe obstruction is indicated by maximum Doppler velocity >2 m/s.[7] The presence of associated cardiac lesions such as atrial septal defect, anomalous venous return and less frequently bicuspid aortic valve, and dilated sinus venosus[8] could be diagnosed, offering a comprehensive anatomic and functional evaluation of these infrequent entities that guides further surgical treatment options.[9],[10]

The case being reported here falls in the Group 1 of Loeffler's classification. The upper pulmonary veins were seen draining into the superior chamber through a common confluence, with the only exit from this chamber seen through the restricted orifice into the right atrium with a maximal pressure gradient of 11 mmHg suggestive of pulmonary venous hypertension leading to pulmonary arterial hypertension. On interrogating the mild TR in apical four-chamber view, it was found to be 74 mmHg which translates to a mean PA pressure of 52 mmHg, suggestive of severe pulmonary arterial hypertension. Physiologically, when blood from the accessory atrial chamber, receiving the pulmonary veins, drains into the right atrium, the hemodynamic features are akin to that of total anomalous pulmonary venous drainage. In this case, it is that of partial anomalous pulmonary venous connection, with obstruction leading to pulmonary arterial hypertension.

The lower pulmonary veins were found to drain into the true atrium with a turbulent flow, yet again suggestive of pulmonary venous hypertension. In this case, a PLSVC was also seen draining into the dilated coronary sinus behind the left atrium. Surgical excision of the intra-atrial membrane could release the pressure of the upper pulmonary veins, which could bring down the PA pressures and give significant symptomatic relief to the child.


  Conclusion Top


Cor triatriatum is undoubtedly a rare congenital cardiac defect, yet to remain undiagnosed for 10 years despite being symptomatic is surprising. A child with recurrent chest infection, and not showing significant improvement to treatment suspecting lung pathology, must have the cardiac status evaluated thoroughly, particularly an echocardiogram becomes advisable. Usually, a transthoracic echocardiography (TTE) with color Doppler is enough to diagnose the entity; however, when limitations in image quality of TTE prevent good morphological evaluation, transesophageal echocardiography may play a crucial role in confirming the diagnosis. Computed tomography scanning and magnetic resonance imaging can also effectively establish the diagnosis, easily showing the atrial accessory membrane, though hemodynamics is best understood through an echocardiogram. This case is being reported in order to further emphasize the role of echocardiography in the modern-day management of a sick child.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Borst H. Ein cor triatriatum. Zentralbl Allg Pathol 1905;16:812-5.  Back to cited text no. 1
    
2.
Humpl T, Reineker K, Manlhiot C, Dipchand AI, Coles JG, McCrindle BW. Cor triatriatum sinistrum in childhood. A single institution's experience. Can J Cardiol 2010;26:371-6.  Back to cited text no. 2
    
3.
Niwayama G. Cor triatriatum. Am Heart J 1960;59:291-317.  Back to cited text no. 3
    
4.
Loeffler E. Unusual malformation of the left atrium; pulmonary sinus. Arch Pathol (Chic) 1949;48:371-6.  Back to cited text no. 4
    
5.
Nassar PN, Hamdan RH. Cor triatriatum sinistrum: Classification and imaging modalities. Eur J Cardiovasc Med 2011;1:84-7.  Back to cited text no. 5
    
6.
Slight RD, Nzewi OC, Mankad PS. Echocardiographic diagnosis of cor triatriatum sinister in the adult. Heart 2004;90:63.  Back to cited text no. 6
    
7.
Houston A, Hillis S, Lilley S, Richens T, Swan L. Echocardiography in adult congenital heart disease. Heart 1998;80 Suppl 1:S12-26.  Back to cited text no. 7
    
8.
Hamdan R, Mirochnik N, Celermajer D, Nassar P, Iserin L. Cor triatriatum sinister diagnosed in adult life with three dimensional transesophageal echocardiography. BMC Cardiovasc Disord 2010;10:54.  Back to cited text no. 8
    
9.
Pericas P, Rodríguez-Fernández A, Melis G, Forteza JF, Figuerola AA, Bethencourt A. Real-time three-dimensional transesophageal echocardiographic imaging of cor triatriatum and persistent left superior vena cava. J Am Soc Echocardiogr 2011;24:706.e1-3.  Back to cited text no. 9
    
10.
Willens HJ, Ferrer PL, Tamer DF, Labrador E, Agatston AS, Keith K, et al. Cor triatriatum sinister in an adult: Management guided by real time three-dimensional transesophageal echocardiography and stress echocardiography. Echocardiography 2010;27:E132-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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