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Year : 2018  |  Volume : 28  |  Issue : 2  |  Page : 143-145

Cor-Triatriatum dexter with associated cyanosis in a 3-month-old girl

1 Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria
2 Department of Nursing, Aminu Kano Teaching Hospital, Kano, Nigeria

Date of Web Publication16-May-2018

Correspondence Address:
Ibrahim Aliyu
Department of Paediatrics, Aminu Kano Teaching Hospital, Kano
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcecho.jcecho_11_17

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Cor-triatriatum is uncommon and cor-triatriatum dexter is rarer, and patients may remain asymptomatic in isolated cases especially if the partitioning is mild and nonobstructing. There may be other cardiac defects associated with it which are mostly right-sided cardiac defects such as atrial septal defect, Ebstein anomaly, and pulmonary stenosis. However, left-sided heart anomaly such as aortic regurgitation has been associated with it, but its association with transposition of the great vessels has not been documented before now. Therefore, the case of a 3-month-old girl with cor-triatriatum dexter with transposition of the great vessels, and atrial septal defect is reported.

Keywords: Atrial septal defect, cor-triatriatum, cor-triatriatum dexter, cyanosis, transposition of the great vessels

How to cite this article:
Aliyu I, Ibrahim ZF. Cor-Triatriatum dexter with associated cyanosis in a 3-month-old girl. J Cardiovasc Echography 2018;28:143-5

How to cite this URL:
Aliyu I, Ibrahim ZF. Cor-Triatriatum dexter with associated cyanosis in a 3-month-old girl. J Cardiovasc Echography [serial online] 2018 [cited 2021 Oct 26];28:143-5. Available from: https://www.jcecho.org/text.asp?2018/28/2/143/232555

  Introduction Top

Cor-triatriatum is an uncommon congenital heart disease in which the atria are three chambered (tri-atrial chambers). It was first reported in 1868.[1] It constitutes 0.1%–0.4% of congenital heart diseases.[2],[3] Cor-triatriatum sinister involves the left atrium and is more common while cor-triatriatum dexter is rare accounting for 0.025% of congenital heart disease.[4] The clinical presentation of cor-triatriatum dexter depends on the severity of obstruction to flow; therefore, in nonobstructive cases, it remains asymptomatic, presenting as incidental findings even in adulthood.[5] In severe cases, they may present in right-sided heart failure, with elevated central venous pressure.[6] Fifty percent of cases of Cor-triatriatum dexter may be associated with other cardiac anomalies of the right heart [7] such as pulmonary stenosis, tetralogy of Fallot, and Ebstein anomaly.[8] Report of aortic regurgitation which is a left heart structure has also been documented with cor-triatriatum dexter.[9] However, transposition of the great vessel with cor-triatriatum is a rarity. Therefore, the case of a 3-month-old girl is reported.

  Case Report Top

A 3-month-old girl presented with difficulty with breathing and central cyanosis shortly after birth; this was associated with poor weight gain. The pregnancy and delivery were not adversely eventful. There was no family history of similar complaint. On examination, she was tachypneic and tachycardic with displaced apex beat (5th left intercostal space lateral to the midclavicular line) and she had the 1st and 2nd heart sounds with systolic murmur. The oxygen saturation was 85%–87% in room air. The chest X-ray showed cardiomegaly with increased pulmonary vascularity, the packed cell volume was 48%, electrocardiography showed biventricular hypertrophy [Figure 1], while transthoracic echocardiography showed a thickened partition in the right atrium arising from the region of the Eustachian valve toward the atrial septum viewed from the subcostal view [Figure 2] dividing it into two chambers; the pulmonary artery was also seen arising from the left ventricle and also visualized from the left parasternal long/short axis view while the aorta arising from the right ventricle [Figure 3]; there were atrial and ventricular septal defects measuring 5 and 6 mm, respectively. Therefore, this is a case of cor-triatriatum dexter with transposition of the great vessels. However, the patient died while awaiting surgery.
Figure 1: Electrocardiography showing biventricular hypertrophy with right atrial enlargement

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Figure 2: Echocardiogram showing right atrial partition in the left image dividing the right atrium into two: upper and lower right atrium

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Figure 3: Echocardiogram view showing transposition of the great vessels

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  Discussion Top

In embryonic life, the right atrium is from two distinct structures, namely, the sinus venosus and the “true” right atrium; the smooth-walled component is from the incorporation of the right horn of the sinus venosus [10] while the trabeculated portion is from the “true” right atrium. The right valve of the right horn serves in directing oxygenated blood from the inferior vena cava through the foramen ovale to the left atrium; this valve (Chiari network) regresses over time forming the crista terminalis superiorly and the Eustachian and Thebesian valves of the inferior vena cava and coronary sinus, respectively, inferiorly. However, the persistence of Chiari network results in partitioning of the right atrium into two chambers (cor-triatriatum dexter).[10]

Cor-triatriatum dexter is reported as rare; in isolated unobstructed cases, they may remain asymptomatic, and if such cases are free of any illness certainly, they will be missed; therefore, a community-based study is warranted to truly ascertain the rarity of this disorder; otherwise, the assertion in our opinion should be “a rare cause of symptomatic congenital heart disease.”

Cyanosis is a rare association with cor-triatriatum dexter because most related congenital heart defects are right sided and mild; however, it maybe witnessed in patients with Ebstein anomaly and severe pulmonary stenosis/atresia, but an associated left-sided heart defect is rare. Furthermore, its association with transposition of the great vessels to best of our knowledge has not been reported.

  Conclusion Top

Cor-triatriatum dexter is rare; it is often asymptomatic in isolated nonobstructed cases. However, other cardiac congenital anomalies maybe associated with it, which are not restricted to right-side heart lesions as exemplified in the index case. Therefore, active search for cor-triatriatum dexter should be encouraged during echocardiography because this may change the epidemiology of this disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Church WS. Congenital malformation of heart: Abnormal septum in left auricle. Trans Path Soz 1868;19:188-90.  Back to cited text no. 1
Thakrar A, Shapiro MD, Jassal DS, Neilan TG, King ME, Abbara S, et al. Cor triatriatum: The utility of cardiovascular imaging. Can J Cardiol 2007;23:143-5.  Back to cited text no. 2
Niwayama G. Cor triatriatum. Am Heart J 1960;59:291-317.  Back to cited text no. 3
Sánchez-Brotons JA, López-Pardo FJ, Rodríguez-Puras MJ, López-Haldón JE. Cor triatriatum dexter in adults. Rev Esp Cardiol 2010;63:998-9.  Back to cited text no. 4
Shirani J. Cor-triatriatum Clinical Presentation. Available from: http://www.emedicine.medscape.com. [Last accessed on 2015 May 15].  Back to cited text no. 5
Hoye DJ, Wilson EC, Fyfe DA, Guzzetta NA. Cor triatriatum dexter: A rare cause of neonatal cyanosis. Anesth Analg 2010;110:716-8.  Back to cited text no. 6
Mazzucco A, Bortolotti U, Gallucci V, Del Torso S, Pellegrino P. Successful repair of symptomatic cor triatriatum dexter in infancy. J Thorac Cardiovasc Surg 1983;85:140-3.  Back to cited text no. 7
Eroglu ST, Yildirir A, Simsek V, Bozbas H, Bilgi M, Ozin B, et al. Cor triatriatum dexter, atrial septal defect, and Ebstein's anomaly in an adult given a diagnosis by transthoracic and transesophageal echocardiography: A case report. J Am Soc Echocardiogr 2004;17:780-2.  Back to cited text no. 8
Arora R, Gupta SK, Kaul UA, Sharma SR. Cortriatriatum dexter and associated aortic regurgitation. Indian Heart J 1983;35:358-60.  Back to cited text no. 9
Savas V, Samyn J, Schreiber TL, Hauser A, O'Neill WW. Cor triatriatum dexter: Recognition and percutaneous transluminal correction. Cathet Cardiovasc Diagn 1991;23:183-6.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

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