Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page
Users Online:220


 
 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 1  |  Page : 19-21

Cor triloculares biatritum with tricuspid atresia in an african child


Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano, Nigeria

Date of Web Publication3-Mar-2014

Correspondence Address:
Aliyu Ibrahim
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano
Nigeria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-449x.127976

Rights and Permissions
  Abstract 

Cor triloculares biatritum is a rare congenital heart disease characterized by a three-chambered heart consisting of two atria and a single ventricle; however the case of a 5-month-old African boy who in addition to this defect had an associated tricuspid atresia and an atrial septal defect is reported.

Keywords: Atrial septal defect, cor triloculares biatritum, three chambered heart, tricuspid atresia


How to cite this article:
Ibrahim A. Cor triloculares biatritum with tricuspid atresia in an african child. Heart India 2014;2:19-21

How to cite this URL:
Ibrahim A. Cor triloculares biatritum with tricuspid atresia in an african child. Heart India [serial online] 2014 [cited 2019 Dec 11];2:19-21. Available from: http://www.heartindia.net/text.asp?2014/2/1/19/127976


  Introduction Top


The heart is a derivative of the embryonic mesoderm and it passes through stages of development from a single tube to the final stage of a four chambered heart; this is a complex process with genetic and environmental influences; therefore it may be error prone resulting in myriad of congenital malformations. [1] A triloculare heart is a three-chambered heart consisting of either two atria and a single ventricle (cor triloculare biatriatum) or single atrium and two ventricles (cor triloculares biventricularis) - the former is a replica of a reptilian heart. [2]

Cor-triloculares biatritum is a rare congenital heart defect and few cases have been reported so far [3],[4] it constituted about 13 out of the 1000 cases of congenital heart defects reported by Abbott [5] and 7 out of 141 cases of congenital cardiac defects reported by Clawson [6] however no known case has been reported among Africans to the best of the author's knowledge, though Akintunde et al. [7] had reported a case of cor triloculares biventricularis with pulmonary stenosis in a Nigerian woman in pregnancy.


  Case Report Top


The present case report is about a 5-month-old male child who had complaint of difficulty with breathing and cyanosis since birth; he was born of a non-consanguineous marriage and there was no similar defect in the family; parents noted that he was not thriving compared with other siblings. On examination; he was centrally cyanosed [Figure 1] and dyspneic. He had a pulse rate of 160/min, the apex was at the 6 th left intercostal space at the anterior axillary line; first and second heart sounds (single S2) with a continuous murmur at the upper left sternal margin, which was also heard posteriorly. There was hepatomegaly but other abdominal organs were not palpable. His oxygen saturations were 82% and 88% in room air and 100% oxygen respectively. He had chest X-ray which showed cardiomegaly with increased pulmonary plethora [Figure 2]; the full blood count showed evidence of microcytic hypochromic red blood cells-red cell count of 5.76 million/mm 3 (4.52-5.90); packed cell volume of 35.5% (41.5-50.4%); mean corpuscular volume of 68.6 fl (75-96 fl); mean corpuscular hemoglobin concentration 28.1 g/dl (32-36 g/dl); mean corpuscular hemoglobin 19.3 pg (27-32 pg); red cell distribution width was 24.7% (11-16%); electrocardiogram showed sinus rhythm with bi-atrial enlargement and right ventricular hypertrophy [Figure 3]; transthoracic echocardiogram showed bi-atria with a sinus venosus type atrial septal defect [Figure 4]; [video 1]

; tricuspid atresia; a single ventricle [video 2]

and a single arterial trunk arising from the ventricle. The final diagnosis of cor triloculares biatriatum with truncus arteriosus and atrial septal defect was made. He has since been on diuretics and nutritional rehabilitation while awaiting corrective surgery.
Figure 1: Presence of cyanosis with digital clubbing

Click here to view
Figure 2: Chest X-ray showing cardiomegaly with pulmonary plethora

Click here to view
Figure 3: Electrocardiogram showing evidence of bi-atrial enlargement with right ventricular hypertrophy

Click here to view
Figure 4: Color flow mapping showing mosaic patterns at the superior area of the atrial septum

Click here to view



  Discussion Top


The history of cor triloculares biatritum though often attributed to Holmes dates back to 1699, when Chemineau described the first case of a heart composed of two atria and a single ventricle. [8] Cor triloculares biatritum depicts a univentricular heart with unique hemodynamics; which involves mixing of both oxygenated and deoxygenated blood in a single ventricle. The clinical feature varies and survival of unrepaired cases into adulthood had been reported. [9],[10] The varied presentations may be dependent on the associated outflow and inflow tracts abnormalities and other cardiac defects. Therefore a small pulmonary artery arising from the ventricle may present a clinical scenario akin to tetralogy of Fallot with significant right to left-right shunting and less pulmonary blood flow; whereas a large pulmonary artery may present a clinical feature similar to transposition of the great vessels with ventricular septal defect; therefore they may present in heart failure due to increased pulmonary blood flow and central cyanosis may be less appreciated in them as was documented in this patient. Furthermore, reports have shown that those with larger pulmonary trunk have better chances of survival to adulthood. [11]

This case also had a continuous murmur over the left upper sternal margin, similar to reports of Brown et al., [12] which they attributed to collateral bronchial circulations, though that could not be substantiated in the index case.

Cor triloculares biatritum may be associated with other congenital heart defects such as transposition of the great vessels; and based on this, Rogers and Edwards [13] had classified it into two major groups; this case belonged to type 1D. Other cardiac defects often seen include truncus arteriosus, atrioventricular valve hypoplasia or atresia which is associated with poor outcome. In the index case, unlike those reported by Castrovinci and Cucci [14] had a tricuspid atresia and his survival up to the age of 5-months was due to the atrial septal defect, which allowed blood flow to the left atrium.


  Conclusion Top


A unique case of an association of cor triloculare biatritum type 1D with atrial septal defect and tricuspid atresia who has survived up to the age of 5 months despite these unfavorable anatomy has been reported. This will however add to the body of knowledge so far known on cor triloculare biatritum.

 
  References Top

1.Campbell M. Causes of malformations of the heart. Br Med J 1965;2:895-904.  Back to cited text no. 1
    
2.Yildiz M, Aykan AC, Guler A, Kahveci G, Ozkan M. The myocardial architecture of cor triloculare biatrium resembling reptiles. Eur J Echocardiogr 2011;12:870.  Back to cited text no. 2
    
3.van Buchem FS, Nieveen J, Marring W. Cor triloculare biatriatum. Cardiologia 1954;24:135-43.  Back to cited text no. 3
[PUBMED]    
4.Abbott ME. Atlas of Congenital Cardiac Disease. New-York: American Heart Association; 1936.  Back to cited text no. 4
    
5.Clawson BJ. Types of congenital heart diseases in 15,597 autopsies. J Lancet 1944;64:134-6.  Back to cited text no. 5
    
6.Heath D. Cor triloculare biatriatum. Circulation 1957;15:701-12.  Back to cited text no. 6
[PUBMED]    
7.Akintunde AA, Aremu AA, Akinboro AO, Akinlade MA. Cor triloculare biventriculare and pulmonary stenosis in a Nigerian primigravid woman: A case report. Int J Cardiovasc Res 2013;2:3.  Back to cited text no. 7
    
8.Khairy P, Poirier N, Mercier LA. Univentricular heart. Circulation 2007;115:800-12.  Back to cited text no. 8
    
9.Penman HG, Whitty RH. Cor triloculare biatriatum. Br Heart J 1963;25:141-4.  Back to cited text no. 9
    
10.Lumish SH, Deshmukh MM, Likoff W. Cor triloculare biatrium with pulmonary stenosis. Am J Cardiol 1959;4:535-9.  Back to cited text no. 10
[PUBMED]    
11.Edwards JE, Chamberlin WB Jr. Pathology of the pulmonary vascular tree. III. The structure of the intrapulmonary arteries in cor triloculare biatriatum with subaortic stenosis. Circulation 1951;3:524-30.  Back to cited text no. 11
    
12.Brown JW, Heath D, Morris TL, Whitaker W. Tricuspid atresia. Br Heart J 1956;18:499-518.  Back to cited text no. 12
[PUBMED]    
13.Rogers HM, Edwards JE. Cor triloculare biatbriatum: An analysis of the clinical and pathologic features of nine cases. Am Heart J 1951;41:299-310.  Back to cited text no. 13
[PUBMED]    
14.Castrovinci F, Cucci CE. Cor triloculare biatriatum combined with atresia (or hypoplasia) of the mitral valve and of the ascending aorta; report of two cases with transposition of the great vessels; discussion of related embryology and physiopathology. Dis Chest 1957;31:180-94.  Back to cited text no. 14
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed2287    
    Printed74    
    Emailed0    
    PDF Downloaded182    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]