|Year : 2014 | Volume
| 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 Publication||3-Mar-2014|
Department of Paediatrics, Aminu Kano Teaching Hospital, Bayero University, Kano
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
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.  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. 
Cor-triloculares biatritum is a rare congenital heart defect and few cases have been reported so far , it constituted about 13 out of the 1000 cases of congenital heart defects reported by Abbott  and 7 out of 141 cases of congenital cardiac defects reported by Clawson  however no known case has been reported among Africans to the best of the author's knowledge, though Akintunde et al.  had reported a case of cor triloculares biventricularis with pulmonary stenosis in a Nigerian woman in pregnancy.
| Case Report|| |
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 3: Electrocardiogram showing evidence of bi-atrial enlargement with right ventricular hypertrophy|
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|Figure 4: Color flow mapping showing mosaic patterns at the superior area of the atrial septum|
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| Discussion|| |
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.  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. , 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. 
This case also had a continuous murmur over the left upper sternal margin, similar to reports of Brown et al.,  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  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  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|| |
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|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]