|Year : 2016 | Volume
| Issue : 1 | Page : 44-46
Cor triatriatum dexter with pulmonary hypertension
O Mustafa Asani1, Safiya Gambo2, Igoche Peter2
1 Department of Paediatrics, Bayero University Kano/Aminu Kano Teaching Hospital, Kano, Nigeria
2 Department of Paediatrics, Aminu Kano Teaching Hospital, Kano, Nigeria
|Date of Web Publication||6-May-2016|
O Mustafa Asani
Department of Paediatrics, Bayero University Kano/Aminu Kano Teaching Hospital, Kano
Cor triatriatum (CT) otherwise known as a tri atrial heart is a rare congenital heart defect. This report describes a rare case of CT dexter in a 2-year-old girl who presented to our facility with cough and difficulty in breathing of a year duration and body swelling of 3 months duration. She was also found to be in heart failure. Transthoracic echocardiography revealed a membrane separating the right atrium into two chambers, with features of pulmonary hypertension. Although the patient was scheduled for follow-up, she however, died 2 days later. Early diagnosis and prompt referral of patients with this rare defect are mandatory to prevent mortality from this defect that is amenable to simple corrective surgery.
Keywords: Cor triatriatum dexter, Kano, pulmonary hypertension
|How to cite this article:|
Asani O M, Gambo S, Peter I. Cor triatriatum dexter with pulmonary hypertension. Sahel Med J 2016;19:44-6
| Introduction|| |
The triatrial heart, cor triatriatum (CT), is when one of the atrial chambers is partitioned into two by an abnormal fibromuscular membrane resulting in a triatrial heart.  It represents 0.1% of all congenital cardiac malformations.  It results from the persistent embryonic right valve of the sinus venosus which becomes a septating membrane at birth. 
It was Andral in 1829 that first recognized the partitioning of the left atrium into two chambers.  About 40 years afterwards, the first detailed structural description was published by Church.  It was, however, Borst in 1905 that coined the term CT.  Affectation may be of the left atrium (CT sinister) or the right (CT dexter).  Clinical presentation is dependent on the affected atrium, the size of communication between the subdivided compartments, and presence of associated anomalies.
These anomalies include ventricular septal defect, atrial septal defect, tetralogy of Fallot, partial anomalous pulmonary venous connection, bicuspid pulmonary valve, and aortic valve atresia to mention a few. The presence of these anomalies is observed in up to 50% of cases. 
CT dexter (affectation of the right) is much less common than the affectation of the left (CT sinister) and accounts for only 0.025% of the anomaly.  Unlike CT sinister in which morbidity is high in untreated cases, significant sequalae is unusual in CT dexter because it is rarely associated with major congenital anomalies and severe symptoms. 
Echocardiography is the method of choice for identifying the triatrial heart in our setting. However, this is not always feasible in a resource constraint setting like ours, and thus cases are often missed. This study aims to describe the presence and presentation of this rare anomaly in a child who had been symptomatic since infancy.
| Case report|| |
A 2-year-old Hausa girl was referred from a specialist private clinic to Aminu Kano Teaching Hospital, Kano for echocardiography. Child has been having recurrent cough and difficulty in breathing of a year duration, and body swelling of 3 months duration. The cough was nonparoxysmal and occurs throughout the day. Difficulty in breathing was exacerbated by lying down flat, has increasing fatigue and dark discoloration of mucous membrane, few weeks prior to the presentation. Body swelling has been recurrent and was first noticed on the legs but gradually involved the abdomen and face. Swelling does not resolve as the day goes by. No change in urine volume or frequency. The perinatal history was not adversely eventful.
Examination revealed a child with generalized body swelling central cyanosis and mild respiratory distress. No digital clubbing. She weighed 10 kg (<5 th centile for age and gender); occipito - frontal circumference = 46 cm (between 10 th and 25 th centile for age and gender); height = 79.5 cm (5 th centile for age and gender); mid arm circumference = 13 cm. Pulse rate was 120/min, full volume and regular while her blood pressure was 70/40 mmHg (<50 th centile), left arm sitting. The apex beat was displaced to 5 th intercoastal space, mid-clavicular line. She had the first and second heart sounds with a grade 4 systolic murmur, maximum around the left lower sternal edge, radiating to the back. Respiratory rate was 68/min. Pulse oxygen saturation (SpO 2 ) was 65% in room air. She has diffuse bilateral coarse crepitations. The liver was found to be 6 cm palpable below the right costal margin, nontender. The result of urinalysis was normal [Figure 1].
|Figure 1: Chest radiograph showing gross cardiomegaly in a 2-year-old girl with cor triatriatum dextrum|
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Chest radiograph showed levocardia, cardiomegaly (cardiothoracic ratio = 69%) with cephalization of the pulmonary veins.
Transthoracic echocardiography revealed membrane (perforated) dividing right atrium into two compartments; severe tricuspid incompetence (TI), V max = 5.2 m/s, peak gradient = 108 mmHg, inferior vena cava collapses <30%; dilated right atrial and right ventricle; severe mitral incompetence (MI) (vena contracta = 8.1 mm); conclusion; CT dextrum with severe pulmonary hypertension (120 mmHg), TI and MI [Figure 2] and [Figure 3]. Mother was counseled on child's condition and need for urgent surgery. The patient was placed on oral frusemide 10 mg twice daily and spironolactone 6.125 mg twice daily and was scheduled for follow-up in our pediatric cardiology clinic. However, the patient died 2 days later.
|Figure 2: Two-dimensional and M-mode echocardiography. A4C apical four chamber. RV: Right ventricle. RA1: Proximal right atrium. RA2: Distal right atrium|
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|Figure 3: Two-dimensional echocardiography, apical four chamber view showing presence of a membrane separating the right atrium into two parts - RA1: Proximal right atrium. RA2: Distal right atrium|
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| Discussion|| |
Diagnosis of this rare anomaly can be made at any age but usually as an incidental finding.  Our observation of the existence of the anomaly in a 2-year-old girl is similar to the observation of Chinawa et al. in Enugu where the condition was reported in a 1½-year-old patient. Ejiroghene and Omotola  also reported the disease in an adult. However, Douglas et al.  in Atlanta and Alboliras et al.  documented the anomaly in younger patients within the neonatal period. Their earlier diagnosis is probably attributable to the presence of sophisticated diagnostic facilities typical of the developed countries. Our observation of the condition in a female patient is in contrast to the slight male predominance of the disease  although both Douglas et al.  and Barrea et al.  also reported female cases. Manifestation varies and depends on degree of obstruction to venous flow as well as other associated anomalies.  The absence of consanguinity observed in our case was also documented in the 1½-year-old boy that was reported by Chinawa et al. 
Our patient presented with congestive cardiac failure. This may be due to the obstruction of systemic venous return in the presence of an intact interatrial septum. Although echocardiography remains the most available diagnostic tool, magnetic resonance imaging is said to be of higher sensitivity.  The cyanosis noted in this patient in the absence of a shunt is likely secondary to the increased pulmonary venous pressure from congestive cardiac failure. The presence of cyanosis was first observed few weeks before presentation.
Pulmonary hypertension in this patient is likely to be secondary to the mitral regurgitation. Mitral regurgitation in this patient which is likely an independent and congenital lesion causes elevated left atrial pressure with subsequent disruption of alveolar capillary complex and structural changes in the distal arterioles leading to reactive pulmonary hypertension. 
The long-term prognosis for isolated CT dexter is good, but there is a scanty report on those associated with pulmonary hypertension, mitral regurgitation, and congestive cardiac failure.  These associated findings may have led to the early mortality of our case.
Treatment is either surgical resection of the membrane or percutaneous catheter disruption in selected cases. 
| Conclusion|| |
In view of the above findings, a high index of suspicion is necessary to identify such rare congenital heart anomalies. Early diagnosis with two-dimensional echocardiography will facilitate prompt referral of this defect that is amenable to simple corrective surgery. Delay in diagnosis could be fatal.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]