Cor-Triatriatum Dexter with Associated Cyanosis in a 3-Month-Old Girl.

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.

INTRODUCTION

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.[23] 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

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

Figure 2

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

Figure 3

Echocardiogram view showing transposition of the great vessels

DISCUSSION

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

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

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Conflicts of interest

There are no conflicts of interest.