A Rare Congenital Heart Disease — Cor Triatriatum

Cor triatriatum is among the rarest of all congenital cardiac anomalies accounting for 0.1%-0.4% of CHD.^3,4 In cor triatriatum the left atrium is divided by a fibro muscular membrane in to two distinct chambers: a posterior-superior chamber receiving the four pulmonary veins and an anterior-inferior chamber (true left atrium) that connects to the left ventricle by means of mitral valve.⁵

This lesion is usually symptomatic during childhood (symptoms of left heart obstruction or arrhythmia). A minority of individuals present in adulthood, when the diagnosis is made incidentally.⁶

We report a case a patient who was hospitalized for treatment of viral hepatitis but she was incidentally diagnosed as having associated cor triatriatum. So, we felt interested to publish this rare case.

Case

A 7 years old girl of 15 kilogram weight was admitted in pediatric ward of a medical college hospital with the complaints by the attendant of low grade irregular fever and palpitation since birth, right upper abdominal pain for 15 days, and yellow coloration of sclera and urine for 8 days. She also had weakness and poor effort tolerance. Her stool color was normal and there was no history of blood transfusion. She was from a lower middle class family and fully immunized. There was no history of chest pain and breathlessness. On examination of the girl she was moderately icteric, not anemic, pulse was 100 per minute, blood pressure was 90/70 mm of Hg. Temperature was normal. There was tender hepatomegaly.  We examined the cardiovascular system and found regular pulse, visible apex beat. Apex was situated 4 cm apart from the midsternal line in the left 4th intercostals space medial to the midclavicular line. There was palpable P2. Second heart sound was found loud in the pulmonary area. There was soft systolic murmur in the mitral area with no radiation, other systemic examination revealed normal. Provisionally the case was diagnosed as congenital heart disease with hepatitis (Viral). Following investigation was done. X-ray chest A/P view was normal (Fig.-1). Total leucocyte count was 14000/cumm, Neutrophil/Lymphocyte/Eosinophil/Monocyte — 50/41/08/01 percent. ESR (Erythrocyte Sedimentation Rate) — 30 mm in the first hour, hemoglobulin percentage — 11.1 gm/dl, Serum Bilirubin — 5.4 mg/dl, SGPT -310 iu/L. HBsAg —Negative, Anti HCV- Negative, ASO titer 200 u/ml, urine for routine examination — normal. ECG showed sinus tachycardia with left atrial hypertrophy (Fig.-2). Color Doppler Echocardiography was done and found cor triatriatum with left atrial enlargement (Fig.- 3 & 4). Finally, the case was diagnosed as CHD (cor triatriatum) with acute viral hepatitis. Patient was treated for acute viral hepatitis and referred to the pediatric cardiologist for better management.

The three main embryological theories explaining the development of cor triatriatum are malseptation, malcorporation, and entrapment.8 It may result from incomplete incorporation of the common pulmonary vein in to left atrium , abnormal overgrowth of septum primum,^3,9entrapment of common pulmonary vein by the left horn of the sinus venosus preventing its incorporation in to the left atrium³ or persistence of a left sided superior vena cava which may impinge on the left atrium, resulting in the formation of an abnormal membrane.⁹  The most frequent cardiovascular abnormalities associated with cor triatriatum sinister in adult are usually related to the spectrum of  left heart obstruction,⁷ mitral regurgitation and supravalvular annulus,^7,9 and PLSVC ( Persistent Left Superior Vena Cava) draining in the coronary sinus.⁷ Other abnormalities  are ostium secondum atrial septal defects, less commonly aortic regurgitation with dissecting aneurysm (bicuspid aortic valve?)⁷ and anomalous partial pulmonary venous return ^7,9 other associated anomalies are the unroofed coronary sinus with a left superior vena cava joining the left atrium, ventricular septal defect, coarctation of aorta, tetralogy of fallot and rarely asplenia and polysplenia.¹⁰ Our case had no other associated cardiac and non cardiac anomalies.

The diagnosis is usually established by 2D TTE (Transthoracic Echocardiography) color flow mapping usually demonstrates increase in velocity and turbulent flow suggesting obstruction that can be assessed by continuous wave Doppler through the membrane.⁹ Transesophageal echocardiography is superior to TTE to diagnose cor triatriatum, providing better imaging of the left atrium, left atrial appendage, morphology of the dividing membrane and the degree of obstruction.⁶ In our patient 2D M Mode Doppler TTE was abnormal with septum and enlargement in the left atrium but there was no other anomalies observed.

Conclusion

Cor triatriatum may be asymptomatic and diagnosed incidentally. Every patient who has palpitation and failure to thrive should have thorough cardiologic evaluation to find out the rare cardiac abnormalities and managed accordingly. If not detected early there might be left heart obstruction leading to left heart failure and morbidity / mortality.

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