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Adult Congenital Heart Disease
Published in Takahiro Shiota, 3D Echocardiography, 2020
Pastora Gallego, Silvia Montserrat
Cor triatriatum sinister is a rare CHD in which a thick fibromuscular or membrane divides the LA into a proximal or superior chamber that drains the pulmonary venous blood and a distal or inferior chamber that is in contact with the AVvalve and contains the atrial appendage and the true atrial septum. It represents 0.1% of all CHDs, and 80% have associated ASD.18 Clinical features mimic those of mitral stenosis.
Scimitar syndrome with hemi-cor triatriatum
Published in Acta Cardiologica, 2023
Sudipta Mondal, Richard Raj R., Arun Gopalakrishnan, Sivadasanpillai Harikrishnan
A 38-year-old lady with class II exertional dyspnoea was referred to us with a diagnosis of atrial septal defect. Clinical examination revealed dextrocardia, cardiomegaly, wide fixed second sound with accentuated pulmonary component and a grade 2 mid-systolic murmur. The electrocardiogram suggested atrial fibrillation and features of dextrocardia. Chest X-ray confirmed dextrocardia with pulmonary plethora (Figure 1(Panel A)). Echocardiogram showed situs solitus, dextroposed heart, d-looped ventricles, large secundum atrial septal defect, right heart volume overload and moderate pulmonary hypertension. A thick membrane-like structure was noted in the left atrium (LA) proximal to left atrial appendage without flow obstruction suggesting non-obstructive cor triatriatum (Figure 1(Panel C, D), Supplementary Video 1). Anomalous drainage of the right lower pulmonary vein into the inferior caval vein was demonstrated on subxiphoid imaging (Figure 1(Panel B), Supplementary Video 2) Cardiac computed tomography confirmed the atrial septal defect with right heart volume overload and the drainage of the left pulmonary veins into a chamber posterior to the left atrium and thereafter to the left atrium constituting the hemi-cor triatriatum (Figure 1(Panel E)). The right upper and lower pulmonary veins were hemianomalous and connected to the inferior caval vein at its entry to the right atrium with the ‘Scimitar’ course (Figure 1(Panel F, H), Supplementary Video 3–5). The right lung was noted to be hypoplastic. Cardiac catheterisation revealed a 1 mm gradient across the hemi-cortriatriatum and right pulmonary venous drainage to the inferior caval vein (Figure 1(Panel G), Supplementary Video 6). Scimitar syndrome with hemi-cortriatriatum has not been reported earlier to the best of our knowledge. It points towards the embryological basis of left atrial development and pulmonary venous incorporation into the left atrium with associated anomalies stressing on the need for strict vigilance while evaluating such cases.