Masroor Sharfi

and 5 more

Here we report an extremely rare case of congenitally corrected transposition of great arteries (CCTGA) associated with Interrupted aortic arch (IAA) type A and ventricular septal defect (VSD) in a preterm baby. Antenatally diagnosed as large VSD and severe Coarctation of aorta. Echocardiogram done revealed that left ventricle with the mitral valve lay on the right side in continuation with the venous right atrium and was connected to the pulmonary artery. The right ventricle lay on the left in continuation with the arterial left atrium and was connected to the aorta. Smallish transverse arch with Interruption of aorta below the left subclavian artery. Large VSD with bidirectional shunt. Small PDA with restrictive right to left flow. As far we know, only one similar case was reported earlier by Cottrell, at el. (4). At that time the initial diagnosis was done by cardiac angiography as 2D color echocardiography was not available at their center, after one year they were able to use echocardiogram and same diagnosis was confirmed. In our case despite the complex anatomy we were able to diagnose our case by 2D color Echocardiogram. The Echocardiogram images and loops were so clear and confirmative that no other cardiac imaging was required. Baby was stable clinically on prostaglandin and underwent successful aortic arch repair (end to end anastomosis) and PA banding. We believe that Echocardiography still is a basic tool for diagnosis of complex congenital cardiac anomalies specially in centers where other higher diagnostic modalities are still not available.