LAUSR

Abstract Anatomically corrected malposition of great arteries constitutes a rare form of congenital cardiac malformation where aorta and pulmonary artery arise from respective ventricles but the alignment of the great vessels does not conform to that of a normal heart. It often poses a diagnostic challenge due to a close resemblance to other great vessel malposition defects. The majority of the patients with this disorder are physiologically corrected and present due to associated lesions, most commonly ventricular septal defects. In this review, we present two children of anatomically corrected malposition who presented with hemodynamically significant ventricular septal defects. The older child had a restrictive defect and was successfully managed by percutaneous closure in-spite of associated septal malalignment. The younger child had a larger defect and underwent surgical closure for the same. None of the children had any associated outflow tract obstructions. Closure of ventricular septal defect with such an anatomy either surgically or by percutaneous route has its own set of challenges. Although the risks of conduction abnormalities are not increased, there is a potential for future development of outflow tract obstruction requiring periodic follow-up. Detailed echocardiography and proper understanding of the unique anatomy forms the basis of successful management of associated lesions in anatomically corrected malposition of great arteries.