Double Outlet Right Ventricle With Intact Ventricular Septum And Interrupted Aortic Arch: A Case Study Of A Foetus With Trisomy 18

Background: 90% of the infants with trisomy 18 suffers from congenital heart defects. Double outlet right ventricle (DORV) is a rare form of congenital heart disease where both the arterial roots arise from the right ventricle. Interrupted aortic arch (IAA) is an extremely rare congenital anomaly representing approximately 1% of congenital heart disease, characterised by lack of luminal continuity between the ascending and descending thoracic aorta Case Report: A still born male foetus of around 20 weeks gestation, weighing 238g with trisomy 18 was collected from the Obstetrics and Gynaecology Department, RIMS, Imphal. On dissection, both the great arteries are committed to the right ventricle and are unobstructed. The aorta arose right to the pulmonary trunk and is interrupted distal to the left subclavian artery. Pulmonary-aortic connection is maintained by a large patent ductus arteriosus. The mitral valve was atretic. The left ventricle was hypoplastic. Conclusion: We found this case notable as this is an exceedingly rare case of DORV with intact ventricular septum, in the setting of mitral atresia, hypoplastic left ventricle associated to type A, IAA in Trisomy 18