Double outlet right ventricle, atrioventricular canal defect with severe pulmonary artery stenosis: a rare cyanotic congenital heart disease.

Double outlet right ventricle (DORV) is characterised by arousal of the aorta and the pulmonary vessel from the right ventricle, and is always accompanied by a ventricular septal defect (VSD). Our patient, a twenty days-old female child, presented to the Paediatrics Unit I of Civil Hospital, Karachi, with complaints of generalised cyanosis and reluctance to feed since birth. Apart from the fact that the neonate was cyanosed with increased respiration, the physical examination was unremarkable. Two-dimensional echocardiography revealed two atrial septal defects (ASD), a ventricular septal defect (VSD) in the membranous area, continuous with primum ASD, resulting in an atrioventricular canal defect. The aorta was dilated with the pulmonary artery stenosis. The left ventricle was rudimentary, and both of the great vessels were arising from the dominant right ventricle. Despite the rarity, DORV with complex anatomy should be considered among the probable differential diagnoses for infants presenting with generalised cyanosis since birth.