ABSTRACT
Aims: To highlight the rare occurrence of double outlet right ventricle (DORV) with Dextrocardia and Situs Inversus in a three-year-old boy viz-a-viz what has been reported in the literatures. Presentation of Case: N.A is a three year old boy who presented with easy fatiguebility, bluish discolouration of lips and tongue and occasional dyspnoea on exertion noticed since about 12 months of life. There was no associated history of cough, leg swelling or frequent hospitalization. Physical examination revealed a small for age, centrally cyanosed boy with conjuctival ejection, and grade 4 digital clubbing. Pulse rate was 100 beats /min regular and synchronous with other peripheral pulses. There was a praecordial bulge to the right with apex beat at the 4th right intercostal space mid-clavicular line. Heart sounds were 1st and 2nd with a grade 2 systolic murmur at the right upper sternal border. The liver was 4 cm palpable below the left costal margin firm non-tender. Pulse oximetry showed SPO2 value of 75-79% in ambient air. He had chest radiograph, electrocardiogram, abdominal ultrasound and two-dimensional echocardiography which confirmed a diagnosis of DORV with Situs totalis. Discussion and Conclusion: Situs Inversus totalis (Dextrocardia co-existing with Situs inversus) is associated with lower incidence of congenital heart defects as compared with Dextrocardia and Situs solitus. The defects commonly associated with it, are a transposition of great arteries (TGA) and ventricular septal defects (VSD) unlike in the index patient where DORV is being reported.