Double Inlet Left Ventricle (DILV) with Mal-posed Great Vessels: A Challenge in a Developing World

Background: Double Inlet Left Ventricle (DILV) with transposition of the great vessels is rare in children with congenital heart disease. We present two cases seen at a paediatric echocardiography centre. Baby ND is a 28-day old female who presented with respiratory distress and cyanosis from the first day of life. Echocardiography showed a double inlet left ventricle and mal-posed great vessels. MB is a seven-month-old female who presented with breathlessness and cyanosis from the first day of life. Examination showed a soft pan-systolic murmur of grade 2 variety at the mid sternal boarder. ECG showed left ventricular dominance and abnormal T changes. Echocardiography revealed a double inlet left ventricle and mal-posed great vessels. Conclusion: Double inlet left ventricle and mal-posed great vessels is a rare complex cardiac anomaly of univentricular physiology. A high index of suspicion (especially if the new born presents with first day history of cyanosis and breathlessness) is necessary for quick referral and surgical intervention.