We describe a neonate presenting with a cholestatic jaundice due to Alagille syndrome. On echocardiography as part of the diagnostic work-up, we noted a slight dilation of the normally functioning left ventricle at the initial examination in the third week of life. Over the next 2 weeks, serial echocardiograms showed slowly progressive dilation and dysfunction of the left ventricle, together with a persistent mild elevation of levels of cardiac Troponin-I in the serum. These findings, unrelated to the underlying Alagille syndrome, prompted cardiac catheterisation, which confirmed that the main stem of the left coronary artery was originating from the pulmonary trunk. Surgery was successfully performed still in the presymptomatic period. The association of Alagille syndrome with anomalous left coronary artery arising from the pulmonary trunk is most unusual. We emphasise the cardiac findings prior to detection of the anomalous origin of the coronary artery.
