LAUSR

Introduction Chylothorax, a relatively rare congenital heart disease early postoperative complication, is occurring more frequently due to complexity of cardiac surgeries. Presentation of case We present a 9-month-old boy who had hypoplastic left heart (HLH) syndrome with interrupted inferior vena cava (IVC) and bilateral superior vena cava (SVC) palliated with left sided modified Blalock-Taussig (MBT) shunt during neonatal period and second stage palliation with left sided bidirectional glen (BDG) procedure and right sided Kawashima procedure develop bilateral chylothorax two weeks after discharge. Discussion This is the first reported case in the literature of a patient who developed chylothorax with relatively low Fontan systemic venous pressures after a Kawashima procedure. Clinically important chylothorax may be a marker of poor long-term outcomes, demonstrating an inability to handle overwhelming lymphatic congestion. Conclusion Early diagnosis of chylothorax in complex cardiac surgeries may permit successful conservative management.