Bilateral chylothorax: A late complication of Kawashima procedure despite normal pulmonary pressures

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.