Extracardiac Fontan operation without cardiopulmonary bypass: A case report

Background and aim: Extracardiac Fontan operation is currently the preferred method of choice for patients with single ventricle physiology. Avoiding cardiopulmonary bypass during this procedure carries potential benefits. We present a case report of extracardiac off-pump Fontan in a 6-year-old girl with univentricular heart. To the best of our knowledge, this is the first extracardiac off-pump Fontan procedure in Indonesia. Case presentation: We present a case of a 6-year-old girl with tricuspid atresia, ventricular septal defect, and pulmonary stenosis who previously underwent central shunt in 2017 and bidirectional cavopulmonary (Glenn) shunt in 2019. We successfully did extracardiac conduit Fontan (ECF) operation without cardiopulmonary bypass. Conclusion: Performing extracardiac off-pump Fontan operation is reported to result in better outcomes. Although the technique may be challenging, we recommend attempting the technique whenever possible to eliminate the adverse effects. Introduction and importance: The extracardiac Fontan operation is preferred for patients with single ventricle physiology, primarily due to the benefits of avoiding cardiopulmonary bypass (CPB). We present a case of an extracardiac off-pump Fontan procedure performed on a 6-year-old girl with a univentricular heart, which is the first of its kind in Indonesia. Case presentation: A 6-year-old girl with tricuspid atresia, a ventricular septal defect, and pulmonary stenosis, previously underwent a central shunt in 2017 and a bidirectional cavopulmonary (Glenn) shunt in 2019. We performed a successful ECF operation without using CPB. Clinical discussion: The ECF has advantages over the lateral tunnel Fontan, including optimal laminar flow in the systemic venous pathway, a potentially lower incidence of arrhythmia, and better ventricular function. Performing ECF without CPB results in favorable intraoperative hemodynamics, early extubation, decreased pleural effusions, and reduced need for inotropic agents and blood products. The patient developed chylothorax on the sixth post-operative day, likely due to an acute rise in systemic venous and lymphatic pressures and the significant discrepancy between the conduit and the inferior vena cava (IVC). This was managed with a low-fat diet. Conclusion: The extracardiac off-pump Fontan operation is associated with better outcomes and fewer complications. Despite its challenges, this approach should be considered to mitigate the adverse effects associated with CPB.