Anesthetic management in tetralogy of fallot with pulmonary atresia and major aortopulmonary collateral arteries in pediatric patients: One year experience

Background: Unlike the typical tetralogy of fallot (TOF), the presence of pulmonary atresia and major aortopulmonary collaterals is recognized as a rare but severe variant of TOF. The objective was to describe the perioperative anesthetic management of pediatric patients who underwent unifocalization procedure and to describe their postoperative morbidity and mortality. Methods: A retrospective observational study was conducted among pediatric patients who underwent unifocalization procedure at Prince Sultan Cardiac Center (PSCC) between October 2017 and October 2018. Unifocalization procedures were performed in a two-staged approach. Anesthetic management of both stages had similar concerns and challenges but with few peculiar issues. These included preoperative assessment, intraoperative management, vascular access, positioning, ventilation, optimizing hemodynamics, cardiopulmonary bypass monitoring, and postoperative management. Results: A total of 19 unifocalization procedures were included. The average age was one year and 52.6% were females. Two patients (10.5%) arrived in theater already with intubated ventilation. Continuous mandatory ventilation was used in 11 (57.9%) patients while one-lung ventilation was used in the rest of patients. Approximately, 30% of patients encountered a stormy postoperative course, 52.6% underwent cardiopulmonary bypass with or without cross-clamping of the aorta, and 10.5% had reperfusion injury. Conclusions: Despite the major challenges of unifocalization, significantly low rates of morbidity and mortality were observed in our patients. A thorough familiarity of different airway and ventilation issues, besides meticulous hemodynamic and anesthetic management, is of paramount importance. The maintenance of hemodynamic stability, hemostasis, and proper ventilation is critical for the success of the operation.