Perioperative anesthetic management in pediatric patients with medically refractory pulmonary arterial hypertension undergoing surgical or transcatheter potts shunt: a retrospective case series

BACKGROUND: Pulmonary arterial hypertension (PAH), a life-threatening disorder in children, necessitates palliative interventions like Potts shunt (surgical/transcatheter) for medically refractory cases. Perioperative anesthesia management remains challenging due to hemodynamic instability. This study describes our institutional anesthetic approach and summarizes the perioperative outcomes of 28 pediatric patients who underwent Potts shunt creation. METHODS: A retrospective analysis was conducted on 28 consecutive children who underwent either surgical or transcatheter Potts shunt at Shanghai Children's Medical Center between 2021 and 2024. Perioperative data were extracted from structured electronic medical records. RESULTS: Among the 28 patients, 18 underwent surgical Potts shunt creation (100% requiring cardiopulmonary bypass [CPB], median CPB duration: 91[82-108] minutes) and 10 underwent transcatheter procedures (20% requiring CPB). Anesthesia induction predominantly utilized ciprofol-alfentanil (60.7%) and s-ketamine (28.6%). Preoperative treprostinil was universal, with 7.1% of patients requiring additional vasoactive agents. Post-shunt hemodynamic support included norepinephrine (92.9%), dopamine (28.6%) and dobutamine (28.6%). Compared with the transcatheter group, surgical patients experienced significantly longer stays in the cardiac intensive care unit (CICU), (8[7-14] vs. 5[4-9] days, p = 0.005), longer durations of mechanical ventilation (2.5[1.5-10.5] vs. 1.8[1.0-4.3] days), and longer total hospitalizations (31[23-36] vs. 25.5[19-46] days). No intraoperative cardiac arrests occurred. The in-hospital mortality rate was 14.3% (4/28). CONCLUSIONS: Both surgical and transcatheter reverse Potts shunt procedures represent feasible salvage therapies for children with refractory pulmonary hypertension, albeit with considerable perioperative risks. Targeted hemodynamic regulation and rigorous intraoperative emergency management are therefore essential to maintain circulatory stability, prevent pulmonary hypertensive crises, and ensure overall clinical safety.