Anesthetic Management of a Pediatric Patient With Severe Idiopathic Pulmonary Arterial Hypertension and a Potts Shunt Undergoing Noncardiac Surgery: A Case Report

Idiopathic pulmonary arterial hypertension (IPAH) presents a significant anesthetic challenge, especially in pediatric patients with advanced disease and prior surgical interventions such as a Potts shunt. We describe the perioperative anesthetic management of a 15-year-old girl with severe IPAH and a functional Potts shunt who required an extensive surgical debridement and flap reconstruction for a pressure ulcer complicated by chronic osteomyelitis. The patient had a history of spinal cord infarction with resulting paraplegia, further increasing the complexity of care. The need for non-cardiac surgery in this high-risk setting demanded meticulous multidisciplinary planning. Anesthetic goals included maintaining hemodynamic stability, avoiding increases in pulmonary vascular resistance, and preventing pulmonary hypertensive crises. Intraoperative management involves, in general, tailored sedation, cautious induction and maintenance of anesthesia, optimized ventilation strategies, and continuous invasive monitoring. Pulmonary vasodilator therapy was continued and adjusted perioperatively, and anticoagulation was carefully managed. This case highlights the importance of individualized anesthetic planning, close collaboration between anesthesiology, cardiology, and surgical teams, and the need for vigilant perioperative monitoring in patients with severe IPAH undergoing non-cardiac procedures. It underscores the critical role of anesthesiologists in minimizing perioperative risk and ensuring favorable outcomes in this vulnerable population.