Anesthetic management of a schoolboy with uncorrected truncus arteriosus type I, and severe pulmonary hypertension undergoing repair of congenital dislocation of the knee. Case report

Abstract

Introduction: The presence of truncus arteriosus represents just 1.2-3% of all complex congenital heart pathologies and if not corrected, less than 20% survive beyond one year of life. When the disease progresses patients usually develop severe pulmonary hypertension and may even develop into Eisenmenger's syndrome. The paper discusses a case of a schoolboy with a diagnosis of uncorrected truncus arteriosus type I, and severe pulmonary arterial hypertension undergoing non-cardiac surgery.

Case discussion: This is a 9-year old schoolboy with complex heart disease and similar pulmonary and systemic blood pressures, undergoing elective orthopedic surgery under regional anesthesia, with lumbar plexus block and posterior sciatic block. This anesthetic approach provided adequate anesthesia with hemodynamic stability and no impact on vascular resistance.

Conclusion: The choice of the anesthetic technique should be a planned decision based on the cardiovascular pathophysiology of the truncus arteriosus, the level of pulmonary hypertension, and the surgical procedure to be performed. Patients with severe pulmonary hypertension are at increased risk of developing suprasystemic pulmonary pressures with considerable hemodynamic involvement. Consequently, the anesthetic technique chosen shall provide adequate anesthesia and ensure the least hemodynamic impact. Whenever possible, it is important to consider the peripheral nerve block as the first line approach for orthopedic surgery.