Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiac anomaly characterized by anomalous drainage of one or more pulmonary veins into the systemic venous circulation, resulting in a left-to-right shunt. Perioperative management can be challenging due to the risk of hemodynamic instability with changes in pulmonary and systemic vascular resistance (SVR), particularly in young children, where the literature is scarce. We report the successful perioperative anesthetic management of a 3-year-old child with uncorrected PAPVC associated with VACTERL anomalies undergoing laparoscopic-assisted anorectoplasty. General anesthesia with epidural analgesia was administered, with careful attention to maintaining a balance between pulmonary and SVR, avoiding factors that increase pulmonary vascular resistance, and ensuring controlled ventilation during pneumoperitoneum and lithotomy positioning. The perioperative course was uneventful, with stable hemodynamics and no hypoxia or cyanotic spells. This case highlights that a physiology-guided anesthetic approach and meticulous monitoring ensure safe outcomes.
Sonika et al. (Mon,) studied this question.