Anesthetic management for a case of an isolated congenital unilateral absence of the right pulmonary artery

: Isolated unilateral absence of the right pulmonary artery without any intracardiac anomaly (APA) is a rare congenital cardiovascular disorder. We report a case of a 47-day-old female infant with right APA who underwent pulmonary angiogram under general anesthesia. Induction and maintenance of anesthesia consisted of total intravenous anesthesia with propofol, remifentanil and vecuronium. Hemodynamic status and oxygenation were stable throughout the examination. Mean pulmonary artery pressure was 22 mmHg. During bronchofiberscopic examination through an endotracheal tube, SpO2 rapidly decreased from 98% to 50% in 10 s despite sufficient preoxygenation with 100% oxygen. Our case suggests that a care must be taken for desaturation during procedures without ventilation in PAP patients.