Diagnosis of absent right superior vena cava with intraoperative transesophageal echocardiography in a child with Holt-Oram syndrome: Anesthetic and perfusion implications.

Holt-Oram syndrome is a rare autosomal disorder with cardiac, vascular, and upper limb anomalies. Previous reports have described anesthetic and perioperative challenges including difficulty in arterial and venous cannulations, airway management and rhythm, and temperature abnormalities. There are no previous reports of absent right superior vena cava (SVC) in children with Holt-Oram syndrome.