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. We present images of a case where the diagnosis of absent right SVC with persistent left SVC was made with intraoperative transesophageal echocardiography and discuss the anesthetic and perfusion implications of such findings.
Keywords: Holt-Oram syndrome; transesophageal echocardiography; venous anomalies.