A life-threatening complication of the arterial tourniquet in Timothy syndrome.
An infant with Timothy syndrome, a congenital syndrome resulting in a long QT interval and high risk of death in childhood, is presented along with his perioperative management. Characteristic life-threatening arrhythmias thought to be caused by excess sympathetic stimulation secondary to an arterial tourniquet are described.
Yates
David
D
Department of Anaesthesia, York District Hospital, York, UK. dave.helen@fsmail.net
Yates
Alan
A
Collyer
Thomas
T
D000015:Abnormalities, Multiple / Q000503:physiopathology / Q000601:surgery; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000628:therapy; D004562:Electrocardiography / Q000379:methods; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000209:etiology / Q000628:therapy; D008133:Long QT Syndrome / Q000151:congenital / Q000503:physiopathology*; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods; D016038:Skin Transplantation / Q000379:methods; D013576:Syndactyly / Q000150:complications / Q000601:surgery*; D014111:Tourniquets / Q000009:adverse effects*; D034941:Upper Extremity / Q000098:blood supply / Q000601:surgery*