Resumo

AGUIRRE-SALAZAR, José Juan; MANCERA-ELIAS, Gabriel  e  ORTIZ-MARMOLEJO, Delfina. Anesthetic management to a pediatric patient with Beckwithwiedemann syndrome associated with long qt syndrome. Anest. Méx. [online]. 2019, vol.31, n.1, pp.43-50. ISSN 2448-8771.

Long QT syndrome is an inherited disorder associated with sudden death characterized by abnormal and prolonged repolarization producing a potentially dangerous substrate in the genesis of ventricular tachycardia, syncope and even sudden death; Events that can be triggered by emotional or physical stress, the symptoms can suddenly appear by convulsions, syncope or sudden death as the first manifestation, this entity may be associated with other genetic conditions such as the Bekwitt Widemann syndrome that is characterized by macrosomia, macroglossia, urogenital anomalies and abdominal wall defect.

The case of a 14-year-old female patient with long Q-T syndrome and Becwitt Widemann's syndrome submitted to an orthopedic procedure requiring anesthesia, the implications of anesthesia and the pathology presented.

Palavras-chave : Long Q-T síndrome; Becwitt Widemann syndrome; anesthesia.