title: Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: a case report and review of literature.
abstract: Williams syndrome is a complex syndrome characterized by developmental abnormalities, craniofacial dysmorphic features, and cardiac anomalies. Sudden death has been described as a very common complication associated with anesthesia, surgery, and procedures in this population. Anatomical abnormalities associated with the heart pre-dispose these individuals to sudden death. In addition to a sudden and rapid downhill course, lack of response to resuscitation is another significant feature seen in these patients. The authors report a five-year-old male with Williams syndrome, hypothyroidism, and attention deficit hyperactivity disorder. He suffered an anaphylactic reaction during CT imaging with contrast. Resuscitation was unsuccessful. Previous reports regarding the anesthetic management of patients with Williams are reviewed and the potential for sudden death or peri-procedure related cardiac arrest discussed in this report. The authors also review reasons for refractoriness to defined resuscitation guidelines in this patient population.
authors.affiliation: Division of Pediatric Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA. pgupta3@partners.org
authors.forename: Punkaj
authors.initials: P
authors.lastname: Gupta
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Tobias
authors.forename: Sunali
authors.initials: S
authors.lastname: Goyal
authors.forename: Martin D
authors.initials: MD
authors.lastname: Miller
authors.forename: Elliot
authors.initials: E
authors.lastname: Melendez
authors.forename: Natan
authors.initials: N
authors.lastname: Noviski
authors.forename: Michael M
authors.initials: MM
authors.lastname: De Moor
authors.forename: Vipin
authors.initials: V
authors.lastname: Mehta
mesh_terms: D000758:Anesthesia / Q000009:adverse effects*; D021921:Aortic Stenosis, Supravalvular / Q000601:surgery*; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed; D018980:Williams Syndrome / Q000150:complications*