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Sudden cardiac death under anesthesia in pediatric patient with williams syndrome: A case report and review of literature


1 Division of Pediatric Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
2 Division of Pediatric Anesthesiology, University of Missouri, Columbia, MO 65212, USA
3 Department of Medical Education, Metrowest Medical Center, Framingham, MA 01702, USA
4 Division of Pediatric Cardiac Anesthesiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
5 Division of Pediatric Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA

Correspondence Address:
Punkaj Gupta
175 Cambridge Street, 5th Floor, Boston MA 02114
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.58834

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Year : 2010  |  Volume : 13  |  Issue : 1  |  Page : 44-48

 

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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.






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1 Division of Pediatric Critical Care, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
2 Division of Pediatric Anesthesiology, University of Missouri, Columbia, MO 65212, USA
3 Department of Medical Education, Metrowest Medical Center, Framingham, MA 01702, USA
4 Division of Pediatric Cardiac Anesthesiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA
5 Division of Pediatric Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA

Correspondence Address:
Punkaj Gupta
175 Cambridge Street, 5th Floor, Boston MA 02114
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9784.58834

Rights and Permissions

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.






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