Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: a case report and review of literature.

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.

Preliminary experience with a combination of dexmedetomidine and propofol infusions for diagnostic cardiac catheterization in children.

No specific regimen has been universally accepted as ideal for procedural sedation during cardiac catheterization in infants and children. In this paper, we retrospectively describe our preliminary experience with a continuous infusion of dexmedetomidine and propofol for sedation during cardiac catheterization in children with congenital heart disease. The short-half life of these two drugs creates a potential for easier titration, quicker recovery and less prolonged sedation-related adverse effects. This combination was not only able to limit the dose of either drugs, but was also very stable from cardio-respiratory standpoint. There were no adverse effects noted in our two patients. This initial experience showed that the combination of propofol and dexmedetomidine as a continuous infusion may be a suitable alternative for sedation in spontaneously breathing children undergoing cardiac catheterization.