Key result
Case report details anesthetic management in an infant with Williams syndrome undergoing aortic stenosis repair.
Why the study?
Williams syndrome involves congenital heart disease and multisystem manifestations that have important implications during the perioperative period, including risk of sudden death related to coronary artery abnormalities.
Case Report (n=1)
Highlights the significant perioperative risks, including sudden death from coronary abnormalities, in patients with Williams syndrome undergoing cardiac surgery.
Alerts to multisystem perioperative risks in Williams syndrome; leaves open evidence-based protocols pending higher-level data.
Williams syndrome, initially described by Williams, Barratt-Boyes, and Lowe in 1961, consists of characteristic dysmorphic features, congenital heart disease, and distinctive behavioral and emotional traits. In addition to acquired and congenital heart disease, manifestations in the renal, endocrine, musculoskeletal, and central nervous system may have implications during the perioperative period. Congenital and acquired heart disease can be a significant issue as sudden death, related to abnormalities of the coronary arteries, has been reported perioperatively in these patients. The authors present a 7-month-old infant, previously diagnosed with Williams syndrome, who required anesthetic care for repair of subaortic and supravalvular aortic stenosis. The potential perioperative implications of Williams syndrome are discussed.
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Medley et al. (2005) conducted a case report in Williams syndrome (n=1). Anesthetic care for repair of subaortic and supravalvular aortic stenosis was evaluated. Anesthetic care was provided for a 7-month-old infant with Williams syndrome undergoing repair of subaortic and supravalvular aortic stenosis, highlighting the perioperative implications of the syndrome.
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