Anaesthesia-Related Haemodynamic Complications in Williams Syndrome Patients: A Review of One Institution's Experience

Abstract

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.

A rare association of central hypothyroidism and adrenal insufficiency in a boy with Williams-Beuren syndrome

“…In many of these patients, there are no changes on pathological examination of the heart either suggesting that the cardiac anomaly was probably not directly linked to cardiac arrest14). Although myocardial ischemia due to coronary perfusion abnormalities and use of anesthetic drugs such as suxamethonium and halothane may explain bradycardia and hypotension leading to cardiac arrest in some WBS patients, these complications have been noted in several patients unrelated to anesthesia or coronary artery hypoperfusion141516). It is notable that preoperative evaluation of hypothalamic pituitary adrenal axis is not carried out as a routine in patients with WBS14).…”

“…Our patient showed secondary adrenal insufficiency which suggests that there is a need for screening for this abnormality in children with WBS. Anesthesia-related hemodynamic complications including sudden cardiac arrest are common in children with WBS14). The usual presenting features in these patients at the time of arrest are hypotension and bradycardia that are refractory to the standard resuscitation protocols14).…”

“…Anesthesia-related hemodynamic complications including sudden cardiac arrest are common in children with WBS14). The usual presenting features in these patients at the time of arrest are hypotension and bradycardia that are refractory to the standard resuscitation protocols14). In many of these patients, there are no changes on pathological examination of the heart either suggesting that the cardiac anomaly was probably not directly linked to cardiac arrest14).…”

“…The usual presenting features in these patients at the time of arrest are hypotension and bradycardia that are refractory to the standard resuscitation protocols14). In many of these patients, there are no changes on pathological examination of the heart either suggesting that the cardiac anomaly was probably not directly linked to cardiac arrest14). Although myocardial ischemia due to coronary perfusion abnormalities and use of anesthetic drugs such as suxamethonium and halothane may explain bradycardia and hypotension leading to cardiac arrest in some WBS patients, these complications have been noted in several patients unrelated to anesthesia or coronary artery hypoperfusion141516).…”

“…Although myocardial ischemia due to coronary perfusion abnormalities and use of anesthetic drugs such as suxamethonium and halothane may explain bradycardia and hypotension leading to cardiac arrest in some WBS patients, these complications have been noted in several patients unrelated to anesthesia or coronary artery hypoperfusion141516). It is notable that preoperative evaluation of hypothalamic pituitary adrenal axis is not carried out as a routine in patients with WBS14). Bradycardia and hypotension are cardinal features of acute adrenal insufficiency17).…”