Sedation in a patient with William Syndrome without supravalvular aortic stenosis and normal QT: Challenge still remains

Abstract

William Syndrome (WS) is a rare genetic condition related to deletion of elastin gene resulting in distinctive facies, cardiovascular diseases, learning difficulties, developmental delay, unique personality characteristics, endocrine involvement, etc.¹ Although successful anaesthesia is reported, literature review indicate significant major adverse cardiac events (MACE) including sudden death, as frequent.² So anaesthetic management of these patients has proved to be challenging. The cardiovascular abnormality, corrected QT, and cardio-depressant actions of anaesthetic drugs need critical considerations.