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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. Although successful anaesthesia is reported, literature review indicate significant major adverse cardiac events (MACE) including sudden death, as frequent. 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. Further, effect of mild to moderate sedation for interventions outside operating room in such patients are not much known. With the advancement of anaesthesia techniques and drugs, whether the cardiovascular response of such patient remains the same, continues to be a query. A 7-year-old boy, malnourished, BMI 14.61 Kg/m, a known case of WS was posted for computed tomography (CT) Aortography and Pulmonary Angiogram. The boy was having typical elfin facies (Figure-1a, 1b). His pulse rate was 105/min, regular; room air saturation 100% with normal respiration and systolic BP 80mmHg by palpation (child not cooperative). Ejection systolic murmur grade-3 at Right second intercostal space with normal S1 and S2 was noted. The child was restless with a history of developmental delay and mental retardation but was able to climb 3-4 flight of stairs without any dyspnea or syncope. The child was primarily planned to undergo circumcision, but, clinical finding directed for further cardiac evaluation. His haemoglobin, electrolytes, resting ECG, and 2-D Echocardiography was normal.

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