LAUSR

The authors have discussed Williams syndrome in detail. The risk of sudden cardiac death in patients with Williams syndrome increases manifold, and the causes are myocardial ischemia and cardiomyopathy.[2,3] In this case, the infant had preoperatively hypertension for the age which was an alarming finding but got neglected. Thorough investigation for this specific finding could have given diagnosis of the patient before elective bilateral congenital hernia reparative surgery and intraoperative death could be prevented. This child diagnosed later having supravalvular aortic stenosis which is a fixed cardiac output state. Anesthetic agents which reduce systemic vascular resistance and reflex tachycardia have detrimental effects on cardiac output, diastolic blood pressure, and coronary perfusion in such patients of fixed cardiac output state. Hence, the goals of anesthesia induction should be for maintaining systemic vascular resistance and avoidance of tachycardia. As authors could not diagnosed the disease, routine induction of anesthesia with their vasodialatory effects was not tolerated by this child.