Abstract
We had a case of anesthetic management of a patient with Noonan syndrome and associated hypertrophic cardiomyopathy (HCM). A 5-month-old boy, who was diagnosed with Noonan syndrome and associated HCM, underwent the repair of pulmonary stenosis and the closure of an atrial septal defect. The HCM persisted after the cardiac surgery, and this caused simultaneous diastolic dysfunction and outlet stenosis of the left ventricle (LV).
Although HCM is one of the major causes of cardiac dysfunction, it is not an indication for surgical treatment. Under these conditions, evaluation of the LV preload was essential for perioperative management.
