We report general management of a patient with Noonan's syndrome and hypertrophic cardiomyopathy. A 29-year-old male needed to have a radicular cyst removed. He had been treated by a pediatrician because of cyanosis on crying and heart murmur after delivery. The diagnosis of Noonan's syndrome was made from his physical features. Cardiac catheterization revealed hypertrophic cardiomyopathy and drugs had been administered. He was also an epileptic and had been receiving valproate. In this division, dental anesthesiologists managed his dental treatment six times (general anesthesia three times, monitoring twice and intravenous sedation once). During operations under general anesthesia, premature ventricular contractions were observed but no severe complications occurred.
In the most recent operation, intravenous sedation was selected because the patient was cocperative for dental treatment. On the morning of the operation, an antibiotic was given because of aortic and mitral regurgitation. At the beginning of the operation, 5mg of midazolam was given intravenously and proper sedation was performed. Prilocaine with felypressin was given as a local anesthetic. Vital signs were stable and no events occurred during the operation.
It is important to choose appropriate management for dental treatment of a patient with Noonan's syndrome, considering the severity of the heart disease, co-operation of the patient and stress of dental treatment.
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