For patients who have Eisenmenger syndrome (ES), perioperative risks are high even for noncardiac surgery, such as dental extractions. We report on the case history of a 38-year-old male patient with Down syndrome (DS), intellectual disability (ID), and ES. The patient was scheduled for extraction of the right maxillary second molar tooth. His physical health was poor. Following oxygenation, midazolam was administered intravenously very slowly until the optimum sedative level was obtained, with a total dosage of 3.5 mg. There were no marked changes in vital signs during the perioperative period, and the patient was discharged the same day. This case suggests that moderate or conscious sedation using midazolam for dental treatment of a patient with DS, ID, and ES was well tolerated. Several critical points are presented in this review.
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