We report our experience in the general anesthesia and perioperative management of patients with intellectual disabilities (IDs) and cyanotic congenital heart disease (CCHD) for dental treatments, and discuss the indications and management of these patients in dental facilities.
Case 1:An 8-year-old ID male with Fontan circulation was scheduled for general anesthesia. The mean pulmonary artery pressure (PAP) was 11mmHg and the mean upper and lower vena cava pressure was 11mmHg. Anesthesia was slowly introduced with midazolam and sevoflurane with fentanyl, and was maintained with sevoflurane. Initially, artificial respiration was performed in a pressure-controlled ventilation (PCV) mode with 6l/min of oxygen and 1% sevoflurane, resulting in a percutaneous oxygen saturation (SpO2) of 97% and a partial pressure of end-tidal carbon dioxide (etCO2) of 50mmHg. After the ventilation volume was increased, pulmonary blood flow decreased by an increased intrathoracic pressure, resulting in a decrease in SpO2 of 88%. Then, when assisted ventilation was employed, SpO2 (92-96%) and etCO2 (37-44mmHg) were maintained under 35% oxygen.
Case 2:A 25-year-old female with ID and Fontan circulation was scheduled for general anesthesia. The mean PAP was stable at 11mmHg and pulmonary vascular resistance was in the normal range (1.48 units/m2). The awake intubation was performed with midazolam and fentanyl, and anesthesia was maintained with sevoflurane. Initially, artificial respiration was performed by PCV with 6l/min of oxygen and 1% sevoflurane, resulting in a SpO2 of 87%. After the ventilation volume was lowered, the SpO2 was 95% and the etCO2 was 75mmHg. Then, when assisted ventilation was used, SpO2 (95-100%) and etCO2 (35-43mmHg) were maintained under 35% oxygen.
Case 3:A 17-year-old male with Down syndrome who had a history of intracardiac repair for tetralogy of Fallot was scheduled for general anesthesia. Due to the high right ventricular (RV) pressure caused by pulmonary valve stenosis, general anesthesia was scheduled after the RV pressure was improved by pulmonary valve replacement. Anesthesia was slowly induced with midazolam and sevoflurane with remifentanil, and maintained using sevoflurane and remifentanil uneventfully.
When preoperative evaluation and management are performed in patients with ID after repair of CCHD, it is important to evaluate whether pulmonary hypertension is absent, pulmonary blood flow (pulmonary/systemic blood flow balance) is stable, and heart failure is mild. In anesthesia management, oxygenation can be controlled through assisted ventilation.
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