Sedation Guidelines for Children with Complex Congenital Heart Disease

Pre-Procedure Assessment and Preparation

The American Academy of Pediatrics recommends careful pre-sedation evaluation for children with complex congenital heart disease, requiring attention to potential pulmonary vascular disease 1, 2
Children with VSD, PA, and ductal stenting are at high risk for pulmonary hypertension and may develop pulmonary hypertensive crises during sedation, according to the American Heart Association 2, 3
Fasting guidelines should be followed: clear liquids up to 2 hours, breast milk up to 4 hours, formula/solids up to 6 hours before procedure, as recommended by the American Academy of Pediatrics 4
Verify availability of resuscitation equipment including age-appropriate airway equipment and resuscitative medications, as suggested by the American Academy of Pediatrics 5, 6

Ketamine 1-2 mg/kg IV is preferred as the primary sedative agent due to its cardiovascular stability and maintenance of respiratory drive, according to the American Academy of Pediatrics 5, 7
A dedicated provider other than the one performing the procedure must continuously monitor the patient throughout sedation, as recommended by the American Academy of Pediatrics 6, 8

Continuous pulse oximetry, ECG, and non-invasive blood pressure monitoring are essential, as suggested by the American Academy of Pediatrics 5, 4
End-tidal CO2 monitoring is strongly recommended to detect early respiratory depression, according to the American Academy of Pediatrics 6, 7
Monitor for signs of pulmonary hypertensive crisis: sudden decrease in oxygen saturation, hypotension, bradycardia, as recommended by the American Heart Association 2, 3

For pulmonary hypertensive crisis: increase FiO2, provide ventilatory support, correct acidosis, and consider inhaled nitric oxide if available, as suggested by the American Heart Association 2, 3
For respiratory depression: position airway, provide supplemental oxygen, and be prepared for bag-mask ventilation, according to the American Academy of Pediatrics 6, 7
For hypotension: administer small fluid boluses (5-10 ml/kg) cautiously and have vasopressors readily available, as recommended by the American Academy of Pediatrics 5, 8

Continue monitoring until the child returns to baseline mental status and vital signs, as suggested by the American Academy of Pediatrics 4, 6
Ensure adequate pain control and anxiolysis during recovery, according to the American Academy of Pediatrics 5, 7
Provide clear discharge instructions to caregivers, including signs of complications to watch for, as recommended by the American Academy of Pediatrics 4, 8

Never use high doses of opioids in this patient population due to risk of respiratory depression, as warned by the American Academy of Pediatrics 5, 6
Do not discharge until the patient has returned to baseline mental status and vital signs, as recommended by the American Academy of Pediatrics 4, 8
Remember that children commonly progress from intended level of sedation to deeper levels; be prepared to rescue from a deeper level than intended, according to the American Academy of Pediatrics 6, 7