Acute Presentation (Tet spell):
Two-fold mechanism to reduce right to left shunt
Reduces systemic venous return
Increases systemic vascular resistance
Done in the parent's arms or while lifting the patient onto the parents shoulders and tucking the knees underneath the chest
Reduces systemic venous return
Increases systemic vascular resistance
Avoid needle sticks if possible as crying worsens pulmonary vascular resistance and right-to-left shunt
Consider intranasal or intramuscular medications if needed
Permit child to remain with parents
Maintain calm, quiet, dim room to reduce agitation
Consider IO line as resuscitation tool
Consider intranasal or intramuscular medications if needed
Morphine 0.1-0.2mg/kg IV or IM
Goal is to ideally avoid IV placement if possible
Decreases ventilatory drive, thus decreasing systemic venous return
Caveat is decrease in vascular resistance
Intranasal Fentanyl 1.5-2 mcg/kg range 
Only one case report but IN administration may avoid the pain from a needle stick
Goal is to ideally avoid IV placement if possible
Decreases ventilatory drive, thus decreasing systemic venous return
Caveat is decrease in vascular resistance
Only one case report but IN administration may avoid the pain from a needle stick
0.25 - 1.0mg/kg IV or 3-5 mg/kg IM
Benefit of sedation without decrease in vascular resistance
Dose: 0.2mg/kg IV
Increases SVR similar to knee to chest positioning
1-2 mEq/kg IV slowly
Corrects metabolic acidosis
Reduces hyperpnea
10-20 cc/kg bolus NS initially
Improves RV filling in the context of restrictive RV physiology
Should be administered in consultation with cardiology and pediatric surgery
Relax spasm causing RV outflow obstruction
Slows HR, decrease right to left shunting
Propranolol IV 0.1-0.2mg/kg over 5min (preferred)
Alternatives when IV propranolol not available (switch to PO propranolol after)
Metoprolol IV 0.1mg/kg over 5 min, q5 min to max dose of 3x
Then may start infusion 1-5 mcg/kg/min
Esmolol 0.5mg/kg over 1 min, then 50 mcg/kg/min over 4 min
Metoprolol IV 0.1mg/kg over 5 min, q5 min to max dose of 3x
Then may start infusion 1-5 mcg/kg/min
Esmolol 0.5mg/kg over 1 min, then 50 mcg/kg/min over 4 min
Then may start infusion 1-5 mcg/kg/min
pRBCs 5-10 cc/kg IV over 5hrs
Appropriate in neonatal period only (prior to closure of ductus arteriosus)
Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension (and apnea)
Maintains the ductus (which completely seals by ~3 wks)
Side Effects:  Hypotension, Bradycardia, Seizures and Apnea
Cardiothoracic surgery to repair the defects early before significant pulmonary hypertension develops
Often staged repair, completed over several surgeries
Optimal age for correction controversial, but if elective repair, 3-11 months of age may be best
Often staged repair, completed over several surgeries
Tetralogy of Fallot
Seizure
Transposition of the great arteries
Breath-holding spell
Ebstein anomaly
Hyperventilation syndrome
Tricuspid atresia
Pregnancy 