Table 1 Summary of the dominant hemodynamic physiologies of CCHD and their management
Dominant physiology | Pathology | Management |
|---|---|---|
Left to right shunt | AVSD, VSD, PDA, AP window | Diuretic therapy Accept saturation > 88% (usually >92%) Avoid unnecessary oxygen therapy Fluid restriction Avoid hyperventilation and alkalosis |
Obstructed pulmonary veins | Obstructed TAPVR | Immediate transport for cardiac surgery |
Cyanosis with unlimited PBF | Unobstructed TAPVR, Tricuspid atresia with no PS, Truncus arteriosus, single ventricle, DORV with no PS | Diuretic therapy Accept saturation > 75% Discharge after ensuring there is no significant PS and the PDA is almost closed Early follow-up in cardiac center |
Cyanosis with limited BPF | Pulmonary atresia, severe PS, TOF, DORV with severe PS, Tricuspid atresia with severe PS, severe Ebstein anomaly | Prostaglandin E1 infusion Accept saturation > 75% Increase the intravascular volume Vasopressors to augment pulmonary flow Transport to a cardiac center |
Parallel circulations | TGA with intact ventricular septum, DORV with malposed great arteries | Prostaglandin E1 infusion Accept saturation > 75% Evaluate the need for balloon atrial septostomy Treat pulmonary HTN aggressively if desaturated with adequate ASD. Transport to a cardiac center |
Obstructed systemic circulation | HLHS, Tricuspid atresia with malposed great arteries and small VSD, Aortic stenosis, Interrupted aortic arch, Coarctation of the aorta | Prostaglandin E1 infusion Accept saturation > 75% Avoid hyperventilation, oxygen therapy and alkalosis. Fluid restriction Utilize ventilation with higher PEEP Transport to a cardiac center |