Double Outlet Right Ventricle (DORV)
Both great arteries (pulmonary and aortic) arise from the right ventricle. Always associated with a VSD.
1 - overriding aorta
2 - ventricular septal defect
Cyanotic defect resulting in delivery of inadequately oxygenated blood.
De-oxygenated blood enters the aorta from the right ventricle and is returned to the body.
VSD location can be subaortic, subpulmonic or doubly committed large VSD.
Varying degrees of pulmonary stenosis may be present.
Subaortic or subpulmonary VSD with pulmonary stenosis
These children present with histories similar to those of children with Tetralogy of Fallot.
If pulmonary oligemia is present, severe cyanosis is seen in the newborn period and the condition is recognized early.
Beyond the newborn period, cyanosis may be accompanied by hypercyanotic spells, polycythemia, and failure to thrive.
Subaortic VSD without pulmonary stenosis
These children present with histories similar to those of children with a large VSD and pulmonary hypertension.
Oxygenation is relatively normal, and patients usually present with CHF and failure to thrive.
Referral usually occurs later unless associated left heart lesions are present.
Subpulmonary VSD without pulmonary stenosis
These children present with histories similar to those of children with transposition of the great arteries.
Cyanosis varies, with oxygen saturations ranging from 40-80%.
IV, O2, Monitor
3cc/kg/hr D10W for infants under 1 year of age, D5W if over 1 year.
Management of CHF if present, consider Lasix.
Use oxygen only to relieve hypoxemia, since it is a pulmonary vasodilator and can exacerbate left-to-right shunt and CHF.
Foley catheter insertion to follow renal perfusion and urine output.
ABG to follow acidosis.