Obstruction of pulmonary blood flow + anatomic defect (ASD/ VSD) between R & L side of heart
Difficulty of blood exiting R heart via pulmonary artery → increase R side pressure > L pressure → desaturated blood shunt R to L → desaturated blood in systemic circulation
Depends on extent of pulmonic valve stenosis & size of VSD
If VSD is large, pressures are equal in R and L ventricles. Blood is shunted in the direction of the least resistance (pulmonary or systemic vascular resistance)
PVR is > than systemic vascular resistance, shunt will be R to L
Clinical manifestations: "TET SPELLS" or "blue spells" with acute episodes of cyanosis and hypoxia, Anoxic after feeding or with crying. RISK of emboli, LOC, sudden death, seizures
Step 1 – Primary Prevention: Eradication of streptococci, Treatment of streptococcal tonsillitis / pharyngitis with Penicillin G(parenteral) – IM x 1, Penicillin V(oral) – oral x 10 days, Erythromycin (if allergic to above)