Congenital Heart Disease (Hypoplastic Left Heart) - Dr. MacNeill

BASICS:

• Most common ductal dependent lesions - coarct, interrupted aortic arch, critical AS, hypoplastic left heart syndrome 
• 30% of critical cardiac defects are initially discharge and will present to ED! 
• Single ventricle heart: Blood must flow through ASD and PDA. Failure occurs when PDA closure begins!

Initial Management: 

• Prostaglandins. 
• Minimize oxygen; O2 will dilate pulmonary vasculature and reduce cardiac output.  
• Minimize fluids.  
• Intubate and minimize oxygen.
  

Stages of repair (Please look at the pictures):

1:Norwood: RV redirected to aorta (systemic).  Shunt placed from RV into pulmonary artery as well (Sano shunt).  Post norwood, patient highly fluid sensitive and high risk for clotting

2: Bidirectional Glenn (4-6 months): Connect SVC to pulmonary artery, disconnect RV from PA.

3: Fontan (1.5 - 4 yrs): Connect IVC to PA.  Fenestrate IVC into the RV as pressure relief for the pulmonary artery.  At this point pt is VERY fluid dependent, venous return is crucial for cardiac output.

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