Congenital Heart Lesions
OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction and R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
Pulmonary Artery
Right Atrium
Right Ventricle
Left Ventricle
Aorta
Left Atrium
Ductus Arteriosus
PPatent FForamen OOvale
Key Points
• Blood flows to the path of least resistance
• Pulmonary resistance < systemic resistance
• All newborns have connections – PDA– PFO
OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction and R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
Left to right shunting
• Right and left side connected
• Increased (too much) pulmonary blood
flow
• Respiratory distress/ CHF
Left to right shunt lesions
• Ventricular septal defect (VSD)
• Atrial septal defect (ASD)
• AV canal
• Patent ductus arteriosus (PDA)
Diagnostic tools
• CXR-- “wet lungs” with cardiomegaly
• EKG-- may have RVH, IRBBB (ASD), abnormal “NW” axis (AV canal), BVH (VSD)
• ABG-- high CO2 late finding; PO2 in 100% not very useful; no acidosis
OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction and R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
Left side obstruction
• Not enough blood to the body
• Hypo-perfusion, acidosis, shock
• +/- connection between right and left
Left side obstructive lesions
• Mitral valve obstruction
• Aortic valve obstruction
• Coarctation of the aorta
• Everything obstructed
– Hypoplastic left heart syndrome
Diagnostic tools
• CXR- may be normal or “wet”
• EKG- often misleading; neonate will not have LVH you would expect from an older person with AS or coarct (and hypoplast will have left forces)
• ABG- may present with profound metabolic acidosis, low CO2 (hyperventilating), PO2 may be lo or hi
OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction & R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
Cyanotic lesions
• Connection - right and left sides
• AND right side obstruction
• Decreased pulmonary blood flow
OR
• Separated systems
• Normal or increased pulmonary blood flow
Cyanotic lesions
• Right side obstructions
– Tricuspid obstruction
– Pulmonary obstruction
– Tetralogy of Fallot
• Separate systems
– Transposition of the great vessels
Diagnostic tools
• CXR- classically, “black lung fields” with “boot” (TOF) or narrow mediastinum (TGA)
• EKG- very often normal, except tricuspid atresia classically “northeast”
• ABG- these are the kids who fail the hyperoxia challenge
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OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction & R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
When is “blue” O.K.?
Mixing lesions
• Very large connection
• Key points-– What goes into the lungs comes out of the
lungs = red – What goes into the body comes out of the
body = blue
• May have right side obstruction
Mixing Lesions
• Single ventricle– Double inlet left ventricle (DILV)– Double outlet right ventricle (DORV)– Primitive ventricle– Hypoplastic right or left ventricle
• Total anomalous pulmonary venous return (TAPVR)
• Truncus arteriosus
OutlineNormal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions• Right side obstruction & R -> L shunt• Transposition
Mixing Lesions
Surgical therapy
Surgical therapy
• Repair vs. palliation
• Palliating a single ventricle - Example:
HLHS
– Stage I: Norwood and BT shunt
– Stage II: Glenn shunt
– Stage III: Fontan
Hypoplastic Left Heart Syndrome
Stage I: Norwood + BT shunt
Stage II: Glenn shunt
Stage III: Fontan
Take-home
• Congenital heart disease is not about murmurs
• Tachypnea, cyanosis, “shock” should all raise red flags
• Exam, CXR,EKG,Sats, ABG are as important as the echo!