Congenital
Heart Lesions
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
•
•
Right side obstruction and R -> L shunt
Transposition
Mixing Lesions
Surgical therapy
Ductus
Arteriosus
Aorta
Pulmonary
Artery
Left Atrium
Patent
Foramen
Ovale
Right Atrium
Left
Ventricle
Right Ventricle
Key Points
• Blood flows to the path of least
resistance
• Pulmonary resistance < systemic
resistance
• All newborns have connections
– PDA
– PFO
Outline
Normal 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
Outline
Normal 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
Outline
Normal 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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Photo - JPEG decompressor
are needed to see this picture.
Outline
Normal 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
Outline
Normal 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!