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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are needed to see this picture.

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!