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What is the defect in ASD?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
There is a hole in the atrial septum - ostium secundum most common.

Acyanotic, L to R shunt

Asymptomatic in childhood, gradually causes problems with RV overload, pulmonary HTN, paradoxical embolus in adults. 

Fixed splitting of S2, P site ejection murmur.  Repaired around age 5 - surgery or catheter. 
What is the defect in VSD - large?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
There is a hole in the ventricular septum.

This is acyanotic, L to R shunt.  Causes CHF due to large pressure gradient between L and R side.

The presentation is at 4-6 weeks as pulmonary vascular resistance falls. 

Needs surgical or catheter closure right away.
What is the defect in VSD - moderate?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
There is a hole in the ventricular septum. 

Acyanotic, L to R shunt.

This is asymptomatic for years, eventually causing pulmonary hypertension and eisenmenger syndrome (now it is too late).  If a child has systolic murmur, evaluate! 

Presents in young adulthood.  There is no surgery for this, as closing VSD would be fatal (no blood would get out of right heart). 

Give therapy for pulmonary hypertension:  prostanoids, Endothelin A/B receptor antagonists, PDE V inhibitors.  CCB if necessary.
What is the defect in VSD - small, muscular?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
There is a hole in the muscular septum. 

This causes an acyanotic, L to R shunt.  Murmur is present, and sometimes louder than the larger VSDs. 

No treatment - the hole usually closes spontaneously.  Should we close it to prevent endocarditis?  (controversy).
What is the defect in AV Canal Defect?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
This AV canal defect is large hole in the middle of the heart that acts as an ASD, VSD, and common AV valve (endocardial cushion defect).  No separated mitral and tc valves. 

Common in Down Syndrome patients.
Acyanotic L to R shunt. 

Gradual onset in first few weeks of life, with need for surgical repair.  Long term outcome is determined by the function of neonatal mitral valve.
What is the defect in Patent Ductus Arteriosus?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
The ductus fails to close at birth, regardless of increased PO2.  This is common in premature babies. 

Acyanotic, L to R shunt (aorta to pulm artery). 

Causes CHF if large, but may not be noticed if small. 

There will be a continuous murmur that is around S2, making it difficult to hear S2. 

Presentation occurs gradually, depending on size of ductus.  Use indocin to close ductus and monitor to see if re-open.  if won't close, use surgery or catheter closure. 
What is the defect in Tetralogy of Fallot?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
due to subpulmonic stenosis, VSD, overriding aorta, RVH. 

Most common cyanotic congenital disease.

Cyanotic, R to L shunt (due to subpulmonic stenosis). 

The presentation depends on the severity of pulmonic stenosis.  May be cyanotic at birth, or gradually develop symptoms during early life - pink tet. 
Baby gets bigger and Cardiac output increases, but stenosis doesn't dilate - more deoxygenated hemoglobin > blue baby.

Use PGE to maintain open ductus, shunt to provide pulmonary blood flow.  Need to do surgical repair, with placement of a pulmonic valve after person gets older (outgrow valve otherwise).
What is the defect in tricuspid atresia?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
In tricuspid atresia, the tricuspid valve fails to form, and the RV is small. The pulmonary valve is often abnormal. 

This must have associated Atrial Septal Defect, since there is no way for blood to get to RV. 

Cyanosis occurs, depending on the pulmonary blood flow. 

Use PGE to maintain ductus, emergent shunt placement to give pulmonary blood flow. 

Bidirectional shunt to provide pulmonary blood flow.  Do complete fontan operation later. 
What is the defect in Transposition of the Great Arteries?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
The great vessels, the aorta and pulmonary trunk, are swapped. 

Cyanotic, parallel circulation, leads to CHF.

Must have associated shunt or will not be born alive.  Willl be symptomatic upon birth. 

Give atrial septostomy, and PGE to maintain ductus. 

Then, do arterial switch operation within first few days of life. 
What is the defect in Truncus Arteriosus?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
In Truncus Arteriosus, the aorta and pulmonary artery fail to separate - there is no aorticopulmonary septum in embryogenesis.  Also, there is a large VSD.  Often have abnormal truncal valve - associated with Di George's Syndrome. (microdeletion on chrom 22).

the baby will be cyanotic (mild) and have CHF symptoms shortly after birth (will not take weeks to show). 

Do rastelli operation and other follow-up operations later in life.  No PGE is needed.   

The quality of truncal valve is important in prognosis. 

There is cyanotic, fully mixed circulation. 

What is the defect in Totally anomalous pulmonary venous return?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
The pulmonary veins connect abnormally to the right heart.  You must have an ASD to survive. 

The baby will be cyanotic and have large flow to lungs at birth.  The circulation is fully mixed. 

Their is definitive surgical repair depending on location of veins (often without sequelae/symptoms).  NO PGE is needed. 
What is the defect in hypoplastic left heart?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
In hypoplastic left heart, the left ventricle fails to form. 

This is acyanotic, L sided obstructive lesion.

This might be okay for a few hours until ductus closes, then shock and acidosis occurs.  FATAL IF NOT FIXED FAST.

This ocurs very early in life (within hours).  Use PGE to maintain ductus and complex surgery (norwood, Fontaine versus transplant). 
What is the defect in coarctaction of the aorta?  What is the Presentation?

What are some clinical findings?  What is the intervention needed?
This results from stenosis in the aorta, often near the ductus. 

Acyanotic, L sided obstructive lesion.

If the stenosis is severe, there is CHF shortly after birth.  If it is less severe, causes hypertension in upper body and hypotension in lower body. 

The presentation depends on severity.  Use PGE to maintain ductus if severe.  Use surgical repair.  If recurrent stenosis, use catheter to go back in. 
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