Congenital heart Flashcards by Leslie Mancini

Cyanotic diseases

Tetralogy of Fallot
Hypoplastic left heart syndrome
Transposition of Great Vessels
Pulmonary Atresia

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Acyanotic

Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect
Coarctation of the Aorta

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cyanotic

Transposition of the Great Vessels

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Transposition of the Great Vessels

Presentation

cyanotic

Problems breathing/ cyanosis
Poor feeding

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Transposition of the Great Vessels

PE and murmur

Physical exam:
Weak pulse
Ashen or bluish skin color

Murmur: systolic

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Arterial Switch Operation

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Hypoplastic Left heart Syndrome (HLHS)

general

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HypoplasticLeft Heart Syndrome syndrome (HLHS)

presentation & murmur

Presentation:
Shock
Cyanosis
Murmur: variable ( depends assoc defects ((usu ASD))
More common in males
25% cardiac death before 7 days old

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HypoplasticLeft Heart Syndrome (HLHS)

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Pulmonary Atresia

general

Pulmonary valve did not form. No blood flow from R ventricle to the pulmonary artery
2 types:
With VSD or without
Can be detected on screening u/s in utero and then fetal ultrasound

*cyanotic
*Critical congenital disease

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Pulmonary Atresia

presentation and Tx

Presentation:
Cyanosis, blue
Murmur: depends assoc VSD/MR/TR
Hyperdynamic apical impulse
Treatment:
Rx to keep the PDA open
Usually surgery- stent PV and patch VSD

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tetralogy of Fallot

general

1)VSD
2)Pulmonary stenosis
3)RVH
4) Overriding aorta

*critical congenital disease
*cyanotic

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Tetralogy of Fallot

preentation/murmur

Presentation:
Cyanosis
Polycythemia
Agitation
Murmur: crescendo-decrescendo holosystolic LSB radiate to back

tet of fallot

Clubbing
“tet spell”
Squatting
Boot shaped heart
Nail clubbing

T of fallot

Dx and Tx

Diagnosis: echocardiogram
Treatment:
Surgery in first 4-12 months of life

VSD (vent sept defect)

general

Communication between the ventricles
4 types:
1) Conoventricular Ventricular Septal Defect
2) Perimembranous Ventricular Septal Defect
3) Inlet Ventricular Septal Defect
4) Muscular Ventricular Septal Defect

Noncyanotic
*most common of all congenital heart defect

VSD

presentation /murmur

Presentation
Weakness
Fatigue
Poor feeding
Doe
Failure to thrive ( poor weight gain)

Murmur: loud high pitched harsh holo-systolic M LLSB; does not change with position

VSD

Dx and Tx

Diagnosis: Echocardiogram
Treatment:
Closure depending on size and symptoms
Ideally wait until > 35 lbs

PDA Patent ductus arteriosus

general

ductus arteriosus fails to close after birth; oxygenated blood from the L heart can flow back to the lungs via the aorta- can lead to pulmonary HTN

See in premies
Treatment with prostaglandin synthesis inhibitors ( indomethacin)
Treatment depends on size- closure device vs ligation

Creates a L to R shunt (Eisenmenger’s syndrome)

PDA

PDA

presentation and murmur

Presentation
Weight loss with poor feeding
Pulmonary congestion
Frequent pulmonary infections

Murmur: continuous machinery murmur

Wide pulse pressure, bounding pulses
Hyperdynamic apical pulse
More likely in premies

# ASD

25% of live births Communication between the R and L atria If R to L shunting, allows deoxygenated blood to dilute oxygenated blood in the Left atrium 3 types: Ostium secundum ( most common/ 80%) Ostium primum Sinus venosus Closure indicated depending on the size/shunt Closure indicated if develop right to left shunt *suspect if cryptogenic stroke!!!

# Atrial Septal Defect (ASD) presentation and murmur

Presentation: Frequent respiratory or lung infections Difficulty breathing Tiring when feeding (infants) Shortness of breath when being active or exercising Skipped heartbeats or a sense of feeling the heartbeat Swelling of legs, feet, or stomach area Stroke Murmur: **systolic 2 LICS “ rumble”, RV heave** Work up: “bubble study” on echocardiogram

# Coarctation of Aorta

Emergency* The descending proximal thoracic aorta is narrowed

# Coarctation of Aorta presentation and murmur

Presentation: pale skin irritability heavy sweating difficulty breathing hypertension Murmur: LUSB continuous, radiates to the back Decreased pulses to lower extremities/groin Difference b/t upper and lower pulses/bp is pathognomonic**

# Coarctation of Aorta Dx and Tx

Diagnosis: Cxray: rib notching and “3”sign angiogram (gold standard) Treatment: Balloon angioplasty +/- stent vs surgical repair Will almost always have a bicuspid aortic valve *increased instance intracranial aneurysm

Side step: cryptogenic stroke

Cannot find a cause of emboli ( no clot, afib, emboli, endocarditis, cad, hypercoagulable syn ) Consider 30 day event monitor to rule out undiagnosed afib Echo/tee with bubble study to look for shunt!!!

Infant born at 32 weeks has loud continuous machinery like murmur; what is best test? what is best test? Cxray Heart cath Mri heart Echocardiogram What medication may be given? indomethacin clopidogrel Antibiotics diuretics

Echocardiogram indomethacin | (Continuous machinery like murmur think PDA)

4 yo presents pediatric clinic. Mother reports he gets winded easily. PE: lower extremities skinny, continuous systolic murmur LUSB. BP and pulses in BUE is > BLE ASD VSD PDA Coarctation of the aorta

Coarctation of the aorta

Which of the following is NOT a component of Tetralogy of Fallot? overriding aorta RVH ASD VSD

ASD

Murmur that increases with Valsalva? HOCM AS MVP AR

HCOM

# POTS Tx

Treatment: Liberalize salt, caution w position changes, compression hose, exercise Rx: metoprolol, fludrocortisone, midodrine