Congenital heart Flashcards
Cyanotic diseases
Tetralogy of Fallot
Hypoplastic left heart syndrome
Transposition of Great Vessels
Pulmonary Atresia
Acyanotic
Acyanotic
Ventricular Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect
Coarctation of the Aorta
cyanotic
Transposition of the Great Vessels
Transposition of the Great Vessels
Presentation
cyanotic
Problems breathing/ cyanosis
Poor feeding
Transposition of the Great Vessels
PE and murmur
Physical exam:
Weak pulse
Ashen or bluish skin color
Murmur: systolic
Arterial Switch Operation
Hypoplastic Left heart Syndrome (HLHS)
general
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
HypoplasticLeft Heart Syndrome (HLHS)
Tx
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
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
tetralogy of Fallot
general
1)VSD
2)Pulmonary stenosis
3)RVH
4) Overriding aorta
*critical congenital disease
*cyanotic
Tetralogy of Fallot
preentation/murmur
Presentation:
Cyanosis
Polycythemia
Agitation
Murmur: crescendo-decrescendo holosystolic LSB radiate to back
tet of fallot
PE
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