Congenital defects Flashcards
Acynotic defects
VSD ASD Atrioventricular canal PDA Coarctation of aorta
Cyanotic defects
5T's Tetralogy of fallot Truncus arteriosus Transposition Tricuspid atresia Total Anomalous pulmonary venous return (TAPVR)
Classification of acyanotic defect
Left to right shunt = increased pulmonary blood flow
Sxs of left to right shunt
- Tachypnea
- Hypertrophy
- Fluid in the lungs on CXR
- May see failure to thrive
Most common congenital defect
VSD
VSD murmur
Holosystolic at LLSB
VSD Physical exam findings
- Failure to thrive
- Poor growth
- Tachypnea
- Hepatomegaly
- GERD
Most common defect associated w/ VSD
Down’s syndrome
What murmer is louder; smaller or larger
Smaller (greater resistance to blood flow)
VSD diagnostic studies
ECG: LV hypertrophy
CXR: Cardiomegaly
ECHO: Location of defect, size of shunt, pressure gradient, other associated lesions
VSD Mgt
- Diuretics
- ACE inhibitors (LV unloading)
- +/- Digoxin
VSD indication for surgery
- Unmanagable heart failure
- Failure of medical mgt
- Shunt > 1.5-2
Most common type of ASD
Secundum ASD (septum primum and secundum don’t overlap)
ASD murmur
Pulmonary flow murmur in 2nd intercostal space (same place as innocent murmur so rule out)
Why is ASD not very symptomatic
Not much increase in blood flow (L&R atria about the same size)
Why is there are risk of paradoxical emboli in ASD
During valsalva the RA pressure goes high and blood goes the opposite way -> blood/clot cn travel to the brain-take care of before adolescence
What size ASD will close on its own
Sxs of ASD
usually asymptomatic +/- fatigue, palpitations, exercise intolerance
When does septation of the AV canal happen
End of 4th wk
Most common prenatal Dx of CHD
AVSD
common defect associated with AVSD
Down’s syndrome (not most common)
Complete AVSD
large VSD and ASD w/ common AV valve (develop regurg) Pulm HTN
Why should you repair complete AVSD in infancy
To prevent pulmonary vascular obstructive disease
partial AVSD
large ASD, no VSD w/ 2 separate AV valves. Norm pulm pressure
When to repair partial AVSD
18-24mos
Problems associated w/ partial AVSD
Subaortic stenosis
Left AV valve regurg
PDA murmur
Continuous mechanical murmur-hyperdynamic precordium (heard during systole & diastole) Can be heard in the back
Sxs of PDA
Cardiomegaly
Wet lungs
Bounding pulses
PDA closure
- Indomethacin/Ibuprofen
- Catheter
- Surgical (left thoracotomy)
What keeps the ductus arteriosus open
Prostaglandins (PGIs)
Stills (innocent) murmur
LSB musical/vibratory
not heard in the back, decreases w/ exp/standing.
Etiology of innocent murmur
Physiologic peripheral pulmonic stenosis
Where is innocent murmur best heard
Axilla bilaterally
Classification of cyanotic CHF
Right to left shunt
- Intra-cardiac defect/obstruction of pulmonary blood flow
- Admixture of pulmonary and systemic venous return
Is obstructive blood flow self correcting
No
Physical exam finding of cyanotic CHD
Cyanosis, bounding/absent/delayed arterial pulse, split S2 / fixed split
how much deoxygenated Hgb makes you look cyanotic
5mg/dl
When can’t you tell if a child is cyanotic
Anemia (check upper and lower extremities)
Tetralogy of fallot
Combination of heart defects that cause obstruction
Xtics of tetralogy of fallot
- RV obstruction (valvar/supravalvar stenosis)
- VSD
- Overriding aorta (straddles septum + blocks RV)
- RV hypertrophy
Tet spell
hyper-cyanotic event due to muscle spasm
Factors that precipitate Tet spell
- Crying
- Dehydration
- Anesthesia
How to fix tet spell
Increase resistance on the left side so blood can stay on the right side->pink baby
- Squatting (push baby’s legs up ton increase venous return)
- volume resuscition
- Vasoconstrictors
Truncus arteriosus
Failure of truncus to separate into aorta and pulm artery
Classification of truncus arteriosus
Right to left shunt
Large VSD
Excessive pulmonary blood flow +/- cyanosis
Genetic association of truncus
22q11 deletion (DiGeorge) in 25%
When to do closure of VSD
Within first 2wks
TAPVR
Pulm veins not getting back to LV
Classification of TAPVR
- Supracardiac (vertical vein to innominate vein)
- Cardiac (to coronary sinus, fix in first 6mo)
- Infracardiac (obstructed, drains into liver via descending vein)
Sxs of infra cardiac TA PVR
Very sick with wet lungs, cyanotic, pulm HTN EMERGENT Repair (Rule out using ECHO)
Coarctation of aorta
Juxtaductal overgrowth of tissue that closes the ductus EMERGENT!! (babies=little/no perfusion of legs, kids=blood supply to legs but weak pulse)
What do you give for coarctation
PGIs
Most common defect associated with Coarctation of aorta
Turner’s syndrome
Coarctation murmur
Systolic, LUSB
presentation of coarctation
Neonates: Extremis-Emergent
Infants: Irritability, tachypnea, poor feeding, FTT, CHFs
Decreased/absent femoral pulses
Teens: HA, nose bleed, diminished femoral pulses, unexplained HTN
Diagnostic studies for coarctation
ECG: LV hypertrophy
CXR: Cardiomegaly, rib notching (yrs)
ECHO: Difficult to visualize
CT Angio/MRA-Gold std
Coarctation mgt
Surgical (left thoracotomy)
Stent recurrences
