aortic stenosis Correct Answer pathophys:
-Left ventricular outflow tract obstruction
represent 10% of all CHD
-Valvular aortic stenosis most common (71%)
-Subvalvular stenosis second (23%). Below the aortic valve
-Supravalvar stenosis (6%). Above the aortic valve, Associated
with WILLIAMS syndrome
neonates: severe/critical AS develop
respiratory distress (due to pulmonary
edema) and poor perfusion (cap refill >2sec,
pale)
children/young adults: typically
asymptomatic but may have:
-CP with exertion
-syncope with exertion
-increased fatigability
-Sudden death reported but rare (1-2% of AS px)
PE:
-Infants: Acyanotic may have tachypnea,
respiratory distress, prolonged cap refill,
cool skin, thready pulses
-2-4/6 systolic ejection murmur at RUSB and radiates to
neck/apex
-May have diastolic decrescendo murmur if aortic insufficiency
present
,dx: LVH on ECG, CXR normal
tx: PGE1 to open ductus, balloon valvuloplasty
apgar scoring Correct Answer
atrial septal defect Correct Answer hole in atrial septum
(opening btwn R and L atrium)
L to R shunt (non cyanotic)
etiologies:
-ostium secundum MC
-ostium premium associated w/ mitral regurgitation
-sinus venous, coronary sinus
-ASD 2nd MC cause of CHD (VSD MC)
sx: asymptomatic until >30y
-infants and young children: recurrent respiratory infections,
failure to thrive, exertion dyspnea
-adolescents/adults: exertional dyspnea, easy fatiguability,
palpitations, atrial arrhythmias, syncope, HF, stroke (paroxysmal
embolus)
PE: systolic ejection crescendo-decrescendo flow murmur at
pulmonic area (LUSB). sounds like PS (functional flow
murmur)
-*widely split fixed S2* does not vary w/ respirations
-loud S1, hyper dynamic RV
dx: CXR -- cardiomegaly
,-ECG: incomplete RBB (rsR' in V1 RAD)
-*crochetage sign*: notching of peak of R wave in inferior leads
-echo gold standard
tx: spontaneous closure likely in 1st y of life. surgical if
asymptomatic (usually btwn 2-4y)
atrioventricular septal defect Correct Answer AKA - AV
canal defect or Endocardial
Cushion Defects
2% of all congenital heart defects
About 70% of children with AVSDs have
T21 (Down's)
40% of T21 patients have CHD, 50% of
these are AVSD
Simply put, the heart is missing the middle part of tissue
-Results in ostium primum ASD, inlet VSD, and a common AV
valve
sx: Develop signs similar to other L-> R shunts
-Tachypnea, prolonged feeds, diaphoresis with feeds, poor
weight gain, increased sleepiness with feeds, recurrent
pulmonary infections
-Must be vigilant in ensuring appropriate weight gain prior to
surgery
-Can be more severe if there is significant AV valve
insufficiency (leakiness)
, PE:
-Failure to thrive, tachypnea, tachycardia
-Hyperactive precordium, systolic thrill at
LLSB
-Grade 2-4/6 systolic regurgitant murmur
audibile at lower left sternal border
-May have diastolic rumble from relative AV stenosis (from
increased flow)
-Hepatomegaly or gallop rhythm if in CHF
ECG: left axis deviation
sx: medically manage until 4-6mo, surgical patch
bacterial conjunctivitis tx Correct Answer -topical antibiotics
(erythromycin, fluroquinolones (moxifloxacin), sulfonamides,
aminoglycosides)
-if contact lenses wearer, cover pseudomonas w/
fluoroquinolone or aminoglycoside
-if chlamydial or gonorrhea, admit for IV and topical antibiotics
(ceftriaxone for gonorrhea, azithromycin for chlamydial)
beck with-wiedemann syndrome Correct Answer abnormal
gene expression affecting chromosome 11p15.5 region
large for gestational age, organomegaly, macroglossia,
hypoglycemia in infancy, earlobe creases and pits, asymmetric
limbs, increased risk of hepatoblastoma and wilms tumor