peds deck 14 Flashcards
aortic stenosis is more common in ___________ (gender)
males
a common feature of williams syndrome is ________________ & _____________
supravalvular Ao stenosis; pulmonary stenosis
common features of williams syndrome
- supravalvular Ao stenosis and pulm stenosis 2. infantile hypercalcemia 3. elf-like features 4. mental retardation
pts with ________________ are at increased risk of sudden death under anesthesia
williams syndrome
risk factors for coaractation of aorta
- male gender (5x more common) 2. gonadal dysgenesis (turners syndrome) 3. VSD 4. PDA 5. aneurysm of circle of willis
murmur with coarctation of aorta
- harsh systolic ejection murmur over the coarc 2. if also have bicuspid aortic valve = systolic ejection click 3. will also have systolic thrill in the suprasternal notch
s/sx of coractation of aorta
- most asymptomatic if have sx… 2. htn and sx of that (HA, epistaxsis, palpitation) 3. occasional claudication 4. increased blood pressure in the RUE & decreased in the lower ext & weak fem pulses
pts with coarctation of aorta have an increased risk for _______________ if they become pregnant
dissection
surgical repiar of coarc is indicated if the gradient pressure > ________ mmHg
30
what are the ways to correct corac of aorta
- surgical repair 2. balloon dilation
what is the disadvantage of using balloon dilation for tx of coarctation of Ao
- increased risk of Ao aneurysm 2. increased risk of re-coarctation
what may you see on CXR with coarctation of Ao
- rib notch via collateral flow from intercostals 2. possible pre and post stenotic dilation of Ao 3. LVH
anesthetic considerations for any shunt that increases pulmonary blood flow (i.e. L –> R shunts)
- decreased fiO2 (< 40) 2. maintain PaCO2 (40) 3. offer high PIP 4. avoid overhydration 5. hct ~ 40 **increase PVR to prevent furtehr increase in PBF
_________________ is the most common cyanotic heart defect after infancy
tetralogy of fallot
what are the four features of tetralogy of fallot
- VSD 2. overriding Ao (overrides L and R ventricle) 3. obstruction of RVOT (subvalvular, valvular, supravalvular, pulmonary artery branches) 4. RV hypertrophy
what are the four main variants of TOF
- TOF with pulmonary stenosis - stenosis may be subvalvar, valvar, or supravalvar 2. TOF with pulmonary atresia –> hypoplastic pulmonary arteries (severe form) 3. TOF with absent pulmonary valve 4. TOF w/ ASD - rarest form
a common RVOT obstruction with TOF is ________________ obstruction which may be fixed or dynamic
subpulmonary
dynamic RVOT w/ TOF due to subpulmonary obstruction often –> _______________
tet spell
resistance to right flow across the RVOT in TOF is ___________ therefore; changes in _____________ affect the magnitude of the shunt severely
fixed; SVR
in TOF a _____________ in SVR increases R–> L shunt = more cyanosis; and _____________SVR will decrease R–> L shunt = increased saturation
decrease; increased
what is the best way to pharmacologically increase SVR with TOF to decrease R–>L shunt
phenylephrine
with TOF amount of shunt is dependent on ________________
degree of RVOT obstruction
which CHD will have a “boot like” appearance on CXR
TOF
physical exam TOF
- cyanosis 2. clubbing 3. systolic ejection murmur 4. EKG = r-axis deviation d/t RVH
the shorter and softer the murmur of pt with TOF , the _______________ severe the RVOT obstruction
more
s/sx of unrepaired TOF in an adult
- dyspnea 2. limited exercise tolerance 3. complications of chronic hypoxia (erythrocytosis, hyperviscosity, stroke, endocarditis, thromboemboli)
T/F: with TOF, if unrepaired die in childhood
TRUE
goal of surgical intervention with TOF
- closure of VSD 2. relief of RVOT obstruction accomplished via resection of hypertrophied muscle bundle & transannular patch placement
anesthesia considerations with TOF
- maintain HR and contractility 2. increase preload 3. increase SVR 4. decrease PVR 5. avoid changes to PVR:SVR (increase particularly) 6. avoid morphine, meperidine, and atracurium d/t histamine release –> decrease SVR 7. tx hypercyanosis promptly 8. avoid RV depression w/ fixed RVOT obstruction
tx of “tet spell” perioperatively
- FiO2 100% 2. administer fluids/albumin to increase intravascular volume 3. increase SVR w/ phenylephrine 4. avoid: inotropes & excess airway pressure 5. place pt in knee to chest position 6. deep anesthesia to decrease obstruction from RVOT goals: use vent to decrease PVR and also maintain-increase SVR
what is the current recommendation for TOF repiar
complete surgical repair at very young age
tx options for TOF
- complete surgical repair at very young age (current rec) 2. palliative shunt or balloon valvuoplasty in severely ill infants or when complete reapir unsuitable (will increase PBF and enlarge pulmonary artery until surgical repiar can be completed)
what are the three types of palliative shunts that are not done very often anymore, but used to be performed for TOF with pulmonary atresia
- waterson 2. potts 3. blalock taussing shunt
TOF with pulmonary atresia palliative shunt where there is side-to-side anastamosis of the ascending aorta and the right pulmonary artery
waterson
palliative shunt for TOF with pulmonary atresia, where there is side-to-side anastamosis of descending Ao to L pulmonary artery
potts
_________________ is a palliative shunt for TOF with pulmonary atresia where the left subclavian artery is end-to-side anastamosed with the L. pulmonary artery; there is also a new form called the _______________ where a dacron graft is used to connect the right subclavian artery to the right pulmonary artery
blalock-taussing shunt; modified blalock taussing shunt
if a palliative shunt for TOF w/ pulmonary atresia is needing to be performed but the coronaries cross over the RVOT or there is long segement atresia, what should be done
RV - PA conduit is placed
___________________ is a R –> L shunt, where the Ao arises from the RV and the PA arises from the LV –> complete separation of pulmonary and systemic circulation
transposition of great arteries
to survive transposition of great arteries, there must be some connection btwn circuits, what are the most common connections?
- PDA or PFO (75%) = most critical 2. ASD and/or VSD (25%)
if a patient with transposition of the great arteries has a connection between circuits via ASD or VSD you would expect _____________ cyanosis and _______________ shunt –> _________________
less; more; vol overload and LV failure
immediate management for pt with transposition of great arteries
- prostaglandin infusion to maintain PDA 2. creation of ASD 3. digoxin/diuretics to tx CHF 4. high FiO2 to decrease PBF and PVR
s/sx of transposition (include EKG/CXR finding)
- cyanosis 2. tachypnea 3. if VSD present = holosystolic murmur 4. EKG = R-axis deviation 5. CXR = cardiomegaly, increased pulmonary vasculature, and Egg shaped heart
why is the prognosis so poor for transposition of great arteries if unrepaired
- progressive hypoxemia and acidosis 2. 90% mortality by 6 months
what are the different surgical procedures for transposition of the great arteries?
- mustard senning procedure (aka baffle and ATRIAL switch) 2. Arterial switch
describe the mustard/senning procedure (atrial switch, baffle procedure) for transposition of great arteries
- atrial septum is excised to allow systemic venous blood to cross mitral into LV and pulmonary venous blood to cross tricuspid into RV 2. so the RV remains the systemic ventricle and LV remains the pulmonic
what are the disadvantages with the mustard senning procedure (baffle/atrial switch) for transposition
RV remains the systemic ventricle (which it is not equipt to do) –> RV failure, atrial arrhythmias, and/or sudden death
describe the atrial switch procedure for transposition of the great arteries
- PA and Ao are transected above the SL valves 2. Ao connected to neoaortic valve arising from LV 3. PA connected to neopulmonic valve arising from RV 4. coronaries are relocated to the neoaorta
what are the advantages of the atrial switch procedure for transposition of the great arteries
- low periop mortality 2. excellent long term outcomes.
__________________ is an abnormal tricuspid valve that sits low –> very large Right atrium and small right ventricle
ebsteins anomaly
80% of pts with ebsteins anomaly have a _________ or __________ –> R–> L shunt
ASD; PFO
