Bernstein #2 Flashcards
4 things that obstruct systemic flow
- Aortic stenosis
- Coarctation of aorta
- Interrupted aortic arch
- Hypoplastic left heart
What is coarctation?
Narrowing of the aorta
3 coarctation tidbits.
- 98% juxtaductal
- Male to female ration 3:1
- 7% of CHD
What other heart anomaly is coarctation associated with?
Bicuspid aortic valve in 70% cases
What genetic syndrome is coarctation seen in?
Turners
Coarctation obstruct what and causes what?
LV outflow
-LV hypertrophy
What is the classic signs of coarctation?
- Diminished or absent femoral pulses
- High BP in upper extremities
- Systolic HTN of upper extremities
- Pulse discrepancy between arms
What are clinical signs of coarctation?
- Lower extremity hypoperfusion
- Acidosis
- Heart failure
- Shock
Coarctation reveals what on CXR?
- Cardiomegaly
- Rib notch
in coarctation is essential to maintain ___________ with ________.
- Ductus arteriosus
- Prostaglandin E
4 ductus arteriosus dependent diseases that obstruct systemic flow.
- Aortic Stenosis
- Coarctation
- Aortic arch interuption
- Hypoplastic left heart
What interventions are needed for systemic flow obstructions?
- Surgery within 1st days of life
- inotropes
- Mech ventilation
- diuretics w/ Qp/Qs
3 obstructions to pulmonary flow
- Pulmonary stenosis
- Tetralogy of fallot
- pulmonary atresia
T/F obstructions to pulmonary blood flow are ductal dependant and require prostaglandin.
True
Name an example of parallel circulation.
D-transposition of great arteries
What is parallel circulation?
- RV ejects into aorta
- LV ejects to lungs
During parallel circulation ______ ______ is used enlarge atrial shunt
Balloon septoplasty
What are 3 single ventricle lesions?
- tricuspid atresia
- Double inlet LV
- Unbalance AV septal defect
What is a double inlet LV?
-both atria empty into LV
What does an unbalanced AV septal defect do?
-complete mixing of systemic and pulmonary blood
Name 2 intrinsic myocardial disorders.
- Cardiomyopathy
- myocarditis
What determines the severity of a shunt?
- Size of orifice
- Outflow resistance on either side
Who needs endocarditis prophylaxis?
- Prosthetic valve
- Previous endocarditis
- Certain CHD
- Cardiac transplants w/ vavuloplathy
What CHD need endocarditis prophylaxis?
- Unrepaired cyanotic CHD including shunts and conduits
- prosthetic material for 1st 6 months
- residual defects
what antibiotics are used for endocarditis?
-Amoxicllin 50
-Ancef - 50
Clindamycin - 20
What is Kawasaki disease?
- Mucocutaneous lymphnode syndrome
- Causes vasculitis
In kawasaki disease, coronary artery dilation can cause what?
- Aneurysm
- MI
Trisomy 21 physical abnormalities.
- Small for age
- Short neck
- Low ears
- Large tongue
- Small teeth and jaw
- Narrow nsaopharynx
- Large tonsils
- Decreased muscle tone
- C spine disorders
Trisomy 21 anesthesia concerns
- OSA
- MR
- Thyroid disease
- Poor vascular access
CV defect in 50% of trisomy 21, what are they?
- AV septal
- VSD
- TOF
- PDA
What effect does anesthesia have on trisomy 21?
- Bradycardia
- Pulmonary HTN
Turner syndrome abnormalities
- X linked
- Webbed neck
- Low ears
- Small jaw
- Lymphedema
- Ovarian failure
- DM
- Liver disease
- Obesity
- Hypothyroid
Turner CV defects
- Aortic coarctation
- Bicuspid aortic valve
- HTN
What is williams syndrome?
- Chromosome 7
- Deletion of elastin gene
- Elfin faces
Williams syndrome abnormalities
- Hypercalcemia
- Hypothyroid
- MR
- Growth deficiency
- Altered neurodevelopment
- Autism
Williams syndrome CV problems
- Aortic stenosis
- Aortic coarctation
- Coronary artery stenosis
- Narrow abd and renal arteries
What must be avoided in williams syndrome anesthesia?
-Hypotension
What is the leading cause of cardiac arrest in the perioperative cardiac arrest registry?
William’s syndrome
What is DiGeorge / Velocardiofacial Syndrome
-22q11.2 deletion syndrome
What does DiGeorge cause?
- Cardiac deficits
- Thymic hypoplasia
- Cleft plaate
- hypocalcemia
What cardiac abnormalities are seen in DiGeorge?
- Conotruncal abnormalities
- Outflow tract
What type of blood do DiGeorge patients need and why?
- Irradiated
- Thymic hypoplasia
What type of CHD does noonan syndrome have?
- Pulmonary valve dysplasia
- Pulmonary stenosis
Why does Noonan need coags?
-Bleeding diathesis
What is marfans syndrome?
- Mutation of fibrillin gene
- Connective tissue protein
What is unique about marfans?
- Aortic dissection can occur at anytime?
- Arrhythmias
- Beta Blockers
- BP control
- Ventricular dilation
What type of pulmonary deformities presents with marfans?
- Chest wall deformities
- Scoliosis
- Restrictive lung disease
What is VACTERL association
- Group of non random abnormalities
- Vertebral
- Anal
- CV
- Trachesophegal
- Renal
- Limbs
An appropriate blood pressure for a neonate should be:
65/40
What is CHARGE association?
- Coloboma (eye)
- Heart defects
- Choanal atresia (blocked nose)
- Retardation of growth
- GU problems
- Ear abnormalities
How do peds compensate for chronic hypoxia?
- ↑ erythropesis
- ↑ volume
- ↑ 2,3 DPG
Why should cyanotic children not be NPO any longer than guidelines?
- Dehydration leads to ↑ viscosity
- ↑ viscosity leads to thrombi formation
HCT over ______ will impair microvascular perfusion.
65%
What 2 things increase a Right to Left shunt?
- ↓ SVR
- ↑ PVR
- Will lead to desaturation
What 2 things will increase a left to right shunt?
- ↑ SVR
- ↓ PVR
Left to right shunt does what?
- Blood is hunted to lungs
- Hypotension
- Acidosis
- ↓ coronary perfusion
CHF signs in PEDS
- Failure to thrive
- Difficult feeding
- Breathlessness
- Chest infection
- Tachycardia
- Murmur
- Hepatomegaly
- Cardiamegaly
- Pulm plethora
- Wheeze
Does the premature infant’s heart exhibit greater or lesser sensitivity to catecholamines?
- Less sensitive
- Already near max
What 2 structures does the PDA connect?
-Descending aorta to main pulmonary artery
When is PDA common?
-Preterm infants
Things needed preop for PDA repair.
- Cross match blood
- ABX
- Vit K
3 problems w/ PDA repair
- Difficult ventilation
- Hemorrhage
- ligation of aorta/pulm artery
Where are pulse ox monitors placed in PDA repair and why?
- R hand
- Lower limb
- If lower limb is lost, indication of aorta clamp
What is the most common cyanotic CHD defect?
Tetralogy of fallot
4 common things in tetraolgy of fallot?
- VSD
- Overriding aorta
- RV outflow tract obs
- RV hypertophy
What is a TET spell?
Dynamic narrowing that leads to a hypercyanotic episode.
-Increases R to L shunt
In tetralogy of fallot, the R to L shunt is dependent on what?
- R ventricular outflow tract obstruction
- VSD
In tetralogy of fallot, hypoxemia depends on what?
relationship between RVOTO and SVR
tetralogy of fallot is associated with what?
DiGeorge
Trisomy 21
In tetralogy of fallot, what causes the hypercyanotic flow?
- Crying
- Feeding
- Metabolic acidosis
- ↑ PaCo2
- Cathecholamine
- Surgical stimulation
What interventions are needed for a TET spell?
- Phenylepherine .5-1 mcg/kg
- 100% O2
- Fluid Bolus
- Sedation (fent, morphine)
- Bicarb
- Beta blocker
Why beta blockers for Tet spell?
- Relaxes infundiblar spasm
- propanolol
