peds deck 15

Question 1

T/F: with ebsteins anomaly the Tricuspid valve is always regurgitant

Answer 1

false; can be regurgitant or stenosed

Question 2

severity of ebsteins anomaly is dependent on what?

Answer 2

the degree of displacement and degree of valve dysfunction

Question 3

most older children with ebsteins anomaly are dx d/t ____________, where adults that are dx are d/t _______________

Answer 3

murmur; SVT/syncope

Question 4

pts with ebsteins anomaly are at increased risk for

Answer 4

1. paradoxical emboli through interarterial connection 2. brain abscess 3. Sudden death 4. 20% = pre-excitation syndrome/wolf-parkinson white with delta wave on ECG

Question 5

management of ebsteins anomaly

Answer 5

1. prevention and tx of complications: SBE prophylaxis with abx, diuretics, digoxin, ablation of accessory pathways 2. severely ill neonates = blalock taussing shunt 3. older children/adult = valve repair (preferred over replacement)

Question 6

complications with surgical intervention for ebsteins anomaly

Answer 6

1. complete heart block 2. persistent SVT 3. residual tricuspid regurgitation 4. prosthetic valve dysfunction

Question 7

describe the difference in transition of blood flow with birth in children born with hypoplastic left heart syndrome

Answer 7

1. decreased PVR and increased SVR (same) 2. small left ventricle is unable to adequately pump systemic blood 3. leads to back up in pulmonary circ, volume overload and pulmonary htn 4. leads to back up and hypertrophy of RV 5. will lead to RV failure and death within days

Question 8

HLHS in its “truest” from is described as:

Answer 8

1. hypoplastic left ventricle 2. hypoplastic MV 3. hypoplastic ascending aorta

Question 9

what are the three stages of surgical repair for hypoplastic left heart syndrome

Answer 9

1. norwood 2. glenn 3. fontan

Question 10

describe the 1st stage of surgical repair for HLHS (Norwood)

Answer 10

1. done within the first few days of life. 2. atrial septectomy 3. RV and single ventricle connected to reconstructed aorta (neoaorta) 4. MBT shunt or RV-PA shunt performed to privde adequate pulmonary blood flow

Question 11

which stage of surgical correction for HLHS is associated with really high mortality rate

Answer 11

stage 1: norwood

Question 12

describe stage 2 of surgical correction for HLHS (Glenn)

Answer 12

1. performed at 3-6 months 2. shunt (MBT or RV-PA) removed 3. pulmonary blood flow est via anastomosis of SVC and PA

Question 13

what is the purpose of stage 2 surgical correction for HLHS (Glenn)

Answer 13

allows remodelling of single ventricle before full fontan circulation est

Question 14

describe stage 3 surgical correction of HLHS (Fontan)

Answer 14

1. performed at 2-4 years of age 2. IVC - PA connection made 3. establishes single ventricle for systemic circulation and all blood returns to the pulmonary circulation passively (via preload)

Question 15

what will aid in successful establishment of fontan circulation

Answer 15

1. transpulmonary gradient (PVR) < 7-8 2. competent AV valve 3. preservation of Ventricular fx

Question 16

complications associated with surgical repair for HLHS

Answer 16

1. continuous decline in survivial 2. atrial arrhythmias 3. protein losing enteropathy 4. CHF 5. progressive ventricular dysfunction 6. stroke 7. ascites 8. pleural effusion 9. 75% = (school aged child) = decreased exercise capacity 10. 25% = neurologic/cognitive dysfunction

Question 17

anesthesia considerations for any pt with single ventricle d/o

Answer 17

1. maintain normal-high preload 2. normal - slightly high HR 3. normal - low PVR 4. maintain NSR 5. support ventricular fx (possibly with inotropes) 6. avoid high ventricular afterload.

Question 18

what are some of the changes that occur in a transplanted heart?

Answer 18

1. direct SNS and PNS is absent 2. altered response to baroreceptors 3. resting HR = 90-110 4. compensation for hypotension is very slow and not tolerated well

Question 19

anesthetic considerations for heart transplanted pt

Answer 19

1. strict aseptic technique 2. decreased response to indirect agents like ephedrine and dopamine 3. increase response to: CCB, BB, adenosine, and direct acting SNS (epi, isopro, dobut) 4. altered response to atropine and glycopyrolate

Question 20

indications for heart transplant (peds)

Answer 20

1. cardiomyopathy with major debility 2. end stage heart failure after surgery (i.e. failed fontan) 3. undx coarctation –> pump failure

Question 21

anesthetically, how should you manage a pt with single ventricle disorder

Answer 21

1. continue PGE to keep PDA 2. PaO2 btwn 40-45 3. spO2 = 70-80% 4. avoid high fiO2 5. maintain mild hypercarbia 6. may need inotropes

Question 22

what is a normal PaCO2 of a newborn

Answer 22

30-53

Question 23

epi dose for anaphylaxis

Answer 23

10 mcg/kg

Question 24

epi dose for hypotension/code (peds) IV

Answer 24

0.01 mg/kg of 1:10,000 (0.1 mL/kg)

Question 25

epi dose for hypotension/code (peds) down ETT

Answer 25

0.1 mg/kg of 1:1000 (0.1 mL/kg)

Question 26

newborn normal vital signs: SBP___________ DBP _________ HR __________ RR ___________

Answer 26

1. SBP = 70 2. DBP = 40 3. HR = 140 4. RR = 40-60

Question 27

normal VS for 1 year old SBP ___________ DBP ___________ HR ____________ RR ___________

Answer 27

1. SBP = 95 2. DBP = 60 3. HR = 120 4. RR = 40

Question 28

normal VS for 3 year old (SBP, DBP, HR, RR)

Answer 28

1. SBP = 100 2. DBP = 65 3. HR = 100 4. RR = 30

Question 29

normal VS for 12 year old (SBP, DBP, HR, RR)

Answer 29

1. SBP = 110 2. DBP = 70 3. HR = 80 4. RR = 20