Test 2 Flashcards

1
Q

What are the 4 characteristic of a transposition of the great vessels?

A
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2
Q

What 2 things maintains the ductal patency for children after birth with a TGA

A
  1. Prostaglanding E1 infusion
  2. Balloon atrial septostomy preformed urgently in neonatal period
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3
Q

What percentage does mixing take place via the PDA or VSD in a TGA patient?

A
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4
Q

What are the 5 things that occur during an arterial switch?

A
  1. The aorta, the pulmonary arteriers , and coronary arteries are disconnected form their origins
  2. The PA is moved anterior to the aorta
  3. Aorta is connected to the left ventricle
  4. Pulmonary artery is connected to the right ventricle
  5. Coronary arteries ar econnected to the neo-aortic root, most crucial part
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5
Q

What is the time frame of age when TGA surgery can take place?

A
  • Occur early at age 2-3 weeks
  • If untreated, pt will die within 1 year d/t hypoxia and heart failure
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6
Q

What are the increased risk post CPD of a TGA patient?

A
  1. Inherently poor LV
  2. Poor myocardial protection
  3. Poor coronary transference
  4. Coronary air
  5. Pulmonary HTN
  6. Avoid LA dilation (careful with fluid boluses)
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7
Q

What occurs during truncus arteriosus?

A
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8
Q

What are the 2 major components of a truncus arteriosus?

A
  1. Common arterial ourlet for aorta and PA associated with a single valve and VSD
  2. Mixed blood at arterial level with high pulmonary blood flow –> heart failure and pulmonary HTN
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9
Q

What is the first surgery for a truncus arteriosus?

A
  1. Early surgery to seperate the pulmonary from the systemic circulation
  2. Close VSD with valved conduit
    • Post-op mortaliity is high (5-25%) d/t potential truncal valve stenosis, coronary abnormalities, pulmonary hypertensive crisis
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10
Q

What are the 6 characteristics of hypoplastic left heart syndrome?

A
  1. Very small LV
  2. Mitral and aortic valve stenosis/artresia
  3. Hypoplastic aortic arch
  4. Pulmonary blood flow from LA via ASD to RA/RV
  5. Single ventricle
  6. Systemic BF from RV to a PA to aorta via PDA
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11
Q

What is the percentage of births that present as HLHS ?

A
  1. 2/10,000 Live births
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12
Q

What are the presentation for a pt with hypoplastic left heart syndrome?

A

Neonate presents with

  1. Tachypnea
  2. Tachycardia
  3. Cyanosis
  4. Systolic murmur
  5. Left side of heart is underdeveloped
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13
Q

What are the three stages of surgery for a pt with HLHS?

A
  1. Norwood
  2. Bidirectional Glenn
  3. Fontan
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14
Q

What occurs during the Norwood surgery procedure?

A
  • Reconstruction of the neo-aorta in the neonatal period
  • Branch pulmonary arteries are disconnected form the pulmonary trunk
  • Only blood supply to the lungs is provided from either a shunt from the subclavian artery (Blalock-taussig shunt) or from the right ventricle (Sanomodification)
    • Both shunts are considered passive blood flow and are dependent on pressures
    • Child remains cyanotic (SpO2 70-80s)
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15
Q

What are the intraoperative considerations for a norwood patient?

A
  • High dose opioid technique
  • Venous access often via femoral vein
    • avoid internal jugual vein d/t use for future Glenn shunt
  • Post-op mycardial dysfunction is common
  • Balancing SVR and PVR after CPB is difficult, often a combination of milrinone/epi/dopamine
  • Sternum remains oepn for several days post-op
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16
Q

What are the Bi-directional Glenn Shunt Anesthetic considerations?

A
  • Glenn operation takes place at about 6 months of age
  • During the Glenn operation, the BT shunt is taken down and a new connection is created from the SVC to the PA
  • The result is a pulmonary blood supply that is provided by systemic venous blood flow form the SVC.
  • Flow is passive and depends on pulmonary arterial pressures
  • The infant remains cyanotic with arterial saturations in the mid-80s because desaturated blood from the IVC continues to flow into the heart and systmic circulation
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17
Q

What are the HLHS Fontan- Anesthetic Considerations?

A
  • The fontan surgery involves connecting the IVC to the PA via extracardiac or intracardiac conduit to create a single ventricle circulation (Fontan)
  • The single RV pumps blood to the systemic circulation while the pulmonary blood supply is provided by passive flow from the SVC and IVC
  • It is common for a small hole (fenestration) to be created between the extracardaic conduit and the RA so that if the PVR rises, blood will be directed to the RA and allow cardaic output to be maintained - a much safer situation than a state of low CO
  • Long term- these patients need heart transplantation d/t one (right) ventricle failure over time
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18
Q

What are the post-operative considerations of an HLHS-Fontan?

A
  • PVR must remain very balanced post-operatively- careful management of lungs is important to minimize atelectasis, and nitric oxide is occasionally required
  • If an inotrope is required milrinone is a good choice due to its beneficial effects on PVR
  • Early extubation is beneficial in terms of hemodynamics
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19
Q

What are the two anesthetic considerations for a HLHS?

A
  1. Essentail to maintain the balance between PVR and SVR
  2. Oxygen is a drug and should be used cautiously in CHD patients
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20
Q

How does FiO2 have an influence on PVR?

A
  1. Hypoxia causes pulmonary vasoconstriction but causes systemic vasodilation
  2. Hypoxia and acidosis are potent stimuli for increase in PVR
  3. Reversely, high FiO2 will cause a significant decrease in PVR and potentially increase blood flow towar the lung and away from systemic circulation
21
Q

How to control SVR/PVR during anesthesia wtih HLHS?

A
  • In some HLHS children (who present for a Norwood procedure) excessive blood flow to the lungs resulting from a significantly lwo PVR and relatively high SVR steals blood from teh systemic circulation, leading to hypotension, myocardial ischemia, adn progressive acidosis.
  • It is dangerous to administer high FiO2 to an HLHS patient, because it can decrease PVR
  • When the child has the reverse occur where PVR is greater than SVR, the child will develop increasing desaurations.
  • It is difficult to manipulate the SVr and PVR predictably –> PVR is poorly understoodand vasoactive drugs work on both circulations
  • Potent volatiles reduce SVR more than PVR
22
Q

What are seven factors that can increase PVR?

A
  1. Hypoxemia
  2. Hypercapnia
  3. Acidemia
  4. Hypothermia
  5. atelectasis
  6. Transmitted positive airway pressure
  7. Stress response/stimulation/light anesthesia
23
Q

What are nine ways PVR is decreased?

A
  1. No peep
  2. Low airway pressures
  3. Lung expansion to FRC
  4. High FiO2
  5. Respiratory and metabolic alkalosis
  6. Low hematocrit
  7. Blunted stress response (deep anesthesia)
  8. Nitric oxide
  9. Vasodilators: milrinone, prostacyclin
24
Q

What are nine ways PVR is increased?

A
  1. Peep
  2. High airway pressures
  3. Atelectasis
  4. Low FiO2
  5. Respiratory and metabolic acidosis
  6. Increased hematocrit
  7. Sympathetic stimulation
  8. Direct surgical manipulation
  9. Vasoconstrictors: phenylephrine
25
Q

What factors INCrease PVR and Decrase the left to right flow?

A
  1. Hypoxia
  2. Hypercarbia
  3. Acidosis
  4. High mean airway pressures
  5. Hypothermia
  6. Catecholamine release
  7. Medications (phenylephrine, N2O, ketamine)
  8. Decreased SVR
  9. B2 agonist
  10. Neuraxial anesthesia
  11. Deep general anesthesia
26
Q

What factors decrease PVR and will INCREASE left to right flow?

A
  1. 100% FiO2
  2. Hypocarbia
  3. Alkalosis
  4. Low mean airway pressure or spontaneous ventilation
  5. Avoiding catecholamine release
  6. Medications (iNO, prostaglandins, milrinone)
  7. Increase SVR
  8. Sympathetic stimulation
  9. Alpha 1 agonist
27
Q

Hypoplastic left heart syndrome: Anesthesia considerations

A
  1. Keep neonate spontaneously breathing with FiO2 of 21% and prostaglandin E1 infusion to maintain ductal patency (to keep PDA open)
  2. Keep normal/slightly elevated PaCO2
28
Q

What is the role of prostaglandin?

A
  1. Prostaglandins are naturally occuring hormone - like substance (they circulate in the fetus’ bloodstream and keep the ductus arteriosus (DA) open.)
  2. After birth: decreased prostaglandin production plus increased oxygen tension will close the ductus arteriosus
  3. If you want to maintain a patent ductus arteriosus (PDA) –> give prostaglandin
  4. If a PDA is undesired –> give indomethacin or PDA ligation
29
Q

What are the effects of prostaglanin E1?

A
  • Prostaglandin E1 maintains patency of the ductus arteriosus in infancy when the body is supplied by the right-to-left ductal flow, as in cases of interrupted aortic arch, critical aortic stenosis and HLHS
  • Conversely, the PDA can supply pulmonary blood flow from the aorta to the lungs in lesions such as pulmonary atresia, tricuspid atresia, adn severe TOF
30
Q

What are 5 SBE prophylaxis for children with highest risk?

A
  1. Amoxicillin PO 50mg/kg
  2. Ampicillin IM/IV 50mg/kg
  3. Cefazolin IM/IV 50 mg/kg
  4. Cetriaxone IM/IV 50 mg/kg
  5. If pateint is allergic to PCN/Ampicillin
    • Clindamycin IV 20mg/kg
31
Q

Avoid Bubbles in IV fluids

A
  • In all children with right to left shunts, there is a potential for these bubbles to be shunted directly into the systemic circulation (Cerebral –>stroke)
  • Even pts wtih left to right shunts can transiently reverse their shunts during coughing or valsalva maneuver, when the normal transarterial pressure gradient is reversed. AVOID bubbles
32
Q

Sevoflurane

A
  • Induction agent of choice –> 1.5 MAC can cause slowing of HR and resp depression
  • Congenital heart disease (CHD) pt responds to slow HR with reduced CO2, hypoventilation, HYpercarbia &Hypoxia, and a rise in PVR
  • SEVO causes mild decrease in SVR
  • Mild to moderate CHD pts tolerate careful induction of SEVO
  • Young infants with severe CHD: IV induction
33
Q

Isoflurane

A

Similar characteristics like Sevo, however pungent odor (not an inhalation induction agent) higher incidence of laryngospasms

34
Q

Halothane

A
  • not available in the USA, but found in other countries
  • Very rapid uptake of Halo, however, Halo can cause significant hypotension, arrhythmias, and bradycardia
35
Q

N2O

A
  • 70% for induciton wtih Sevo is good
  • Avoid N20 as maintenance in CHD patients, because of risk of intravasulcar air emboli and the potential of increase in PVR
  • N20 is reported to incrase PVR in adults, however, ess than 50% N20 has little effect on PVR in infants
  • N2O may expand microbubbles and macrobubbles, thu increasing obstruction to blood flow in arteries and capillaries
  • N2O mildly decreases CO in infants and should be avoided in children with limited pulm blood flow, pulm HTN and depressed myocardial function
36
Q

Ketamine

A
  • Good analgesic agent which increases HR, BP, and Co by stimulating the release of endogenous catecholamines
  • Poor choice if tachycardia is undesired (Aortic Stenosis) or where catecholamine stimulation is already maximized (severe cardiomyopathy)
  • IV dose: 1-2 mg/kg
  • Ketamine is thought to mildly increase PVR in CHD children
37
Q

Etomidate

A
  • Short- acting induction drug has favorable hemodynamic profile with little effect on BP, HR, or CO
  • Increased mortality with continuous etomidate infusion r/t adrenal supression
  • Inhibition of steroid synthesis can occur also after single dose.
38
Q

Propofol

A
  • Induction causes decrease in SVR, BP, and CO wtih variable effect on HR
  • Although propofol can be used safely in children with CHD, it is generally avoided in severe CHD children because of its effect on SVR and and BP
  • AVOID propofl in patients with fixed CO (severe aortic or mitral stenosis) severe hypotension
  • Propofol/dexmedetomidine infusion during CPB (cardiopulm bypass) to reduce risk of awareness
39
Q

Fentanyl

A
  • In neonate and infants, the use of high-dose opioid anesthesia provides excellent hemodynamic stability, with suppression of the hormonal and metabolic stress response, however, pt probably remains intubated
  • Often used combination: high-dose fentanyl, midazolam, paralytics with low Sevo/Isu
  • Sufentanil: 5-10 times more potent than fentanyl, has a large margin of safety
40
Q

Inhaled Nitric Oxide NOi

A
  • VERY specific pulmonary vasodilator with minimal effect on systemic circulation
  • Acts on guanylate cyclase to produce pulm vascular smooth muscle relaxation
  • In Cath lab, inhaled nitric oxide is used to assess reactivity of pulmonary vasuclarture to vasodilation in children with pulmonary hypertension versus fixed pulmonary vascular obstructive disease. It facilitates operative planning and management.
  • NOi is administered in 1-80 ppm usually 20-40
  • Blood methhemoglobin concentration needs to be check in long term use
41
Q

Flolan

A
  • Naturally occurring prostaglandin
  • Potent vasodilator (SVR and PVR)
    • Tx for primary pulm HTN (PPH)
  • Bronchodilation
  • Inhibition of platelet aggregation
  • Rapid metabolism via enzymatic degredation
  • HL of 6 minutes
42
Q

What are the common side effects of flolan?

A
  1. Flushing
  2. Headache
  3. N/V
  4. Hypotension
43
Q

What are some contraindications of Flolan?

A
  • CHF d/t severe LV systolic dysfunction
44
Q

What are the units that Flolan is measure by?

A

ng/kg/min

45
Q

What occurs if Flolan is turned off quickly?

A

Rebound pulmonary HTN

46
Q

The pediatric pateint is scheduled for a radiofrequency ablation of an aberrant conduction pathway. What is a general anesthetic typically required for this scenario?

A
  • Radiofrequency ablation is a nonsurgical approach designed to eliminate atrail or ventricular re-entrant tachyarrhythmias. The technique requires mapping and precision ablation of the aberrant pathway, using radiofrequency ablation cathetier
  • During the ablation, unexpected movement may result in catheter dislodgement and damage to normal conducting tissue; therefore, general anesthesia is usaully required in younger children
  • Anesthetic agents and technique should be chosen to maintain circulating catecholamines, adn avoid suppression of arrhythmogenesis for identifying of the aberrant pathway.
47
Q

Anesthesia for cardiac electrophysiology study and ablation in the EP lab

A
  • The electrophysiology (EP Lab) study is a unique situation in which arrhythmias are sought and sometimes even provoked so that they may be eleiminated via ablation
  • Isoproterenol is often used to introduce tachyarrhythmias during mapping
  • Discuss with your MDA and EP physician what medications are appropriate for electrophysiology study
    • Avoid Lidocaine or medicaitons that slow tachyarrhythmias– opioids, volatiles, dex
    • Inhalation okay but convert to propofol infusion
  • Avoid Phenylephrine treat wtih fluids, and vasopressin
48
Q

Dextrocardia and how to place EKG leads properly

A
  • Dextrocardia is a condition(birth defect) where the heart is located on teh right side of hte body, as opposed to the left.
  • Patient often undiagnosed until they receive their first chest x-ray or ECG
  • When you encounter patient patient wtih dextrocardia, it is important to place ecg leads backwards
  • If the leads are placed in traditional location, ecg will show inverted p and t waves
49
Q
A