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N935 Advanced Pathophysiology II > Peds Congenital Hearts and Things > Flashcards

Peds Congenital Hearts and Things Flashcards

1
Q

causes of congenital heart defects

A
  • chromosome abnormalities
  • single-gene abnormalities
  • conditions during pregnancy that affect the baby
  • combination of genetic and environment problems
  • unknown causes (idiopathic)
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2
Q

diagnosis of congenital heart defect

A
  • in utero
  • found with newborn physical
  • ECHO, EKG, CXR
  • cardiac cath, cMRI, CT, TEE, Holter recording
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3
Q

preoperative eval for congenital heart defect

A
  • heart murmur
  • functional status, growth and development (are they meeting milestones?)
  • review most recent echo/labs/tests
  • children with history of CHF, cyanosis, pulmonary HTN, and young age are at a potentially higher risk
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4
Q

characteristics of fetal circulation

A
  • high PVR
  • low SVR
  • most oxygenated blood from umbilical vein shunts across the ductus venosis and foramen ovale to perfuse the heart and brain
  • Hgb F has P50 of 19 mmHg and greater affinity for oxygen than Hgb A
  • fetal pH is 7.25-7.35 (slightly acidic)
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5
Q

L to R shunts

A
  • connects arterial and venous circulation resulting in increased pulmonary blood flow
  • pulmonary overcirculation
  • leads to an increase in RV preload because a large amount of LV output bypasses the systemic circulation, enters the lungs and rapidly returns to the L side of the heart
  • pink lesions
  • PDA, ASD, VSD
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6
Q

R to L shunts

A
  • venous blood ejected systemically
  • decreased pulmonary blood flow and patients are cyanotic
  • blue lesions
  • ASD or VSD with pulm HTN, TET during TET spell
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7
Q

obstructive lesions

A
  • prevent ventricular flow from either side of the heart
  • decrease cardiac output
  • coarctation of the aorta, aortic stenosis
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8
Q

mixed or cyanotic lesions

A
  • mixing of venous and arterial blood
  • HLHS
  • these lesions can also lead to pulmonary over-circulation and CHR
  • occur when a functional single ventricle ejects the mixed systemic and pulmonary venous return
  • the patients are cyanotic and often dependent on the PDA at birth
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9
Q

Eisenmenger’s syndrome

A
  • when large VSDs are uncorrected, the resulting pulmonary HTN can reverse the shunting of blood across the defect
  • the previously L to R shunt becomes R to L because of Pulm HTN
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10
Q

Qp

A
  • total pulmonary blood flow

- sum of effective pulmonary blood flow and recirculated pulmonary blood flow

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11
Q

Qs

A
  • total systemic blood flow

- sum of effective systemic blood flow and recirculated systemic blood flow

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12
Q

single ventricle physiology

A
  • complete mixing of pulmonary and systemic venous blood at the atrial or ventricle level
  • blood then equally distributed out to both the systemic and pulmonary beds
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13
Q

three things are true in single-ventricle physiology

A
  • ventricular output is the sum of pulmonary blood flow (Qp) and systemic blood flow (Qs)
  • distribution of systemic and pulmonary blood flow is dependent on the relative resistances to flow (both intra and extracardiac) into the two parallel circuits
  • oxygen saturations are the same in the aorta and the pulmonary artery
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14
Q

what is normal Qp/Qs?

A
  • 1:1 which has equal RV and LV output

- pulmonary blood flow is equal to systemic blood flow

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15
Q

what is Qp/Qs?

A

-a ratio of estimated pulmonary to systemic blood flow that is useful in determining over circulation to the pulmonary system or LV workload

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16
Q

formula for Qp/Qs

A

Qp/Qs = SaO2 - SvO2/SpvO2 - SpaO2

  • SaO2 = aortic O2 sat
  • SvO2 = SVC O2 sat
  • SpvO2 = pulmonary vein O2 sat
  • SpaO2 = pulmonary artery O2 sat
  • derivation of Fick’s law
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17
Q

how is Qp/Qs measured

A

cardiac cath, measure oxygen saturations in all four of these areas to calculate

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18
Q

shortcut for Qp/Qs + four assumptions made

A
  • the patient is breathing room air and pulmonary venous blood is fully saturated
  • oxygen consumption is normal, resulting in a SvO2 of 25-30% less than SaO2
  • the patient is NOT severely anemic (has a normal SVC O2 saturation)
  • complete mixing results in aortic and pulmonary artery O2 saturations being equal
  • most cases = assumptions valid and allow a rapid determination of Qp/Qs based on SpO2 alone
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19
Q

Qp/Qs <1

A
  • shunt is right to left

- patient will be cyanotic

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20
Q

Qp/Qs 1-2

A
  • shunt is minimally L to R

- patient will be asymptomatic

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21
Q

Qp/Qs 2-3

A
  • shut is moderate L to R

- mild symptoms of CHF

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22
Q

Qp/Qs >3

A
  • shunt is LARGE L to R

- severe symptoms of CHF

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23
Q

ASD

A
  • atrial septal defect
  • as many as 1 in 5 healthy adults still have a PFO
  • often asymptomatic and discovered incidentally (murmur)
  • large defect left untreated can cause R sided volume overload (Qp/Qs >2) with RA and RV dilation and increased pulmonary blood flow
  • repair can be closure device in cath lab or surgery
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24
Q

VSD

A
  • ventricular septal defect
  • most common congential defect in children
  • leads to pulmonary overcirculation due to L to R shunting in isolated lesion
  • large defect –> equal pressure in both ventricles –> PVR 1/6 SVR –> so more pulmonary blood flow –> CHF –> damage to pulm vascular bed
  • as PVR falls in firth months of life, flow across the VSD can increase GREATLY (Qp/Qs >3, meaning the L heart has to pump 3xs normal volume to meet the usual systemic demands)
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25
Q

restrictive VSD

A

small size and limited pulmonary over circulation

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26
Q

unrestrictive VSD

A

large flow across the septum with balance between SVR and PVR

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27
Q

indications for surgery with VSD

A
  • poor feeding
  • reduced weight gain
  • increased in incidence of respiratory infection
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28
Q

what is at risk during a VSD repair

A

-conduction system because it runs along the ventricular septum

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29
Q

PDA

A
  • patent ductus arteriosus
  • leftover fetal artery connection between the aorta and pulmonary artery
  • unrestricted PDA will have significant L to R shunting
  • significant diastolic runoff into pulmonary circulation lowers systemic DBP, which compromises distal and coronary perfusion
  • closure - commonly done with cardiac cath (coil)
  • surgical closure = L thoracic approach, closed by suture tie or metal clip
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30
Q

CAVC

A
  • complete atrio-ventricular canal
  • free communication between all four chambers of the heart
  • located where the atrial septum joints the ventricular septum; involves atria, ventricles, tricuspid and mitral valves
  • result is formation of a single large valve; the common AV valve; often regurgitant
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31
Q

greatest percentage of kids with CAVC

A

down syndrome

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32
Q

surgical repair of CAVC

A
  • septum patch and new valves
  • usually done < 6 months of age before pulmonary vascular changes develop
  • problems can be residual septal defects, AV valve regurg, post-op pulmonary reactivity (esp if high Qp/Qs prior to surgery) and conduction system damage
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33
Q

Coarctation

A
  • narrowing in the aorta commonly occurring immediately distal to the origin of the L subclavian artery
  • most often located near ductus arteriosus
  • proximal to ductus = pre ductal; distal to the ductas = post ductal
  • frequently associated with bicuspid aortic valve
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34
Q

critical coarc

A
  • circulatory collapse
  • shock
  • acidosis
  • because POOR distal perfusion
  • PGE1 (prostin) started to reopen ductus and distal perfusion remains ductal dependent until surgery
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35
Q

coarc surgical repairs

A
  • resection and end to end anastamosis
  • suclavian flap = subclavian artery used as a flap to enlarge the constricted part of the aorta; bad thing = lose the subclavian to the L arm so needs to be perfused by collaterals
  • balloon angioplasty (cath lab)
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36
Q

coarc presentation

A
  • upper extremity HTN
  • decreased lower extremity pulses
  • LVH
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37
Q

where is BP measured in coarc

A
  • R arm

- this is because the aortic cross clamp will be proximal to the L subclavian

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38
Q

pulmonary valve stenosis

A
  • narrowing of pulmonary valve that causes the RV to work harder to pump blood past the blockage
  • symptoms depend on the severity of obstruction
  • often treated with balloon dilation
  • usually part of other complex lesions
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39
Q

aortic valve stenosis

A
  • narrowing of aortic valve that causes LV to work harder to pump blood past the blockage
  • severe AS in utero may impair LV development
  • balloon dilation is an option
  • valve replacement at young age may require revisions over time
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40
Q

Ross Procedure

A
  • performed on patients with aortic stenosis as alternative to prosthetic valve replacement
  • diseased aortic root resected and the patient’s own pulmonary valve root is excised and implanted into the aortic position
  • coronary arteries re-implanted into the neo-aortic root
  • RV to PA conduit and valve made with cadaveric tissue
  • RV to PA connection may require revision over time but provides better long term solution to the aortic valve
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41
Q

advantages of Ross procedure

A
  • freedom from long-term anticoagulation

- valve grows as the patient grows

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42
Q

disadvantages of Ross procedure

A

-single valve disease (aortic) is treated with two valve procedure

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43
Q

BTT shunt (formerly BT shunt)

A
  • Blalock-Taussig-Thomas Shunt [Helen Taussig peds cardiologist, Alfred Blalock peds surg, and Vivien Thomas surgical assistant]
  • operation to create a type of systemic to pulmonary shunt
  • important uses in certain types of cyanotic lesions to get some blood flow to the lungs
  • crucial palliation prior to complete repair later in life
44
Q

classic BTS

A
  • subclavian artery is divided and directely anastomosed to the ipsilateral pulmonary artery
  • allows patient’s own subclavian artery to grow so no need for revision
  • pulses in ipsilateral arm will be decreased or non-palpable
  • prudent to expect classic BTS in older adult survivors of CHD
45
Q

modified BTS (MBTS)

A
  • synthetic shunt between the subclavian artery and PA
  • ipsilateral arm reflects true pressure and available for art-line placement
  • artifical material will not grow with the patient
  • hypotension leads to SLUGGISH flow and possible thrombosis which can be critical
46
Q

when was tetralogy of fallot first described

A

1888

47
Q

what are the four key features of tetralogy of fallot

A
  • VSD
  • RVOT (r ventricular outflow tract obstruction)
  • overriding aorta (aorta lies directly over VSD)
  • RV hypertrophy (secondary to pressure overload)
48
Q

when is TOF repair done?

A
  • usually in first 6 months of life

- however if neonate too small can palliate with BTS

49
Q

what determines the degree of cyanosis in TOF?

A
  • limitation of pulmonary blood flow

- magnitude of ventricular level R to L shunting

50
Q

hypercyanotic spell or TET spell

A

-acute dynamic increase in pulmonary outflow tract obstruction (spasm) may result in intensely cyanotic episode due to R to L shunting

51
Q

causes of TET spell

A
  • crying
  • feeding
  • acidosis
  • catecholamines
  • surgical stimulation
52
Q

treatment TET spell

A
  • increase SVR to relax the spasm
  • child will squat to increase afterload (the position increases SVR, reduces HR, and reduces R to L shunt across the VSD)
  • anesthetic treatment includes 100% FiO2, sedation, fluid, beta blocker, alpha agonist (to increase afterload and slow down the HR)
53
Q

TOF repair

A
  • closure of VSD with patch
  • removal of some thickened muscle to relieve RVOTO
  • eliminates intracardiac shunting at the ventricular level (so no more cyanosis) and addresses the RVOTO
  • may also include enlarging the L and R pulmonary arteries
54
Q

anesthesia for TOF repair

A
  • avoid Tet spell
  • generous premedication
  • sufficient anesthesia/analgesia
  • avoid reductions in SVR
  • RV output is limiting factor on overall CO
  • treat tet spell quickly with phenyl if necessary
55
Q

treatment for TET spell intraoperatively

A
  • 100% oxygen
  • knees to chest
  • fluid bolus
  • hyperventilation
  • sedation
  • esmolol (0.5 mg/kg IV) or propranolol (0.1-0.3 mg/kg IV)
  • phenylephrine 1-10 mcg/kg IV
56
Q

Post TOF repair

A
  • hypertrophied RV has poor compliance which is worse in immediate post op period due to R ventriculotomy (must maintain adequate filling)
  • PFO or small ASD created that becomes a pop off if R sided pressures increase; so CO is maintained at expense of modest systemic desat
  • over time, tricuspid valve may become incompetnet leading to RV overload and ventricular ectopy
  • conduction system may be damaged in the repair
  • RV decompensation over time due to free pulmonary insufficiency in trans-annular patch repair
57
Q

HLHS

A
  • Hypoplastic Left Heart Syndrome
  • multitude of defects with the common denominator being under development of the L side of the heart
  • results in single ventricle physiology AND complete mixing of systemic and pulmonary circulation
  • expected oxygen saturation 75-80%
58
Q

HLHS at birth

A
  • RV provides pulmonary blood flow
  • systemic blood flow is from the PA via the PDA (ductal dependent)
  • if the PDA closes the neonate will present in shock due to severely reduced systemic perfusion
  • most are diagnosed in utero and PGE1 is started to maintain ductal patency
59
Q

three surgical palliative operations for HLHS

A
  • stage 1 = norwood (soon after birth)
  • stage 2 = bidirectional glenn (4-12 months)
  • stage 3 = fontan (1.5-3 years old)
60
Q

what is the goal of palliation for HLHS

A

to separate the pulmonary and systemic circulations

61
Q

norwood with shunt

A
  • connection between systemic to pulmonary circulation
  • atrial septectomy and creation of ONE common atrium
  • reconstruction of PA to aortic arch (neo-aorta)
  • ligation of PDA
  • establish pathway for blood flow to the lungs with BTS/MBTS/Sano shunt
62
Q

what oxygen sats will the patient have after a norwood?

A

75-80%
SpO2 > 85% excessive pulmonary blood flow
SpO2 < 70% inadequate pulmonary blood flow

63
Q

sano shunt

A
  • gore-tex graft from RV to pulmonary artery
  • provides for better pulmonary perfusion
  • downside = leaves scar tissue in the RV
64
Q

bidirectional glenn

A
  • direct anastomosis of SVC and a pulmonary artery branch
  • ligation of shunt
  • bidirectional = blood flow to both the R and L pulmonary arteries
  • requires LOW PVR and blood flow is passive
  • maintain adequate volume and low PVR
  • expected arterial oxygen sat is 75-85% (IVC venous blood continues to flow into the heart and therefore systemic circulation hence the lower sat)
65
Q

fontan procedure

A
  • inferior vena cava connected to the pulmonary vaculature
  • allows for passive blood flow from IVC to lungs while bypassing the heart
  • completes the separation of the pulmonary and systemic circulations
  • expected art oxygen sat = 88-93% (coronary sinus is NOT included in fontan anastomosis)
66
Q

extra-cardiac fontan

A

conduit sutured from IVC to pulmonary circulation

67
Q

lateral tunnel fontan

A

baffle within the RA directs IVC blood into the pulmonary circulation

68
Q

fenestrated fontan

A

allows for a pop-off hole in the RA from the conduit or tunnel

69
Q

HLHS additional considerations

A
  • prior to stage 1, ductal dependent (PDA must be kept open with prostin)
  • restrict excessive pulmonary blood flow
  • higher than expected O2 sat may mean inadequate systemic perfusion and pulmonary overload (consider NIRS)
  • patient may need inotropes (dopa, epi)
  • minimize myocardial depression
  • prevent and treat pulmonary HTN crisis
70
Q

how can you restrict excessive pulmonary blood flow in HLHS?

A
  • allow mild hypercarbia (PCO2 45-55 mmHg)
  • allow low oxygen concentrations
  • use PEEP
71
Q

chronic fontan complications

A
  • dysrhytmias (frequent due to elevated atrial pressure and atrial suture lines)
  • protein losing enteropathy (poorly understood development of hypoalbuminemia despite normal renal and hepatic fxn)
  • thrombosis (dysrhythmias that cause venous stasis or sluggish flow)
72
Q

s/s of phtn crisis

A
  • desaturation
  • bradycardia
  • systemic hypotension
73
Q

known factors to increase pulmonary vascular tone

A
  • hypoxemia and use of <30% FiO2
  • hypercarbia and acidosis
  • hypothermia
  • atelectasis
  • transmitted positive pressure & PEEP
  • stress response/stimulation/light anesthesia
74
Q

known factors to decrease pulmonary vascular resistance

A
  • increasing oxygen to 100%
  • hyperventilation
  • potent inhalation agents reduce SVR more than PVR
  • nitric oxide
75
Q

nitric oxide

A
  • powerful smooth muscle vasodilator with a short half life
  • currently used in neonates to promote capillary and pulmonary dilation to treat pulmonary HTN
  • available in concentrations 100 ppm and 800 ppm
  • over dose = methemoglobin and pulmonary toxicity
76
Q

MOA of nitric oxide

A
  • stimulates guanylate cyclase which leads to formation of cyclic-GMP
  • cyclic GMP activates protein kinase G, which causes reuptake of calcium
  • fall in concentration of calcium stops the MLCK cross bridge cycle and leads to relaxation of smooth muscle
77
Q

Subacute bacterial endocarditis (SBE)

A
  • infection caused by bacteria that enter the bloodstream and settle in the heart lining, a heart valve, or blood vessel
  • IE is uncommon, but children with uncorrected CHD are at increased risk
  • certain procedures performed on these patients require antibiotic prophylaxis
78
Q

most common cause of SBE

A

stphylococcus aureus

79
Q

most commonly used antibiotics for SBE prophylaxis

A
  • cefazolin 50 mg/kg IV

- cetriaxone 50 mg/kg IV

80
Q

what agent is recommended if penicillin allergy

A
  • has to be a true allergy
  • doxycycline 4.4 mg/kg
  • NOT clindamycin (associated with higher complications like death from c-diff infection)
81
Q

when should abx for SBE prophylaxis be administered?

A

30-60 minutes prior to the procedure

82
Q

what HIGH risk patients is SBE prophylaxis suggested for?

A
  • prosthetic cardiac valve or material
  • previous or recurrent infective endocarditis
  • congenital heart disease (unrepaired, cyanotic, palliative shunts; within 6 months following a complete repair; repair with residual defect at site of prosthetic material; certain types of conduit/valve placements)
  • heart transplant with developed valvopathy
83
Q

procedures requiring prophylaxis if patient is in the HIGH RISK category

A
  • dental procedures that involve manipulation of gingival tissue, perforation of oral mucosa, or teeth extractions/drainage of an abscess
  • respiratory tract procedures involving biopsy/incision
  • procedures on infected skin and msk tissue
  • cardiac surgery
84
Q

prophylaxis NOT recommended for these procedures even if HIGH risk

A
  • GI (if not ongoing GI infection)
  • GU
  • routine dental cleaning
  • brochoscopy without biopsies
85
Q

Down Syndrome

A
  • trisomy 21
  • chromosomal disease with distinct facies, significant airway and cardiac findings with varying degrees of intellectual disability
86
Q

Down Syndrome HEENT/airway

A
  • brushfield spots on iris
  • upslanting eyes
  • narrow nasopharynx
  • small ears
  • macroglossia
  • pharyngeal hypotonia
  • high-arched palate
  • tonsil and adenoid hypertrophy
  • micrognathia
  • short broad neck
  • small trachea
87
Q

Down Syndrome chest

A
  • chronic upper airway obstruction with hypoventilation and OSA
  • recurrent pulmonary infections
88
Q

Down Syndrome CV

A
  • 1/2 have CHD –> ASD, VSD, AV canal, PDA, TOF

- L to R shunting may lead to pulm HTN and pulm vascular disease

89
Q

Down Syndrome neuromuscular

A
  • hypotonia
  • dementia + parkinson’s in older adults
  • intellectual decline may occur with age
90
Q

Down Syndrome ortho

A
  • joint laxity
  • alantoaxial cervical instability (7-36%)
  • short stubby hands
  • single horizontal palmar crease
91
Q

Down Syndrome GI/GU

A
  • congenital duodenal atresia

- increased incidence of Hirschsprung

92
Q

Down Syndrome other

A

-increased incidence of leukemias, hypothyroidism, and antithyroid antibodies

93
Q

anesthesia for patients with Down Syndrome

A
  • assess airway CV and ROS
  • assess for alantoaxial instability
  • assess for OSA
  • CHD - SBE prophylaxis
  • prone to bradycardia on induction
  • challenging vascular access
  • downsize ETT because subglottic stenosis
  • DD varies
  • hypothyroid
  • postop stridor and respiratory complications common
94
Q

DiGeorge Syndrome

A
  • 22q11 micro deletion
  • gene involved in the developmental process and includes defects in the development of the thymus, parathyroid, and great vessels
  • has been associated with prenatal exposure to alcohol and accutane
  • M&M associated with cardiac defects, T cell immunodeficiency, and seizures r/t hypocalcemia
95
Q

DiGeorge Syndrome S/S

A
  • micrognathia
  • small mouth opening
  • short trachea
  • conotruncal cardiac defects
  • hypocalcemia
96
Q

how can immune disorder with DiGeorge be treated?

A

thymus transplant

97
Q

anesthesia for DiGeorge

A
  • micrognathia may mean difficult intubation
  • short trachea may lead to endobronchial intubation
  • choanal atresia precludes nasal trumpets or nasal intubation
  • all blood products must be irradiated to kill leukocytes which can cause GVHD
  • careful asepsis
  • calcium level MUST be evaluated
  • parathyroid dysfunction –> SIGNIFICANT hypocalcemia
  • CHD = SBE prophylaxis
98
Q

williams syndrome

A
  • deletion of chromosome 7

- first described in 1961

99
Q

williams syndrome clinical features

A
  • elfin facies
  • small teeth
  • vocal cord paralysis
  • mild mental disability
  • cerebral artery stenosis with ischemic events
  • HTN
  • abdominal aortic coarc
  • cocktail party personality
100
Q

williams syndrome CV

A
  • stenotic lesions at multiple levels possible –> valvar pulmonary stenosis, branch pulmonary stenosis, aortic stenosis, supravalvular aortic stenosis with coronary artery stenosis (impeded flow to coronaries)
  • RISK OF SUDDEN DEATH due to severe myocardial ischemia, LV dysfunction, ventricular arrhythmias
101
Q

williams syndrome anesthetic considerations

A
  • risk of sudden death outside of hospital as well as during cardiac cath and during anesthesia (thought to be due to severity of vascular stenosis and valve stenosis)
  • baseline EKG and ECHO
  • prepare for ECMO
  • SBE prophylaxis
102
Q

noonan syndrome

A
  • autosomal dominant
  • characterized by…
  • hypertelorism (increased distance between orbits)
  • mircognathia
  • short stature
  • pectus excavatum/carinatum
  • bleeding diasthesis
  • CHD
103
Q

noonan syndrome anesthetic considerations

A
  • possible difficult intubation, usually less marked with age
  • difficult PIV placement if there is significant edema
  • chest deformities may lead to decreased lung function
  • bleeding diathesis may increase amt of periop bleeding
  • renal impairment may affect metabolism of renally excreted drugs
  • spinal abnormalities may make epidural catheter placement difficult
  • SBE prophylaxis and HOCM considerations
104
Q

marfan syndrome

A
  • multisystem disorder resulting from a mutation in connective tissue protein
  • fibrillin which is a major element of extracellular microfibrils in the elastic and non-elastic connective tissues
  • involves CV, skeletal and ocular systems
  • high degree of incomplete expressivity so MUCH individual variation
105
Q

marfan syndrome clinical features

A
  • narrow facies with high arched palate and crowded teeth
  • pectus excavatum
  • lower FVC due to early airway closure from not enough elastic tissue
  • OSA
  • aortic or pulmonary artery dilation
  • mitral valve prolapse
  • wide lumbosacral canal and spinal arachnoid cysts
106
Q

marfan syndrome anesthetic considerations

A
  • avoid HTN for those at risk for aortic dissection
  • preop ECHO
  • beta blockers
  • SBE prophylaxis esp with mitral valve prolapse and insufficiency
  • increased risk for pneumothorax so careful with PPV
  • careful with positioning due to joint laxity
  • may require larger than normal doses of spinal/epidural meds due to increased length and increased CSF

N935 Advanced Pathophysiology II (16 decks)

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