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Peds Cardiology > 13. ACHD > Flashcards

13. ACHD Flashcards

1
Q

New adult patient with history of bicuspid aortic valve and mild dilation of ascending aorta… imaging?

A

Echo + some type of more advanced imaging at least once (MRI or CTA)

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2
Q

The risk of warfarin embryopathy during pregnancy is low enough to continue warfarin during pregnancy if the dose is under what?

A

5mg

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3
Q

In pregnancy, if the coumadin daily dose needed is >5mg daily, what should be done for anticoagulation?

A

Stop coumadin by week 6 gestation and resume after week 12…. give LMWH during this interval

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4
Q

If using LMWH during pregnancy for anticoagulation, what is the target Xa

A

0.7-1.3 (the higher end if it is a mechanical AV valve)

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5
Q

When does warfarin need to be stopped in all patients during pregnancy?

A

36 weeks gestation (in anticipation of delivery)

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6
Q

What should you switch warfarin to at 36 weeks gestation?

A

LMWH

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7
Q

When do you need to stop LMWH prior to delivery?

A

12 hours

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8
Q

How to resume anticoagulation post-partum?

A

If no unexpected bleeding, start coumadin and bridge with LMWH until INR is at least 2

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9
Q

Why is pregnancy a hypercoagulable state?

A

Elevated estrogen

*Stays like this first several weeks post-partum

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10
Q

What is risk of baby inheriting Holt-Oram?

A

50%

*AD, so 50% expected to inherit assuming complete penetrance

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11
Q

Secundum ASD + Short arms with thumb proximally displaced?

A

Holt Oram

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12
Q

Inheritance of Holt Oram?

A

AD

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13
Q

% Chance that a child of a parent with Holt-Oram will have CHD?

A

35%

*50% of children will have Holt Oram, 75% with Holt Oram have CHG, so 35% children will have CHD

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14
Q

What causes diastolic murmur in a large ASD?

A

Flow across the tricuspid valve (excess flow from L-R shunt)

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15
Q

Presence of a diastolic rumble in an ASD is associated with a Qp:Qs of what?

A

1.5-2

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16
Q

What is the systolic murmur due to in ASD?

A

Flow across pulmonary valve

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17
Q

In a large VSD, what is a diastolic murmur due to?

A

Flow across the mitral valve

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18
Q

Medication recommendation for patients with an ASCVD >7.5%

A

High intensity or moderate intensity statin

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19
Q

In a patient with any residual R-L shunting and atrial arrhythmia, what is indicated?

A

Warfarin

*Risk of paradoxical embolus

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20
Q

What does a Fontan fenestration gradient best correlate to?

A

Transpulmonary gradient

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21
Q

What is an expected Fontan fenestration gradient?

A

5-8mmHg

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22
Q

What can cause an increased Fontan fenestration gradient?

A

Obstruction in the Fontan circuit, lungs or pulmonary veins

*Primarily dependent on PVR

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23
Q

True or False: Elevated LA or ventricular end diastolic pressures increase the transpulmonary gradient?

A

False- no change

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24
Q

What is a low Fontan fenestration gradient associated with?

A

Hypovolemia

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25
Q

3/6 continuous murmur at LSB, peaks at 2nd heart sound, pulses all palpable, BP 120/40…. most likely cause?

A

PDA

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26
Q

If a PDA is audible in an adult with a wide pulse pressure, what else would expect to see on echo?

A

Left heart enlargement

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27
Q

What should continuous murmus make you think of?

A
  1. PDA

2. AP collateral

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28
Q

What should you think of in a patient with PA-VSD and a continuous murmur over the right back?

A

Right-sided aortic arch with PDA

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29
Q

What kind of murmur does a coronary fistula cause?

A

Continuous

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30
Q

When would you repair an ASD with adequate rims surgically over transcathter based?

A

If any additional cardiac surgery is indicated (coronary revascularization, valve repair, arrhythmia)

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31
Q

Can you calculate a Qp:Qs in a patient with Classic Glenn the lateral tunnel Fontan?

A

No- 2 sources of PBF (SVC to RPA and IVC to LPA)

*Can assess PBF or PVR

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32
Q

In a Fontan, why would the right pulmonary vein be desaturated and left pulmonary vein be normal?

A

If patient had a classic Glenn… no hepatic factor to right lung

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33
Q

What kind of testing is needed to confirm the presence of an active hepatitis C infection?

A

PCR

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34
Q

What does a positive antibody for Hep C indicate?

A

Immunization or prior infection

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35
Q

Prior to what year was formal testing for Hep C not available?

A

1992

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36
Q

What % of CHD patients that had heart surgery prior to 1992 have Hep C

A

5%

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37
Q

BNP levels have been shown to have prognostic value in what type CHD?

A

Eisenmenger

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38
Q

BNP levels above what correlate with poor long-term outcome in patients with Eisenmenger?

A

140

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39
Q

ECG with RAE and LAD, echo with RA/RV enlargement… what lesion?

A

Primum ASD

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40
Q

Where are primum ASD located?

A

Anterior/inferior aspect of atrial septum at level of MV/TV

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41
Q

What is a primum ASD often associated with?

A

Cleft in left AV valve

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42
Q

How should primum ASDs be closed?

A

Surgically

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43
Q

After a cardiac arrest, which patients require ICD?

A

Those with non-reversible causes

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44
Q

When can ablation be offered with v-tach?

A

If it is slow, stable and monomorphic

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45
Q

True or False: Patients with ToF have higher incidence of ICD complications as compared to those who are post-MI

A

True- 30% in patients with ToF compared to 10% in post-MI population

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46
Q

True or False: The patients with ToF who have ICDs have an increased incidence of inappropriate shocks as compared to other CHD

A

False- Incidence is similar (25%)

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47
Q

True or False: B-blockers, amiodarone and sotalol can decrease risk of appropriate ICD shocks in ToF patients

A

False- they don’t decrease risk

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48
Q

Can ToF patients with free PI get a transvenous ICD?

A

Yes

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49
Q

What kind of patients should get transvenous pacing or ICD leads?

A

Anyone with residual intracardiac shunts

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50
Q

TBX5

A

Holt Oram

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51
Q

How is Holt Oram inherited?

A

AD

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52
Q

TGFBR 1 and TGRBR 2

A

Loeys Dietz

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53
Q

FBN 1

A

Marfan

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54
Q

NKX2.5

A

ASD + Heart block

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55
Q

Familial occurrence of ASDs and progressive AV block?

A

NKX 2.5

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56
Q

GATA IV mutation

A

ASD without AV block

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57
Q

TBX5

A

Holt Oram

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58
Q

NOTCH mutation

A

AVSD

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59
Q

What structure is associated with a septum primum

A

Valve of fossa ovalis

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60
Q

What is the embryologic origin of the valve of the fossa ovalis?

A

Septum primum

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61
Q

Where do the superior and inferior lumbus originate from?

A

Septum secundum

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62
Q

What structures are important in septation of the AV septum and delamination of the AV valves

A

Endocardial cushions

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63
Q

What are the 4 predictors in the CARPREG score?

A
  1. Prior cardiac event (pulmonary edema, arrhythmia, stroke, cardiac death)
  2. Baseline NYHA class II or cyanosis
  3. Left heart obstruction (MV area <2cm2, aortic valve area <1.5cm2, or peak LVOT gradient >30mmHg by echo)
  4. Reduced systemic EF <40%

*Boston study also included decreased subpulmonary ventricular function and/or severe PI as predictor in risk index

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64
Q

What is the risk for cardiac complication during pregnancy with 0 predictors, 1 predictor and >1 predictor based on CARPREG?

A 
0 = 5%
1 = 25%
>1 = 75%
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65
Q

What medication can cause fetal renal dysfunction in the 3rd trimester and is considered a teratogen?

A

ACEi

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66
Q

True or False: ACEi should be avoided throughout pregnancy

A

True

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67
Q

What combination of drugs are safe in pregnancy and provide a similar physiologic response to an ACEi?

A

Hydralazine and nitrates

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68
Q

What condition has an exceptionally high risk of complication and death during pregnancy and th peripartum period?

A

Eisenmenger

*Avoid pregnancy in these patients

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69
Q

An aortic measurement above what is considered an absolute contraindication to pregnancy in patients with Marfan?

A

40mm

*Risk for dissection

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70
Q

What anticoagulant is a teratogen that needs to be avoided in the 1st trimester?

A

Warfarin

*Crosses placenta

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71
Q

What changes to delivery plan need to be made if Mom is anticoagulated with warfarin?

A

Can’t be delivered vaginally due to risk of fetal intracranial bleeding

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72
Q

The risk of warfarin embryopathy is low if therapeutic anticoagulation can be achieved with a daily dose under what?

A

5mg daily

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73
Q

What is the recommendation for anticoagulation during pregnancy if daily coumadin dose is >5mg?

A
  • Alternative anticoagulation during 1st trimester

- LMWH is safe and doesn’t cross placenta

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74
Q

What must be done for LMWH dosing during pregnancy?

A
  • Have to monitor anti-Xa levels at least weekly

* Can’t use weight based dosing due to altered volume of distribution and drug metabolism

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75
Q

Best birth control for a Fontan with thrombus history on warfarin and multiple partners?

A

Depo-Provera IM

*Some risk for hematoma at injection site

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76
Q

Best birth control for a Fontan with thrombus history on warfarin and one partner?

A

Mirena IUD

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77
Q

Why isn’t a Mirena IUD a good form of birth control for someone with multiple partners?

A

Increased risk of PID

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78
Q

What type of birth control isn’t a good choice for someone at risk for a thrombus?

A

Estrogen containing

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79
Q

What condition results from a mutation of the TGF-B receptor that results in arterial fragility?

A

Loeys-Dietz

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80
Q

What type of medication reduces TGF-B signaling?

A

ARBs

*Reduces risk of arterial complications in Loeys-Dietz

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81
Q

What medication should all patients with Loeys-Dietz be on?

A

ARBs

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82
Q

Bifid uvula, mild hypertelorism, no ectopia lentis, dilated sinus of Valsalva, family history of aortic dissection

A

Loeys-Dietz

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83
Q

What change in heme labs result from prolonged cyanosis?

A

Secondary erythrocytosis

*Increase in Hgb needed to provide appropriate O2 delivery

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84
Q

Is a secondary erythrocytosis associated with stroke or other small vessel occlusion?

A

Not unless the patient is microcytic

*Microcytic cells are less deformable as they traverse small capillary beds

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85
Q

What should be done for someone who has dyspnea, hypoxia, secondary erythrocytosis and a low MCV?

A

Iron therapy- Normalize MCV and ferritin

*Dyspnea is likely from poor O2 delivery

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86
Q

True or False: Pregnant women can undergo surgical valve replacement during pregnancy if indicated (i.e. symptomatic)?

A

True- Low risk to Mom and relatively low risk to fetus

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87
Q

What should the LDL goal be in someone with history of CoA?

A

<70

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88
Q

First line medication to lower LDL?

A

Statin

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89
Q

What medication would you use for someone with HTN in the setting of a late CoA repair?

A

ARB (Losartan)

*Low side effect profile, minimal HR changes, possible protection against aortic dilation

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90
Q

What causes HTN in the setting of late CoA repair?

A

Stiff arterial vasculature

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91
Q

What HTN meds should you avoid in the setting of a low resting HR?

A
  1. Metoprolol

2. Diltiazem

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92
Q

What % of the adult population has a PFO?

A

25-30%

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93
Q

True or False: There is no data that treatment with meds or closure to prevent paradoxical emboli is indicated in an asymptomatic patient with ASD?

A

True

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94
Q

True or False: A PFO can cause RV volume overload?

A

False

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95
Q

What is platypnea-orthodexia syndrome?

A

Positional desaturation/hypoxia (supine saturation normal, desaturates standing)

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96
Q

What causes platypnea-orthodexia?

A

Positional R-L shunting (across PFO, AVM, etc)

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97
Q

True or False: Elderly patients with PFO are more prone to R-L shunting as cardiac geometry changes with age?

A

True

*As in platypnea-orthodexia

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98
Q

How do you treat platypnea-orthodexia if due to a PFO?

A

Close the PFO

99
Q

What needs to be considered in an older adult with an ASD, dyspnea and HTN?

A

LV diastolic dysfunction- Need to get a right and left heart cath for filling pressures

  • L-R shunt volume may be increased due to LV diastolic dysfunction
  • If LV pressures too high, may become more dyspneic with ASD closure (no pop-off so LA pressures will increase)
100
Q

What chamber can become enlarged with an ASD?

A

Right heart

101
Q

What needs done prior to closing an ASD in an older adult with elevated left sided filling pressures?

A

Cath with balloon occlusion of ASD to ensure LA pressures don’t become excessively increased with ASD closure

102
Q

What non-cardiac testing should be done in patients with CoA?

A

Brain MRI to assess for intracranial aneurysms

103
Q

What routine cardiac evaluations should happen in patients with repaired CoA?

A
  • TTE: Assess function of LV and aortic valve

- MRI or CT: Assess for thoracic aorta dilation and CoA repair site

104
Q

What testing should be done for a Marfan patients with hypersomnolence?

A

Sleep study to assess for OSA

105
Q

What can OSA cause in Marfan patients?

A
  • Daytime hypersomnolence
  • HTN
  • Aortic dilation
106
Q

What anti-depressant is safest in cardiac patients?

A

Sertraline

107
Q

What cardiac relevant side effects can citalopram and amitriptyline cause?

A

Prolonged QT

108
Q

What cardiac relevant side effects can venlafaxine and bupropion cause?

A

HTN and tachycardia

*Inhibits the neuronal uptake of norepinephrine

109
Q

How does Dabigatran work?

A

Oral direct thrombin inhibitor

110
Q

True or False: Dabigatran doesn’t need to be monitored to ensure therapeutic anticoagulation?

111
Q

What conditions is Dabigatran approved for use in?

A

Atrial Fibrillation

*Not for use in mechanical valves

112
Q

What instances should Dabigatran not be used?

A
  • No data pregnancy
  • Not useful for bridging anticoagulation
  • Shouldn’t be used in patients with bleeding issues
113
Q

When does dabigatran need to be discontinued?

A

1-2 days prior to surgery

*If abnormal creatine clearance, increase to 3-5 days

114
Q

True or False: There is no direct reversal agent available to Dabigatran

115
Q

Which drug does simvastatin have an important drug-drug interaction with?

A

Amlodipine

116
Q

Using simvastatin and amlodipine together increase the risk of what?

A

Myopathy and rhabdomyolysis

*If need to use both, dose of simvastatin shouldn’t be >20mg/day

117
Q

Atenolol is what FDA pregnancy classification?

A

D

  • Positive evidence of human fetal risk, benefits may be acceptable despite risk
  • Lower birthweight infants
118
Q

Metoprolol, amlodipine, verapamil and diltiazem are what FDA pregnancy classification?

A

C

*Either animal studies with adverse effects or no controlled studies in women/animals. Only give if potential benefit justifies potential risk to fetus

119
Q

What procedure is an option for patients with menometrorrhagia who are on anticoagulation?

A

Endometrial ablation

*Safe, minimally invasive, can significantly reduce menstrual bleeding (esp >35years), no need for interruption of anticoagulation

120
Q

When should you not pursue an endometrial ablation?

A

If future pregnancies are wanted

121
Q

What procedure can provide similar relief of menometrorrhagia to a hysterectomy?

A

Endometrial ablation, but no need to stop anticoagulation

*Hysterectomy can provide same symptom relief, but need to stop anticoagulation for surgery

122
Q

What can’t you use in women with menometrorrhagia who have an increased risk of thrombosis?

A

Estrogen containing contraception

123
Q

How should flecainide be initiated?

A

Inpatient admission for 5 full doses to steady state with daily ECG to check QRS duration

124
Q

What class if flecainide?

A

IC antiarrythmic

125
Q

What can flecainide cause?

A

QRS prolongation- Can cause proarrhythmia

126
Q

What systems/organs does amiodarone negatively effect?

A

Liver
Pulmonary
Thyroid

127
Q

How often should PFTs be done in someone on amiodarone?

A

At least yearly

128
Q

How often should LFTs be done in someone on amiodarone?

A

Twice yearly

129
Q

How often should thyroid studies be cone in someone on amiodarone?

A

Every 3-6 months

130
Q

What pulmonary vasodilator has the highest incidence of lower extremity edema?

131
Q

What is important to counsel on for patients taking the “mini pill” progesterone only OCP?

A

Need to take dose at exact same time daily

*Abx may reduce effectiveness, so need other contraception if needing SBE for some reason

132
Q

What should be done for symptomatic sinus venosus ASD with PAPVR (RUPV to SVC-RA junction) and right heart dilation?

A

Surgical ASD closure + Pulmonary venous baffle

*ASD closure alone would allow persistent L-R shunting through the right superior pulmonary vein

133
Q

What guides management of a VSD?

A

Hemodynamic effects

134
Q

What are some signs that a VSD is small and of no hemodynamic consequence?

A
  • No LA/LV chamber enlargement
  • Normal RVSP
  • High flow velocity across VSD (least trustworthy of findings)
135
Q

Does a VSD need SBE prophylaxis?

136
Q

What should you look for in a patient with cardiomegaly on CXR, mild flow acceleration at the pulmonary valve and moderate dilation of RA/RV?

A

ASD

*Mild PS shouldn’t cause RA/RV enlargement, so need to look for why chamber dilated…a hemodynamically significant ASD would result in L-R shunting with RA/RV dilation

137
Q

What chamber would enlarge with a PDA and normal PVR?

A

Left chambers… increased pulmonary venous return

138
Q

What is the most common late post-op complication after ASO?

A

Supravalvar PS

*Can also see coronary ostial stenosis, atrial arrhythmias, aortic stenosis (all less common or rare)

139
Q

What could be done for supravalvar PS following ASO?

A

Stent (avoiding PV)

*Balloon valvuloplasty w/o stenting has a low success rate

140
Q

How long is SBE prophylaxis needed following VSD surgery with a non-valvular prosthetic patch and no residual peri-patch leak?

141
Q

How long does someone with a history of bacterial endocarditis need SBE prophylaxis?

142
Q

True or False: SBE prophylaxis is required for upper endoscopy and colonoscopy?

A

False- SBE prophylaxis isn’t indicated for patients undergoing non-dental intervention in the absence of active systemic infection

143
Q

What form of CHD is clearly associated with atrioventricular re-entrant accessory pathway tachycardia and life-threatening ventricular arrhythmias?

A

Ebstein

*Especially true for patients with a decline in hemodynamic status

144
Q

What type of tachycardia is more likely to cause a sudden LOC with no prodromal sympatoms?

A

Ventricular tachycardia

145
Q

True or False: Myoclonus is common in any LOC?

146
Q

Junctional bradycardia is common in CHD after what procedures?

A

Mustard/Senning

Fontan

147
Q

True or False: Onset of a junctional rhythm in a Fontan patient which causes hemodynamic impact is a class I indication for atrial or dual-chamber pacemaker placement?

148
Q

What would increasing abdominal girth and decreasing exercise tolerance with a liver edge 4cm below the costal margin in a Fontan patient be a sign of?

A

Elevated IVC pressure and CVP

149
Q

What CHD would you think of in an adult patient undergoing a right heart cath for biventricular systolic dysfunction who develops 3rd degree heart block with echo showing chordal attachments from left-sided AV valve to IVS?

A

cc-TGA

  • Conduction system is in an abnormal location and structure, so more vulnerable to physical trauma from cath
  • Also susceptible to spontaneous heart block associated with increasing age
150
Q

How might a diagnosis of cc-TGA be delayed into adulthood?

A

If there is adequate systemic ventricular function and no obvious murmur from a VSD or pulmonary/subpulmonary stenosis

151
Q

When is routine phlebotomy for erythrocytosis in cyanotic patients recommended?

A

Euvolemic patient with Hgb >20 and Hct >65 with symptoms of hyperviscosity (HA, vision change, neuro symptoms)

*Need equal volume crystalloid replacement

152
Q

True or False: Routine phlebotomy for erythrocytosis in cyanotic patients isn’t recommended in the absence of symptoms

153
Q

What can routine phlebotomy for erythrocytosis in cyanotic patients result in?

A

Iron deficiency anemia with resultant microcytosis

*Microcytosis increases viscosity which perpetuates a cycle of phlebotomy and worsening microcytosis leading to symptoms

154
Q

True or False: Microcytosis independently increases viscosity

155
Q

What should be done for a patient with hyperviscosity, but no symptoms?

A

Order iron studies and consider replacement as indicated

156
Q

Per the 2008 ACC/AHA valvular heart disease guidelines, which patients with valve disease need cath with coronary angiography?

A
  • Men >35
  • Premenopausal women >35 with coronary RF
  • Postmenopausal women
157
Q

What may happen is someone stops smoking 1 week prior to a surgery?

A

They may have an increase in respiratory secretions in the perioperative period

158
Q

What screening do family members of a patients with a bicuspid aortic valve need?

A

TTE- All 1st degree relatives should be screened

159
Q

What 2 things may be identified in a 1st degree relative of someone with a bicuspid aortic valve?

A
  • Bicuspid aortic valve

- Isolated ascending aorta dilation

160
Q

Abnormalities resulting in bicuspid aortic valve have a clear association with what?

A

Abnormal aortic dilation

*Process isn’t isolated to aortic valve alone

161
Q

Long-term surveillance for CoA repair should include what?

A
  • MRI/MRA head

- CT or MRI thoracic aorta

162
Q

What neurological complication are CoA patients at risk for?

A

Cerebral aneurysms- Need screening with MRI/MRA head

163
Q

Why should patients who are s/p CoA repair get a CT or MRI of the thoracic aorta?

A

They are at risk for pseudoaneurysm formation at site of prior surgical repair

*Echo evaluation of aortic lumen/blood flow doesn’t exclude pseudoaneurysm formation

164
Q

What are some late post-op complications after AVSD repair?

A
  • LVOTO
  • Heart block
  • Left AVVR
165
Q

What should be done if a new-onset atrial arrhythmia is seen in a AVSD repair patient?

A

Thorough anatomic and hemodynamic evaluation for post-op complications (think about a-fib)

166
Q

In a post-op AVSD repair with isolated LVOTO, what gradients are an indication for surgical intervention?

A

Mean >50mmHg

Peak >70mmHg

167
Q

Why are ACEi relatively contraindicated in the presence of fixed LVOTO?

A

-May result in hypotension and coronary hypoperfusion

168
Q

What would cause an elevated RVSP estimate by TR jet velocity with normal RV/PA pressures by cath in a patient with T21 and CAVD s/p repair?

A

LV-RA shunting

*LV-RA shunting with normal right pressures results in high velocity L-R shunt… this Doppler signal may contaminate the TR signal

169
Q

What can occur in adulthood in patients with an isolated membranous VSD?

A

Development of double chamber RV

170
Q

Describe double chamber RV

A
  • Proximal, upstream portion of RV at high pressure separated by abnormal muscular hypertrophy from a more distal low-pressure outflow portion
  • PA pressure distal to the obstruction should be normal
171
Q

What changes in exam/workup might suggest development of double-chambered RV?

A
  • Increase in murmur intensity
  • New thrill
  • Findings of RV pressure loading- Increased RV impulse
  • RVH by ECG
172
Q

What is the management for double-chambered RV?

A

Surgical resection

173
Q

What are risk factors for sudden death after ToF repair?

A
  • QRS >180 msec
  • Poor RV hemodynamics
  • Older age at repair
  • Prolonged palliative shunts
174
Q

In an adult ToF with a QRS >180msec and syncope, what should be done next?

A
  • Consider EP study for assess for inducible ventricular arrhythmia
  • Could argue for ICD regardless
175
Q

True or False: Atrial arrhythmia and AV node dysfunction are known complications following ToF repair, but aren’t a primary indication for an EP study in the setting of syncope

176
Q

What are some complications following a Mustard/Senning?

A
  • Sinus node dysfunction
  • Atrial arrhythmia
  • Baffle leak
  • Baffle obstruction
177
Q

What do you need to ensure before doing a transvenous pacemaker in someone who is s/p Mustard or Senning?

A

Cath to assess for any SVC/baffle stenosis or baffle leak

*Possible to get worsening of stenosis, paradoxical emboli across a baffle leak or incorrect positioning of the ventricular leads across the baffles

178
Q

What is a potential complication of a baffle leak in someone with a Mustard/Senning?

A

Paradoxical emboli

179
Q

What should be considered for someone with exertional chest pain who is s/p ASO?

A

Coronary obstruction

180
Q

What anatomy may pose a higher risk of coronary obstruction s/p ASO?

A

Single coronary artery

181
Q

What is the gold standard for coronary assessment s/p ASO?

A

Coronary angiography

*CTA may be appropriate

182
Q

What can happen to function after a pacemaker is placed and ventricular pacing initiated?

A

Pacemaker induced cardiomyopathy (progressive systemic ventricular dysfunction)

*Need to have close surveillance via echo after pacemaker placement

183
Q

What can help with pacemaker induced cardiomyopathy?

A

Consideration of biventricular pacing

*May reverse dysfunction in some patients

184
Q

What may need to be done for a VQ scan in a Fontan patient with suspected PE?

A

Injection from upper and lower extremities

*There is streaming of blood flow in Fontan physiology, so SVC may preferentially go to one side and IVC to other

185
Q

True or False: Patients with Fontan palliation are at increased risk for systemic venous thromboembolic events

186
Q

What is the “gold standard” for PE assessment in Fontan?

A

Invasive pulmonary angiography

187
Q

True or False: An intramural course of an anomalous coronary artery in a long segment through the wall of the aorta is a risk factor for cardiac ischemia and death

188
Q

What is the recommended management of an RCA the arises from the left coronary cusp with a proximal intramural course subsequently passing between the PA and aorta?

A

Unroofing of intramural RCA

189
Q

What is a risk with a small PDA that is hemodynamically insignificant?

A

Possible endocarditis

190
Q

What is Scimitar syndrome?

A

Anomalous connection of the right lower pulmonary vein to the IVC

*Sometimes the right upper/middle pulmonary veins also connect to the IVC

191
Q

What % of cases of Scimitar syndrome have associated intracardiac CHD?

A

25%

*Usually an ASD

192
Q

True or False: In the majority of cases of Scimitar, the atrial septum is intact?

193
Q

Why is the cardiac silhouette shifted rightward in Scimiatr?

A

Right lung hypoplasia

194
Q

Describe the right lung in Scimitar

A

-Right lower love hypoplasia with sequestration and arterial supply from a vessel originating from the descending aorta

195
Q

What is the ECG likely to show in an adult patient with cc-TGA?

A

Complete heart block

*By 40, complete heart block and need for pacemaker is common in patients with cc-TGA

196
Q

Q waves in I and aVL?

197
Q

Northwest/left QRS axis?

198
Q

What would you see on the ECG in someone with Ebsteins or s/o VSD/ToF repair?

199
Q

What should you consider in a teenage with stridor during exercise (symptoms present throughout life) and periodic cyanosis as a baby?

200
Q

Describe an LPA sling

A
  • Origin of LPA is from the RPA (not at the level of the true PA bifurcation
  • LPA crosses between the bronchus and esophagus
201
Q

What is seen on a barium esophagram in an LPA sling?

A

Anterior indentation of the esophagus

202
Q

What is thought to cause congenital complete AV block?

A

Transplacental passage of autoantibodies against Ro/La intracellular ribonuclear proteins from Mom (possible with autoimmune diseases like SLE or Sjogrens)

203
Q

Permanent pacemaker implantation is indicated for congenital 3rd degree AV block in what situations independent of symptoms?

A
  • Wide QRS escape rhythm (unreliable, can cause abrupt pauses leading to arrest)
  • Complex ventricular ectopy
  • Ventricular dysfunction
204
Q

What can help with ventricular dysfunction in the setting of complete congenital heart block?

A

Pacemaker

*ACEi can be used in combination with device therapy

205
Q

True or False: All patients with congenital AV block should have a screening EP study in adolesence

A

False- No indication

206
Q

In patients with CHD, what are 2 class I indications for ICD when other remediable causes (hemodynamic or arrhythmic) have been excluded?

A
  • Spontaneous sustained VT

- Unexplained syncope with inducible sustained hypotensive VT

207
Q

What type of ICD/Pacemaker is needed for patients with cyanosis and intracardiac shunts?

A

Epicardial

208
Q

Why should you not use transvenous leads in a patient with an intracardiac shunt?

A

Transvenous leads have a >2 fold increased risk of systemic thromboemboli independent of warfarin or ASA

209
Q

What is a unique atrial arrhythmia seen in patients who have had extensive scarring of the atria?

A

Incisional atrial flutter

210
Q

What % of patients can have incisional atrial flutter following Fontan?

211
Q

Which has slower rates, atrial flutter or IART?

212
Q

What is the difference on ECG between IART and Flutter?

A
  • IART has an isoelectric baseline between 2 consecutive P-waves
  • Flutter has constant activity (causes saw-tooth pattern)
213
Q

What should you consider for someone with baseline bradycardia and no variation in heart rate?

A

IART- Get an ECG

214
Q

What is the management for IART?

A

Cardioversion

*Need to check for thrombus or have anticoagulation prior to cardioversion

215
Q

What is the most common presentation of a small-moderate ASD?

A

Exercise intolerance (2nd decade)

216
Q

What are common exam findings of an ASD?

A
  • Fixed, split S2

- Pulmonary flow murmur (from L-R atrial shunting)

217
Q

What is often seen on the ECG with an ASD?

A

Incomplete RBBB
rSR’ in V1
Possible RAE

218
Q

What is helpful to diagnose an ASD in an adult or obese patient?

A

Agitated saline contrast echo

  • Agitated saline injected into peripheral vein while imaging RA/LA…shows contrast to LA
  • Can also do a TEE or MRI
219
Q

True or False: Any patient who survived cardiac arrest due to non-reversible causes should have an ICD

220
Q

When could ablation be considered over ICD in someone with v-tach?

A

If it is slow, stable and monomorphic

221
Q

What is the incidence of ICD complications?

222
Q

Which is more effective in preventing recurrent arrhythmias and SCD, anti-arrhythmics or ICD?

223
Q

True or False: B-blockers, amiodarone and sotalol can help to decrease the risk of appropriate ICD shocks in a patients with ToF?

224
Q

What type of ICD is preferred in patients who don’t have a specific indication for surgery to replace the pulmonary valve?

A

Transvenous

225
Q

True or False: Transvenous pacemaker can be done in someone with free PI

226
Q

What can cardiac resynchronization therapy with biventricular pacing help to do in someone with ToF?

A
  • Improve hemodynamics and cardiac symptoms

- Decrease QRS duration

227
Q

Is biventricular pacing an alternative to ICD in someone with a survived cardiac arrest?

228
Q

What are some ECG clues to IART?

A
  • HR above normal in patient with sinus node dysfunction
  • Marked variability of ventricular rate or no heart rate variability
  • Abnormal P wave axis
  • Prolongation of PR interval
  • Variability of PR interval throughout the tracing
229
Q

What is needed in all atrial fibrillation or atrial flutter?

A

Antithrombotic therapy

*Coumadin and rivaroxaban

230
Q

How should atrial fibrillation or flutter be managed in a new diagnosis with stable hemodynamics and no RVR?

A

Anticoagulate for 3 weeks then cardioversion

231
Q

What is an advantage of rivaroxaban over coumadin?

A

No need to check INR to adjust dose

*Similar efficacy and safety profile to Coumain

232
Q

What role does digoxin or beta blockers play in atrial fibrillation?

A

Slow conduction in the AV node and prevent rapid conduction of atrial arrhythmia

233
Q

Irregularly irregular rhythm

234
Q

What might cause syncope in a-fib with RVR?

A

Catecholamine surge increasing ventricular rate

235
Q

True or False: Decreased function can result from a-fib with RVR?

236
Q

In a patient with syncope and depressed function due to a-fib with RVR, what needs to be done?

A

TEE to check for large thrombus then cardioversion- Don’t really have room to anticoagulate for 3 weeks then cardiovert

237
Q

How does TEE assessment for thombus factor into decision to cardiovert for a-fib?

A
  • No thrombus, can cardiovert immediately (still some small risk of clot causing stroke)
  • Large thrombus requires anticoagulation for a period of time prior to cardioversion
238
Q

What types of patients need ACHD center for non-cardiac surgery?

A
  • Fontan
  • Severe PAH
  • Cyanotic CHD
  • Complex CHD
  • Malignant arrhythmia
239
Q

When should patients s/p AVSD repair have surgical intervention for LVOTO?

A
  • Maximal instantaneous gradient >70mmHg

- Lower gradient in association with significant MR or AI

240
Q

What is a class 1 indication for surgical intervention of supravalvar AS?

A

Mean gradient 50mmHg or higher in asymptomatic patients

241
Q

Patients with supravalvar AS and a gradient <50mmHg should be considered for surgical intervention in what circumstances?

A
  • Symptomatic
  • LVH
  • Planning pregnancy
242
Q

How often should asymptomatic patients with a max instantaneous pulmonary valve gradient >30mmHg have a follow-up echo?

A

Every 2-5 years

243
Q

Noninvasive testing for ischemia provocation is recommended how often for patients after ASO?

A

Every 3-5 years

244
Q

True or False: A small coronary fistulae with no symptoms, no murmur and no evidence of hemodynamic compromise doesn’t need further evaluation or treatment

Peds Cardiology (20 decks)

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