13. ACHD Flashcards
(244 cards)
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)
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
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
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)
5
Q
When does warfarin need to be stopped in all patients during pregnancy?
A
36 weeks gestation (in anticipation of delivery)
6
Q
What should you switch warfarin to at 36 weeks gestation?
A
LMWH
7
Q
When do you need to stop LMWH prior to delivery?
A
12 hours
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
9
Q
Why is pregnancy a hypercoagulable state?
A
Elevated estrogen
*Stays like this first several weeks post-partum
10
Q
What is risk of baby inheriting Holt-Oram?
A
50%
*AD, so 50% expected to inherit assuming complete penetrance
11
Q
Secundum ASD + Short arms with thumb proximally displaced?
A
Holt Oram
12
Q
Inheritance of Holt Oram?
A
AD
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
14
Q
What causes diastolic murmur in a large ASD?
A
Flow across the tricuspid valve (excess flow from L-R shunt)
15
Q
Presence of a diastolic rumble in an ASD is associated with a Qp:Qs of what?
A
1.5-2
16
Q
What is the systolic murmur due to in ASD?
A
Flow across pulmonary valve
17
Q
In a large VSD, what is a diastolic murmur due to?
A
Flow across the mitral valve
18
Q
Medication recommendation for patients with an ASCVD >7.5%
A
High intensity or moderate intensity statin
19
Q
In a patient with any residual R-L shunting and atrial arrhythmia, what is indicated?
A
Warfarin
*Risk of paradoxical embolus
20
Q
What does a Fontan fenestration gradient best correlate to?
A
Transpulmonary gradient
21
Q
What is an expected Fontan fenestration gradient?
A
5-8mmHg
22
Q
What can cause an increased Fontan fenestration gradient?
A
Obstruction in the Fontan circuit, lungs or pulmonary veins
*Primarily dependent on PVR
23
Q
True or False: Elevated LA or ventricular end diastolic pressures increase the transpulmonary gradient?
A
False- no change
24
Q
What is a low Fontan fenestration gradient associated with?
A
Hypovolemia
25
3/6 continuous murmur at LSB, peaks at 2nd heart sound, pulses all palpable, BP 120/40.... most likely cause?
PDA
26
If a PDA is audible in an adult with a wide pulse pressure, what else would expect to see on echo?
Left heart enlargement
27
What should continuous murmus make you think of?
1. PDA
| 2. AP collateral
28
What should you think of in a patient with PA-VSD and a continuous murmur over the right back?
Right-sided aortic arch with PDA
29
What kind of murmur does a coronary fistula cause?
Continuous
30
When would you repair an ASD with adequate rims surgically over transcathter based?
If any additional cardiac surgery is indicated (coronary revascularization, valve repair, arrhythmia)
31
Can you calculate a Qp:Qs in a patient with Classic Glenn the lateral tunnel Fontan?
No- 2 sources of PBF (SVC to RPA and IVC to LPA)
*Can assess PBF or PVR
32
In a Fontan, why would the right pulmonary vein be desaturated and left pulmonary vein be normal?
If patient had a classic Glenn... no hepatic factor to right lung
33
What kind of testing is needed to confirm the presence of an active hepatitis C infection?
PCR
34
What does a positive antibody for Hep C indicate?
Immunization or prior infection
35
Prior to what year was formal testing for Hep C not available?
1992
36
What % of CHD patients that had heart surgery prior to 1992 have Hep C
5%
37
BNP levels have been shown to have prognostic value in what type CHD?
Eisenmenger
38
BNP levels above what correlate with poor long-term outcome in patients with Eisenmenger?
140
39
ECG with RAE and LAD, echo with RA/RV enlargement... what lesion?
Primum ASD
40
Where are primum ASD located?
Anterior/inferior aspect of atrial septum at level of MV/TV
41
What is a primum ASD often associated with?
Cleft in left AV valve
42
How should primum ASDs be closed?
Surgically
43
After a cardiac arrest, which patients require ICD?
Those with non-reversible causes
44
When can ablation be offered with v-tach?
If it is slow, stable and monomorphic
45
True or False: Patients with ToF have higher incidence of ICD complications as compared to those who are post-MI
True- 30% in patients with ToF compared to 10% in post-MI population
46
True or False: The patients with ToF who have ICDs have an increased incidence of inappropriate shocks as compared to other CHD
False- Incidence is similar (25%)
47
True or False: B-blockers, amiodarone and sotalol can decrease risk of appropriate ICD shocks in ToF patients
False- they don't decrease risk
48
Can ToF patients with free PI get a transvenous ICD?
Yes
49
What kind of patients should get transvenous pacing or ICD leads?
Anyone with residual intracardiac shunts
50
TBX5
Holt Oram
51
How is Holt Oram inherited?
AD
52
TGFBR 1 and TGRBR 2
Loeys Dietz
53
FBN 1
Marfan
54
NKX2.5
ASD + Heart block
55
Familial occurrence of ASDs and progressive AV block?
NKX 2.5
56
GATA IV mutation
ASD without AV block
57
TBX5
Holt Oram
58
NOTCH mutation
AVSD
59
What structure is associated with a septum primum
Valve of fossa ovalis
60
What is the embryologic origin of the valve of the fossa ovalis?
Septum primum
61
Where do the superior and inferior lumbus originate from?
Septum secundum
62
What structures are important in septation of the AV septum and delamination of the AV valves
Endocardial cushions
63
What are the 4 predictors in the CARPREG score?
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
64
What is the risk for cardiac complication during pregnancy with 0 predictors, 1 predictor and >1 predictor based on CARPREG?
```
0 = 5%
1 = 25%
>1 = 75%
```
65
What medication can cause fetal renal dysfunction in the 3rd trimester and is considered a teratogen?
ACEi
66
True or False: ACEi should be avoided throughout pregnancy
True
67
What combination of drugs are safe in pregnancy and provide a similar physiologic response to an ACEi?
Hydralazine and nitrates
68
What condition has an exceptionally high risk of complication and death during pregnancy and th peripartum period?
Eisenmenger
*Avoid pregnancy in these patients
69
An aortic measurement above what is considered an absolute contraindication to pregnancy in patients with Marfan?
40mm
*Risk for dissection
70
What anticoagulant is a teratogen that needs to be avoided in the 1st trimester?
Warfarin
*Crosses placenta
71
What changes to delivery plan need to be made if Mom is anticoagulated with warfarin?
Can't be delivered vaginally due to risk of fetal intracranial bleeding
72
The risk of warfarin embryopathy is low if therapeutic anticoagulation can be achieved with a daily dose under what?
5mg daily
73
What is the recommendation for anticoagulation during pregnancy if daily coumadin dose is >5mg?
- Alternative anticoagulation during 1st trimester
| - LMWH is safe and doesn't cross placenta
74
What must be done for LMWH dosing during pregnancy?
- Have to monitor anti-Xa levels at least weekly
| * Can't use weight based dosing due to altered volume of distribution and drug metabolism
75
Best birth control for a Fontan with thrombus history on warfarin and multiple partners?
Depo-Provera IM
*Some risk for hematoma at injection site
76
Best birth control for a Fontan with thrombus history on warfarin and one partner?
Mirena IUD
77
Why isn't a Mirena IUD a good form of birth control for someone with multiple partners?
Increased risk of PID
78
What type of birth control isn't a good choice for someone at risk for a thrombus?
Estrogen containing
79
What condition results from a mutation of the TGF-B receptor that results in arterial fragility?
Loeys-Dietz
80
What type of medication reduces TGF-B signaling?
ARBs
*Reduces risk of arterial complications in Loeys-Dietz
81
What medication should all patients with Loeys-Dietz be on?
ARBs
82
Bifid uvula, mild hypertelorism, no ectopia lentis, dilated sinus of Valsalva, family history of aortic dissection
Loeys-Dietz
83
What change in heme labs result from prolonged cyanosis?
Secondary erythrocytosis
*Increase in Hgb needed to provide appropriate O2 delivery
84
Is a secondary erythrocytosis associated with stroke or other small vessel occlusion?
Not unless the patient is microcytic
*Microcytic cells are less deformable as they traverse small capillary beds
85
What should be done for someone who has dyspnea, hypoxia, secondary erythrocytosis and a low MCV?
Iron therapy- Normalize MCV and ferritin
*Dyspnea is likely from poor O2 delivery
86
True or False: Pregnant women can undergo surgical valve replacement during pregnancy if indicated (i.e. symptomatic)?
True- Low risk to Mom and relatively low risk to fetus
87
What should the LDL goal be in someone with history of CoA?
<70
88
First line medication to lower LDL?
Statin
89
What medication would you use for someone with HTN in the setting of a late CoA repair?
ARB (Losartan)
*Low side effect profile, minimal HR changes, possible protection against aortic dilation
90
What causes HTN in the setting of late CoA repair?
Stiff arterial vasculature
91
What HTN meds should you avoid in the setting of a low resting HR?
1. Metoprolol
| 2. Diltiazem
92
What % of the adult population has a PFO?
25-30%
93
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?
True
94
True or False: A PFO can cause RV volume overload?
False
95
What is platypnea-orthodexia syndrome?
Positional desaturation/hypoxia (supine saturation normal, desaturates standing)
96
What causes platypnea-orthodexia?
Positional R-L shunting (across PFO, AVM, etc)
97
True or False: Elderly patients with PFO are more prone to R-L shunting as cardiac geometry changes with age?
True
*As in platypnea-orthodexia
98
How do you treat platypnea-orthodexia if due to a PFO?
Close the PFO
99
What needs to be considered in an older adult with an ASD, dyspnea and HTN?
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
What chamber can become enlarged with an ASD?
Right heart
101
What needs done prior to closing an ASD in an older adult with elevated left sided filling pressures?
Cath with balloon occlusion of ASD to ensure LA pressures don't become excessively increased with ASD closure
102
What non-cardiac testing should be done in patients with CoA?
Brain MRI to assess for intracranial aneurysms
103
What routine cardiac evaluations should happen in patients with repaired CoA?
- TTE: Assess function of LV and aortic valve
| - MRI or CT: Assess for thoracic aorta dilation and CoA repair site
104
What testing should be done for a Marfan patients with hypersomnolence?
Sleep study to assess for OSA
105
What can OSA cause in Marfan patients?
- Daytime hypersomnolence
- HTN
- Aortic dilation
106
What anti-depressant is safest in cardiac patients?
Sertraline
107
What cardiac relevant side effects can citalopram and amitriptyline cause?
Prolonged QT
108
What cardiac relevant side effects can venlafaxine and bupropion cause?
HTN and tachycardia
*Inhibits the neuronal uptake of norepinephrine
109
How does Dabigatran work?
Oral direct thrombin inhibitor
110
True or False: Dabigatran doesn't need to be monitored to ensure therapeutic anticoagulation?
True
111
What conditions is Dabigatran approved for use in?
Atrial Fibrillation
*Not for use in mechanical valves
112
What instances should Dabigatran not be used?
- No data pregnancy
- Not useful for bridging anticoagulation
- Shouldn't be used in patients with bleeding issues
113
When does dabigatran need to be discontinued?
1-2 days prior to surgery
*If abnormal creatine clearance, increase to 3-5 days
114
True or False: There is no direct reversal agent available to Dabigatran
True
115
Which drug does simvastatin have an important drug-drug interaction with?
Amlodipine
116
Using simvastatin and amlodipine together increase the risk of what?
Myopathy and rhabdomyolysis
*If need to use both, dose of simvastatin shouldn't be >20mg/day
117
Atenolol is what FDA pregnancy classification?
D
* Positive evidence of human fetal risk, benefits may be acceptable despite risk
* Lower birthweight infants
118
Metoprolol, amlodipine, verapamil and diltiazem are what FDA pregnancy classification?
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
What procedure is an option for patients with menometrorrhagia who are on anticoagulation?
Endometrial ablation
*Safe, minimally invasive, can significantly reduce menstrual bleeding (esp >35years), no need for interruption of anticoagulation
120
When should you not pursue an endometrial ablation?
If future pregnancies are wanted
121
What procedure can provide similar relief of menometrorrhagia to a hysterectomy?
Endometrial ablation, but no need to stop anticoagulation
*Hysterectomy can provide same symptom relief, but need to stop anticoagulation for surgery
122
What can't you use in women with menometrorrhagia who have an increased risk of thrombosis?
Estrogen containing contraception
123
How should flecainide be initiated?
Inpatient admission for 5 full doses to steady state with daily ECG to check QRS duration
124
What class if flecainide?
IC antiarrythmic
125
What can flecainide cause?
QRS prolongation- Can cause proarrhythmia
126
What systems/organs does amiodarone negatively effect?
Liver
Pulmonary
Thyroid
127
How often should PFTs be done in someone on amiodarone?
At least yearly
128
How often should LFTs be done in someone on amiodarone?
Twice yearly
129
How often should thyroid studies be cone in someone on amiodarone?
Every 3-6 months
130
What pulmonary vasodilator has the highest incidence of lower extremity edema?
Bosentan
131
What is important to counsel on for patients taking the "mini pill" progesterone only OCP?
Need to take dose at exact same time daily
*Abx may reduce effectiveness, so need other contraception if needing SBE for some reason
132
What should be done for symptomatic sinus venosus ASD with PAPVR (RUPV to SVC-RA junction) and right heart dilation?
Surgical ASD closure + Pulmonary venous baffle
*ASD closure alone would allow persistent L-R shunting through the right superior pulmonary vein
133
What guides management of a VSD?
Hemodynamic effects
134
What are some signs that a VSD is small and of no hemodynamic consequence?
- No LA/LV chamber enlargement
- Normal RVSP
- High flow velocity across VSD (least trustworthy of findings)
135
Does a VSD need SBE prophylaxis?
No
136
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?
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
What chamber would enlarge with a PDA and normal PVR?
Left chambers... increased pulmonary venous return
138
What is the most common late post-op complication after ASO?
Supravalvar PS
*Can also see coronary ostial stenosis, atrial arrhythmias, aortic stenosis (all less common or rare)
139
What could be done for supravalvar PS following ASO?
Stent (avoiding PV)
*Balloon valvuloplasty w/o stenting has a low success rate
140
How long is SBE prophylaxis needed following VSD surgery with a non-valvular prosthetic patch and no residual peri-patch leak?
6 months
141
How long does someone with a history of bacterial endocarditis need SBE prophylaxis?
Life
142
True or False: SBE prophylaxis is required for upper endoscopy and colonoscopy?
False- SBE prophylaxis isn't indicated for patients undergoing non-dental intervention in the absence of active systemic infection
143
What form of CHD is clearly associated with atrioventricular re-entrant accessory pathway tachycardia and life-threatening ventricular arrhythmias?
Ebstein
*Especially true for patients with a decline in hemodynamic status
144
What type of tachycardia is more likely to cause a sudden LOC with no prodromal sympatoms?
Ventricular tachycardia
145
True or False: Myoclonus is common in any LOC?
True
146
Junctional bradycardia is common in CHD after what procedures?
Mustard/Senning
| Fontan
147
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?
True
148
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?
Elevated IVC pressure and CVP
149
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?
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
How might a diagnosis of cc-TGA be delayed into adulthood?
If there is adequate systemic ventricular function and no obvious murmur from a VSD or pulmonary/subpulmonary stenosis
151
When is routine phlebotomy for erythrocytosis in cyanotic patients recommended?
Euvolemic patient with Hgb >20 and Hct >65 with symptoms of hyperviscosity (HA, vision change, neuro symptoms)
*Need equal volume crystalloid replacement
152
True or False: Routine phlebotomy for erythrocytosis in cyanotic patients isn't recommended in the absence of symptoms
True
153
What can routine phlebotomy for erythrocytosis in cyanotic patients result in?
Iron deficiency anemia with resultant microcytosis
*Microcytosis increases viscosity which perpetuates a cycle of phlebotomy and worsening microcytosis leading to symptoms
154
True or False: Microcytosis independently increases viscosity
True
155
What should be done for a patient with hyperviscosity, but no symptoms?
Order iron studies and consider replacement as indicated
156
Per the 2008 ACC/AHA valvular heart disease guidelines, which patients with valve disease need cath with coronary angiography?
- Men >35
- Premenopausal women >35 with coronary RF
- Postmenopausal women
157
What may happen is someone stops smoking 1 week prior to a surgery?
They may have an increase in respiratory secretions in the perioperative period
158
What screening do family members of a patients with a bicuspid aortic valve need?
TTE- All 1st degree relatives should be screened
159
What 2 things may be identified in a 1st degree relative of someone with a bicuspid aortic valve?
- Bicuspid aortic valve
| - Isolated ascending aorta dilation
160
Abnormalities resulting in bicuspid aortic valve have a clear association with what?
Abnormal aortic dilation
*Process isn't isolated to aortic valve alone
161
Long-term surveillance for CoA repair should include what?
- MRI/MRA head
| - CT or MRI thoracic aorta
162
What neurological complication are CoA patients at risk for?
Cerebral aneurysms- Need screening with MRI/MRA head
163
Why should patients who are s/p CoA repair get a CT or MRI of the thoracic aorta?
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
What are some late post-op complications after AVSD repair?
- LVOTO
- Heart block
- Left AVVR
165
What should be done if a new-onset atrial arrhythmia is seen in a AVSD repair patient?
Thorough anatomic and hemodynamic evaluation for post-op complications (think about a-fib)
166
In a post-op AVSD repair with isolated LVOTO, what gradients are an indication for surgical intervention?
Mean >50mmHg
| Peak >70mmHg
167
Why are ACEi relatively contraindicated in the presence of fixed LVOTO?
-May result in hypotension and coronary hypoperfusion
168
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?
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
What can occur in adulthood in patients with an isolated membranous VSD?
Development of double chamber RV
170
Describe double chamber RV
- 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
What changes in exam/workup might suggest development of double-chambered RV?
- Increase in murmur intensity
- New thrill
- Findings of RV pressure loading- Increased RV impulse
- RVH by ECG
172
What is the management for double-chambered RV?
Surgical resection
173
What are risk factors for sudden death after ToF repair?
- QRS >180 msec
- Poor RV hemodynamics
- Older age at repair
- Prolonged palliative shunts
174
In an adult ToF with a QRS >180msec and syncope, what should be done next?
- Consider EP study for assess for inducible ventricular arrhythmia
- Could argue for ICD regardless
175
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
True
176
What are some complications following a Mustard/Senning?
- Sinus node dysfunction
- Atrial arrhythmia
- Baffle leak
- Baffle obstruction
177
What do you need to ensure before doing a transvenous pacemaker in someone who is s/p Mustard or Senning?
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
What is a potential complication of a baffle leak in someone with a Mustard/Senning?
Paradoxical emboli
179
What should be considered for someone with exertional chest pain who is s/p ASO?
Coronary obstruction
180
What anatomy may pose a higher risk of coronary obstruction s/p ASO?
Single coronary artery
181
What is the gold standard for coronary assessment s/p ASO?
Coronary angiography
*CTA may be appropriate
182
What can happen to function after a pacemaker is placed and ventricular pacing initiated?
Pacemaker induced cardiomyopathy (progressive systemic ventricular dysfunction)
*Need to have close surveillance via echo after pacemaker placement
183
What can help with pacemaker induced cardiomyopathy?
Consideration of biventricular pacing
*May reverse dysfunction in some patients
184
What may need to be done for a VQ scan in a Fontan patient with suspected PE?
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
True or False: Patients with Fontan palliation are at increased risk for systemic venous thromboembolic events
True
186
What is the "gold standard" for PE assessment in Fontan?
Invasive pulmonary angiography
187
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
True
188
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?
Unroofing of intramural RCA
189
What is a risk with a small PDA that is hemodynamically insignificant?
Possible endocarditis
190
What is Scimitar syndrome?
Anomalous connection of the right lower pulmonary vein to the IVC
*Sometimes the right upper/middle pulmonary veins also connect to the IVC
191
What % of cases of Scimitar syndrome have associated intracardiac CHD?
25%
*Usually an ASD
192
True or False: In the majority of cases of Scimitar, the atrial septum is intact?
True
193
Why is the cardiac silhouette shifted rightward in Scimiatr?
Right lung hypoplasia
194
Describe the right lung in Scimitar
-Right lower love hypoplasia with sequestration and arterial supply from a vessel originating from the descending aorta
195
What is the ECG likely to show in an adult patient with cc-TGA?
Complete heart block
*By 40, complete heart block and need for pacemaker is common in patients with cc-TGA
196
Q waves in I and aVL?
ALVAPA
197
Northwest/left QRS axis?
AVSD
198
What would you see on the ECG in someone with Ebsteins or s/o VSD/ToF repair?
RBBB
199
What should you consider in a teenage with stridor during exercise (symptoms present throughout life) and periodic cyanosis as a baby?
LPA sling
200
Describe an LPA sling
- Origin of LPA is from the RPA (not at the level of the true PA bifurcation
- LPA crosses between the bronchus and esophagus
201
What is seen on a barium esophagram in an LPA sling?
Anterior indentation of the esophagus
202
What is thought to cause congenital complete AV block?
Transplacental passage of autoantibodies against Ro/La intracellular ribonuclear proteins from Mom (possible with autoimmune diseases like SLE or Sjogrens)
203
Permanent pacemaker implantation is indicated for congenital 3rd degree AV block in what situations independent of symptoms?
- Wide QRS escape rhythm (unreliable, can cause abrupt pauses leading to arrest)
- Complex ventricular ectopy
- Ventricular dysfunction
204
What can help with ventricular dysfunction in the setting of complete congenital heart block?
Pacemaker
*ACEi can be used in combination with device therapy
205
True or False: All patients with congenital AV block should have a screening EP study in adolesence
False- No indication
206
In patients with CHD, what are 2 class I indications for ICD when other remediable causes (hemodynamic or arrhythmic) have been excluded?
- Spontaneous sustained VT
| - Unexplained syncope with inducible sustained hypotensive VT
207
What type of ICD/Pacemaker is needed for patients with cyanosis and intracardiac shunts?
Epicardial
208
Why should you not use transvenous leads in a patient with an intracardiac shunt?
Transvenous leads have a >2 fold increased risk of systemic thromboemboli independent of warfarin or ASA
209
What is a unique atrial arrhythmia seen in patients who have had extensive scarring of the atria?
Incisional atrial flutter
210
What % of patients can have incisional atrial flutter following Fontan?
7%
211
Which has slower rates, atrial flutter or IART?
IART
212
What is the difference on ECG between IART and Flutter?
- IART has an isoelectric baseline between 2 consecutive P-waves
- Flutter has constant activity (causes saw-tooth pattern)
213
What should you consider for someone with baseline bradycardia and no variation in heart rate?
IART- Get an ECG
214
What is the management for IART?
Cardioversion
*Need to check for thrombus or have anticoagulation prior to cardioversion
215
What is the most common presentation of a small-moderate ASD?
Exercise intolerance (2nd decade)
216
What are common exam findings of an ASD?
- Fixed, split S2
| - Pulmonary flow murmur (from L-R atrial shunting)
217
What is often seen on the ECG with an ASD?
Incomplete RBBB
rSR' in V1
Possible RAE
218
What is helpful to diagnose an ASD in an adult or obese patient?
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
True or False: Any patient who survived cardiac arrest due to non-reversible causes should have an ICD
True
220
When could ablation be considered over ICD in someone with v-tach?
If it is slow, stable and monomorphic
221
What is the incidence of ICD complications?
30%
222
Which is more effective in preventing recurrent arrhythmias and SCD, anti-arrhythmics or ICD?
ICD
223
True or False: B-blockers, amiodarone and sotalol can help to decrease the risk of appropriate ICD shocks in a patients with ToF?
False
224
What type of ICD is preferred in patients who don't have a specific indication for surgery to replace the pulmonary valve?
Transvenous
225
True or False: Transvenous pacemaker can be done in someone with free PI
True
226
What can cardiac resynchronization therapy with biventricular pacing help to do in someone with ToF?
- Improve hemodynamics and cardiac symptoms
| - Decrease QRS duration
227
Is biventricular pacing an alternative to ICD in someone with a survived cardiac arrest?
No
228
What are some ECG clues to IART?
- 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
What is needed in all atrial fibrillation or atrial flutter?
Antithrombotic therapy
*Coumadin and rivaroxaban
230
How should atrial fibrillation or flutter be managed in a new diagnosis with stable hemodynamics and no RVR?
Anticoagulate for 3 weeks then cardioversion
231
What is an advantage of rivaroxaban over coumadin?
No need to check INR to adjust dose
*Similar efficacy and safety profile to Coumain
232
What role does digoxin or beta blockers play in atrial fibrillation?
Slow conduction in the AV node and prevent rapid conduction of atrial arrhythmia
233
Irregularly irregular rhythm
A-fib
234
What might cause syncope in a-fib with RVR?
Catecholamine surge increasing ventricular rate
235
True or False: Decreased function can result from a-fib with RVR?
True
236
In a patient with syncope and depressed function due to a-fib with RVR, what needs to be done?
TEE to check for large thrombus then cardioversion- Don't really have room to anticoagulate for 3 weeks then cardiovert
237
How does TEE assessment for thombus factor into decision to cardiovert for a-fib?
- 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
What types of patients need ACHD center for non-cardiac surgery?
- Fontan
- Severe PAH
- Cyanotic CHD
- Complex CHD
- Malignant arrhythmia
239
When should patients s/p AVSD repair have surgical intervention for LVOTO?
- Maximal instantaneous gradient >70mmHg
| - Lower gradient in association with significant MR or AI
240
What is a class 1 indication for surgical intervention of supravalvar AS?
Mean gradient 50mmHg or higher in asymptomatic patients
241
Patients with supravalvar AS and a gradient <50mmHg should be considered for surgical intervention in what circumstances?
- Symptomatic
- LVH
- Planning pregnancy
242
How often should asymptomatic patients with a max instantaneous pulmonary valve gradient >30mmHg have a follow-up echo?
Every 2-5 years
243
Noninvasive testing for ischemia provocation is recommended how often for patients after ASO?
Every 3-5 years
244
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
True
