Cardiology Boards Flashcards
1
Q
What fetal finding is thought to lead to TOF/absent pulmonary valve?
A
Premature PDA closure/absent PDA
2
Q
List the 4 causes of long RP tachycardia
A
- Sinus tachycardia
- Atrial tachycardia
- Atypical AVNRT
- PJRT
3
Q
Which valve is Libman-Sacks lesion associated with? What disease is Libman-Sacks lesion associated with?
A
Mitral valve, SLE
4
Q
Adenosine causes what action on the coronary vessels?
A
Vasodilation (low O2 > adenosine monophosphate (can’t generate ATP) > adenosine > vasodilation)
5
Q
NO > cGMP and prostaglandin both cause smooth muscle what?
A
Smooth muscle relaxation
6
Q
Which receptors release Ca from the sarcoplasmic reticulum?
A
Ryanodine receptor
7
Q
Which receptors allow Ca into the myocyte?
A
L-type voltage gated Ca channel
8
Q
What’s the most common form of bicuspid aortic valve?
A
Fusion of R-L > R-non > L-non
9
Q
List two equations for resistance.
A
R = deltaP/Q
R = (8xlengthxviscosity)/pi*R^4
10
Q
Which of the aortic arches is each structure derived from:
carotid
arch
PAs/PDA
subclavian
A
carotid (III)
arch (IV)
PAs/PDA (VI)
subclavian (VII)
11
Q
What percent of TOF patients have an ASD/PFO? Right aortic arch?
A
80% and 25%
12
Q
Most common coronary arrangements in TGA?
A
Normal (70%)
LCx from RCA (16%)
Single right, inverted RCA/LCx
13
Q
Where is the transverse sinus?
A
Between great vessels and atrial walls (looks like LCA)
14
Q
Where is the oblique sinus?
A
Between posterior LA and reflections of SVC and pulmonary veins
15
Q
What is the normal ratio of septal to LV free wall thickness?
A
1.1 (increases with age to 1.2)
Ratio of LV:RV thickness is 3
16
Q
Most likely structure ruptured in trauma?
A
RV. If atrium is ruptured it’s typically the appendage.
17
Q
Repetitive exercise results in what changes for resting HR, resting BP, blood volume, stroke volume, myocardial O2 demand?
A
HR - decreased
BP - decreased
blood volume - increased
SV - increased
myocardial O2 demand - decreased
18
Q
How much displacement of the tricuspid valve is consistent with Ebstein anomaly?
A
8 mm/m2
19
Q
List AVSD types.
A
Complete
Intermediate - large ASD/VSD, separate
Transitional - small VSD, large ASD, separate
Partial - no VSD, large ASD, separate
20
Q
Most common form of HLHS?
A
MA/AA (36-46%)
MS/AA (20-30%)
MS/AS (13-26%)
21
Q
Name parts of a sarcomere
A
A band - thick (myosin) filament
I band - thin (actin) filament
H zone - central clearing
22
Q
Most common morphologies of truncal valve?
A
Tricuspid (70%)
Quadricuspid (20%)
Bicuspid (10%)
Always in continuity with mitral valve
23
Q
80% of Ebstein patients also have what?
A
ASD
24
Q
Which two structures have the lowest SaO2 in the fetus?
A
SVC and coronary sinus
25
What is the most common great artery configuration in DORV?
Side-by-side with aorta rightward (2/3)
26
What is deficient in Pompe disease?
acid ɑ glucosidase (GAA)
27
Premature ductal constriction in the fetus results in what postnatal finding?
PPHN (RV hypertrophic and dilation)
Ductal pulsatility index <1.9 suggestive
28
What are the major and minor criteria of acute rheumatic fever?
Polyarthritis, carditis, subcutaneous nodules, erythema marginatum, syndham chorea
CRP>3, ESR>60, fever, polyarthralgia, long PR
29
List the three indications for surgical repair of double chambered RV
1. Pressure gradient of at least 40 mmHg
2. Worsening pressure gradient
3. Symptoms
30
How often should adults with repaired coarctation undergo cross-sectional/3D imaging?
Every 5 years (looking for aneurysm)
31
Ventricular dilation and low resting EF in otherwise healthy teen can be what?
Athletes heart (volume overload from high output leads to dilation)
32
Differential for a change in QRS?
Intermittent preexcitation
Aberrant conduction
Ventricular rhythm
Twin AV nodes
33
Ecg patterns with digoxin toxicity?
Slopes diffuse ST depression
AV block
Bidirectional VT
34
Treatment for digoxin toxicity?
Antidote (often refractory to defibrillation)
35
What happens to digoxin level with amiodarone?
Increases
36
When is an upright T wave in V1 normal?
1st week of life
Adolescence and beyond
37
Severity of pulmonary valve stenosis by peak velocity/gradient?
Mild: <3 m/s; peak gradient, less than 36 mm Hg
Moderate: 3-4 m/s; peak gradient, 36 to 64 mm Hg
Severe: >4 m/s; peak gradient, greater than 64 mm Hg
38
What are the anatomic borders of the aortic isthmus?
Left subclavian and ductus arteriosus
39
Most common coronary abnormality in TOF?
Conal branch (accessory LAD) from RCA 10-15%
LAD from RCA coursing anteriorly to RVOT 5%
40
Most common great artery relationship in tricuspid atresia?
Normally related (75%)
41
How is Ca2+ removed during myocyte relaxation?
Ca2+ ATPase SERCA pumps on sarcoplasmic reticulum
42
What percent of patients with d-TGA have a VSD?
40-45%
43
Which atria are the limbus and valve of the fossa ovalis in?
limbus - RA
valve - LA
44
When is fetal hemoglobin (alpha/gamma) replaced with adult hemoglobin (alpha/beta)?
3 months of age
Fetal has higher affinity for O2
45
How does norepinephrine activate beta 1 receptor?
Gs subunit > activates adenylate cyclase > ATP to cAMP > activates protein kinase a
46
What causes an ostium primum ASD?
Endocardial cushion failure
47
LSVC is persistence of what embryologic structure?
Left horn of the sinus venosus
48
How much deoxyhemoglobin must be present for visible cyanosis?
5 g/dL (so may not be visible in anemic patients)
49
What protein binds calcium allowing cross bridges to form?
Troponin C
50
Living at altitude has what effect on PA pressure?
Elevated, especially with exercise (higher incidence of PDA)
51
What does second heart sound like in d-TGA?
Loud, single (because of anterior aorta)
52
DORV is most commonly associated with aortic or pulmonary stenosis?
Pulmonary stenosis (half)
53
Contractile myocytes (not smooth muscle) are found in what vessels?
Pulmonary veins just before insertion into LA
54
Most common type of truncus?
Type I (MPA from left/posterior side of truncus) 50-65%
Type II 30-45%
Type III 5-10%
RAA in 35%
55
Which PA is most commonly absent in truncus and TOF?
TOF - opposite side from aortic arch
Truncus - same side from aortic arch
56
Which embryologic aortic arch typically regresses?
Fifth
57
Borders of the triangle of Koch?
Coronary sinus ostium, septal leaflet of TV, tendon of Todaro
58
What is the valve at the junction between the great cardiac vein and the coronary sinus?
Vieussens valve
59
What is the pH change for acute respiratory acidosis?
Decrease by 0.08 for each increase in PaCO2 by 10 mmHg
60
Metabolic compensation for respiratory acidosis
Bicarb increase by 3.5*(change in PaCO2/10)
61
What is negative neurodevelopment effect of loop diuretic?
Ototoxicity, related to peak level (slowing infusion may be useful), bumetanide less toxic
62
What is responsible for the high PVR in fetus?
Low alveolar and blood oxygen tension
63
What is the coronary supply of the AV node?
Right coronary artery
64
What is the law of Laplace?
Wall Tension = pressure*radius for a thin-walled sphere or cylinder
Wall Stress = Wall Tension / 2*thickness
Wall Stress = pressure*radius / 2*thickness
65
Steps of the actin/myosin power stroke?
-Ca2+ binds troponin C, tropomyosin moves out of the way so actin binding site can form
-Crossbridge forms (baseline, non-energy using)
-Phosphate leaves myosin strengthening the bond
-Adenosine leaves myosin--power stroke
-ATP binds myosin causing separation
-ATP hydrolysis causing relaxation of myosin head
66
When to suspect familial hypercholesterolemia
Fasting LDL >160 + fhx premature coronary artery disease/elevated LDL in first degree relative
LDL>190 in child under 20 has 80% chance of familial hypercholesterolemia
Mutations in genes for LDL receptor, apolipoprotein B, or PCSK9 (proprotein convertase subtilisin/kesin)
Statin inhibits 3-hydroxy-3-methylglutaryl coenzyme A reductase (rate-limiting enzyme in cholesterol biosynthesis), upregulates LDL receptors in liver
Goal with treatment LDL<130
Young: pitavastatin, pravastatin
Potent: atorvastatin, rosuvastatin
Downsides: myopathy, transaminitis, teratogenicity, GI upset
67
Cardiac diagnoses associated with pectus excavatum
Mitral valve prolapse or aortic root dilation (isolated or with Margan, Loeys-Dietz, Ehlers-Danlos)
68
What is the law of Laplace?
Wall Tension = pressure*radius for a thin-walled sphere or cylinder
Wall Stress = Wall Tension / 2*thickness
Wall Stress = pressure*radius / 2*thickness
69
Steps of the actin/myosin power stroke?
-Ca2+ binds troponin C, tropomyosin moves out of the way so actin binding site can form
-Crossbridge forms (baseline, non-energy using)
-Phosphate leaves myosin strengthening the bond
-Adenosine leaves myosin--power stroke
-ATP binds myosin causing separation
-ATP hydrolysis causing relaxation of myosin head
70
When to suspect familial hypercholesterolemia
Fasting LDL >160 + fhx premature coraonary artery disease
Elevated LDL in first degree relative
LDL>190 in child under 20 has 80% chance of familial hypercholesterolemia
Mutations in genes for LDL receptor, apolipoprotein B, or PCSK9 (proprotein convertase subtilisin/kesin)
Statin inhibits 3-hydroxy-3-methylglutaryl coenzyme A reductase (rate-limiting enzyme in cholesterol biosynthesis), upregulates LDL receptors in liver
Young: pitavastatin, pravastatin
Potent: atorvastatin, rosuvastatin
71
Cardiac diagnoses associated with pectus excavatum
Mitral valve prolapse (isolated or with Margan, Loeys-Dietz, Ehlers-Danlos)
72
What is Danon disease?
Glycogen storage disease IIb
Hypertrophic cardiomyopathy, intellectual disability, WPW, skeletal myopathy
LAMP2 mutation (lysosomal associated membrane protein)
73
Fabry Disease
X-linked lysosomal disorder of glycosphingolipid metabolism (deficient or absent lysosomal α-galactosidase A activity, GLA gene)
Burning/paresthesias; LVH, short PR, fibrosis causing systolic dysfunction and arrhythmia, valve insufficiency
Enzyme therapy can halt disease progression
74
In combination with R aortic arch, what causes complete vascular ring?
Draw from the top in axial view: aberrant L subclavian and L PDA
To determine whether there is a vascular ring present, you must know arch sidedness, aortic arch branching, and side/presence of PDA
75
What is the Belhassen VT pattern?
RBBB, left axis deviation, Q in 1 and aVL (left to right), inferior leads negative QRS (bottom to top)
76
Electrocardiographic sign for ALCAPA?
Deep Q waves in I and aVL
77
What is Danon disease?
Glycogen storage disease IIb
Hypertrophic cardiomyopathy
78
Fabry Disease
X-linked lysosomal disorder of glycosphingolipid metabolism (deficient or absent lysosomal α-galactosidase A activity)
Burning/paresthesias; LVH, short PR, fibrosis causing systolic dysfunction and arrhythmia, valve insufficiency
79
In combination with R aortic arch, what causes complete vascular ring?
Draw from the top in axial view: aberrant L subclavian and L PDA
80
What is the ligament of Marshall?
Vestige of left cardinal vein, anterior to pulmonary veins, LPA
81
If there is tricuspid-aortic continuity, what type of VSD is present?
perimembranous
82
"Pentalogy" of Fallot adds what defect present in 80% of TOF patients?
ASD or PFO
83
What is effect of ANP on kidney?
-Dilating the afferent arteriole and constricting the efferent arteriole
-Distal tubules to decrease sodium resorption
84
Cardiac situs is determined by the position of what structure?
right atrium
85
What is the ligament of Marshall?
Vestige of left cardinal vein, anterior to pulmonary veins, LPA
86
Which great vessels are intra-pericardial?
Ascending aorta, MPA, terminal SVC
87
Borders of the transverse sinus?
atria vs. great vessels
88
Single, firm, intramural tumors involving the ventricular free wall or septum is likely to be what?
Fibroma
89
HOCM murmur louder/softer
Louder: isoproterenol, nitroglycerin, exercise, standing from squat, straining portion of Valsalva
Softer: phenylephrine
90
What CHD is phenylketonuria associated with?
Left-sided lesions (HLHS, coarct)
Septal defects
TOF
91
Superior axis (left or NW) is associated with which CHD?
AVSD, tricuspid atresia
92
Most common viral causes of myocarditis?
Coxsackie and adenovirus
Rare: CMV, parvo, fluA, HSV, EBV, HIV, RSV
93
IAA without a VSD is associated with what CHD?
AP window
Type A interrupted arch (think of it like severe coarctation)
Type B most common in DiGeorge
Type C very rare
94
Still's mumur is best heard in what position?
supine
95
What causes an ANTERIOR esophageal indentation?
PA sling
96
Well-circumscribed, noncapsulated, intramural, or intracavitary nodules, bright appearance on echo
Rhabdomyoma
97
Retinoic acid results in what category of defect?
Conotruncal
98
Obstruction, emboli, and B symptoms attached to atrial septum?
Myxoma - most common adult cardiac tumor, second most common childhood cardiac tumor, 75% located in LA
99
Heart disease associated with WPW?
Hypertrophic cardiomyopathy
LVNC
Ebstein anomaly
ccTGA
100
What systolic murmur increases in intensity after PVC?
Aortic stenosis
101
Most common CHD associated with ccTGA?
VSD (80%)
102
Additional echo finding to look for in dTGA with VSD?
Sub-pulmonary stenosis (LVOTO) - needs REV or Nikaidoh
103
Taussig-Bing anomaly
DORV, side by side GA, subpulmonary VSD (TGA physiology, cyanotic)
104
Valsalva effect on murmurs
Forced exhale - decreases venous return (so most murmurs decrease, exception is HOCM and MR)
105
Constrictive pericarditis vs. restrictive cardiomyopathy
Restrictive CM: atrial enlargement, RVSP>50mmHg, no respiratory variation, left sided pressures more elevated (PCWP and LVEDP at least 4 mmHg higher than RAP and RVEDP, can bring out difference with volume load
Constrictive: septal bounce, equal RVEDP and LVEDP, normal PVRi, RSVP<50mmHg
106
What portion of the aorta is at risk of enlargement with bicuspid aortic valve?
Ascending (root with Marfan/Loey's Deitz)
107
Natural history of rhabdomyomas in tuberous sclerosis?
Most have complete resolution
108
Friction rub is loudest in what position?
Leaning forward
109
Q waves appear where in ccTGA?
right precordial leads (as opposed to left precordial leads as in normal patient)
110
What is most common CHD in congenital Rubella?
pulmonary stenosis and PDA
111
Most common cause of sudden death in athletes?
HOCM (44%), coronary anomalies (17%)
112
Giant cell myocarditis
- Viruses, toxins, drugs
- Often presents with fulminant myocarditis (biventricular dysfunction without dilation)
- Steroids are mainstay of treatment along with immunosuppression (cyclosporin); rituximab and anakinra second line
- Worse prognosis than other forms - can have giant cell infiltration of donor heart
113
Consequences of premature ductal constriction?
RV dilation/hypertrophy
Hydrops
PVR up (more pulm flow)
RV hypoplasia/PS (right to left at asd)
114
What is treatment for familial chylomicronemia syndrome?
Reduce fat intake to <10-15% of calories
No medications currently approved by FDA
Genetically mediated deficiency in lipoprotein lipase
115
Natural history of SVT in infants
Most resolve after 1st year
116
Differential diagnosis of short RP tachycardia
Atrioventricular reentrant tach
Typical AVNRT
Junctional tachycardia
117
What causes increased a wave in RA and LA tracing?
Elevated RVEDP
Tricuspid stenosis
Complete heart block
Elevated LVEDP
Mitral stenosis
AV dyssynchrony
118
Fick equation?
Q (L/min/m2) = VO2 (mL/min/m2) / [(Hgb g/dL x 1.36 mL O2 / g Hgb x 10 dL/L x Sat + 0.03 mL O2/L/mmHg x PaO2 mmHg) - same for lower Sat]
119
Fick equation?
Q (L/min/m2) = VO2 (mL/min/m2) / [(Hgb g/dL x 1.36 mL O2 / g Hgb x 10 dL/L x Sat + 0.003 mL O2/L/mmHg x PaO2 mmHg) - same for lower Sat]
120
Qeff
Qeff = VO2 / content in PV - MV
121
Easy calculation for PVR/SVR when Qp:Qs = 1
Transpulmonary gradient - Transsystemic gradient
122
Allergic reaction from Amplatzer device most likely caused by
Nickel allergy (titanium-nickel compound)
123
When are you unable to calculate Q?
Multiple sources of flow with different saturations
124
Patients taking NPH insulin are at risk of hypersensitivity to what medication used in cardiac surgery?
Protamine
125
What is incidence of device erosion for Amplatzer ASD occluder?
1 / 1,000 (0.1%)
Gore has none
126
Which has highest incidence of acute aortic wall injury as treatment for coarctation in older kids - balloon, stent, or surgery?
Balloon
127
Restrictive vs constrictive
The following features are more suggestive of restriction rather than constrictive pericarditis or tamponade: RVSP >50 mm Hg, LVEDP − RVEDP >4 mm Hg, PCWP − mean RAP >4 mm Hg, and RVEDP/RVSP <0.3, normal RA respiratory variation
128
Restrictive vs constrictive
The following features are more suggestive of restriction rather than constrictive pericarditis or tamponade: RVSP >50 mm Hg, LVEDP − RVEDP >4 mm Hg, PCWP − mean RAP >4 mm Hg, and RVEDP/RVSP <0.3
129
ACHD cath balloon valvuloplasty indications
mean >40 mmHg asymptomatic
mean >30 mmHg with symptoms or dysfunction (aortic is cath only, for intervention it's 60 and 50 with < moderate calcification/regurg)
130
Class I indications for pediatric pulmonary valvuloplasty
- Critical PS (cyanosis, PDA dependence)
- Peak gradient >= 40 mmHg (echo or cath)
- Significant stenosis with RV dysfunction
*<15% risk of recurrence
131
Indications for transcatheter ASD closure
- Hemodynamically significant, suitable anatomy (class I)
- TIA/stroke or symptomatic cyanosis with transient R-L (class IIa)
- Hypercoagulable, transvenous pacing system (class II)
132
What's associated with 3-methylglutaconic aciduria type II (Barth syndrome)?
Neutropenia
Dilated cardiomyopathy / LVNC
Proximal skeletal myopathy
Growth failure
Endocardial fibroelastosis
tafazzin (TAZ) mutation, X-linked
133
What's associated with 3-methylglutaconic aciduria type II (Barth syndrome)?
Neutropenia
Dilated cardiomyopathy
Proximal skeletal myopathy/weakness
Hypotonia
Growth failure
Endocardial fibroelastosis
3-methylglutaconic aciduria
134
22q11.2
Mutation in TBX1
DiGeorge
IAA-B, truncus, right aortic arch, TOF, conotruncal VSD
135
JAG1
Alagille
Peripheral PA hypoplasia, TOF
136
7q11, ELN mutation
Wiliams
Supravalvar AS, PPS
137
PTPN11
Noonan
PS, HCM, PAPVR
Nuchal translucency, cystic hygroma, polyhydramnios
138
Turner (XO)
Coarctation, bicuspid aortic valve, HLHS, VSD, PAPVR (up to 25%)
Can have aortic dilation and dissection, need MRI/CT at some point
Non-cardiac: hypertension (horseshoe kidney), obesity, dyslipidemia, type II diabetes, sensorineural hearing loss, autoimmune disorders such as celiac
139
Right aortic arch is most often seen in which form of TOF?
TOF/PA/MAPCAS (PA with VSD)
140
Most common post-op issue following sano shunt?
PA stenosis
141
In what scenario do you worry about retrograde coarctation?
Ductal stent in hybrid procedure
142
What is a classic Glenn?
RSVC to RPA, disconnect RPA from LPA
143
What is a charged Glenn?
Same as pulsatile Glenn - pulm valve left patent
144
LPA stent for Glenn
- Should only be considered if can be dilated to adult size except in extreme situations
- Stent placement in Glenn want gradient < 3-5 mmHg
145
Treatment for fetal SVT
Digoxin first line, high level (2)
Fetal transfer decreases in presence of hydrops
Refractory SVT or with hydrops: flecainide/sotalol (better fetal transfer or amio (poor fetal transfer)
146
Treatment for fetal SVT
Digoxin first line, high level (2)
Fetal transfer decreases in presence of hydrops
147
Risk factors for sudden death in HOCM
- Septal thickness >3cm
- Family history (?)
- Syncope, non-neurally mediated
- BP decrease or inadequate rise with exercise
- NSVT
- Late gadolinium enhancement on MRI
148
Amiodarone increases effects of what medications?
Warfarin, digoxin, phenytoin, class I antiarrhythmics
149
Treatment for Lyme disease
doxycycline
amoxicillin and cephalosporins can also be used
Hospitalize and IV abx if PR>300, 2nd or 3rd degree block; if don't need hospitalization, PR <300, doxy ok
150
What are symptoms of Kearns-Sayre Syndrome?
Mitochondrial myopathy
ophthalmoplegia, ptosis, retinal degeneration, ataxia, heart block, protein in CSF, short stature or other endocrinopathies (diabetes, hypoparathyroid, growth hormone deficiency)
Pacemaker indicated in presence of bifascicular block
151
Prognosis of Long QT with 2:1 AV block
Poor (50% mortality in infancy)
Beta blocker and pacemaker
152
First line treatment for WPW with SVT?
Propranolol
Second line: flecainide
153
First line treatment for WPW with SVT?
Propranolol
154
Normal V1 T wave
positive at birth
negative 1 week - teenage
positive after teenage
155
P-wave morphology in PJRT
Deeply negative p-waves in II, III, and aVF
156
Most common sequelae of maternal amiodarone
Neonatal thyroid dysfunction
157
Class I indications for pacemaker in kids
1. High grade block or sinus node dysfunction AND symptoms, ventricular dysfunction, or low CO
2. Post-op high grade block persisting >7d
3. Congenital 3rd degree block with wide QRS escape, complex ventricular ectopy, rate less than 50 bpm in asymptomatic infant, or rate less than 70 bpm in CHD
- For sinus pause and dizziness without syncope, neurogenic, try other therapies first
158
Class I indications for pacemaker in kids
1. High grade block or sinus node dysfunction AND symptoms, ventricular dysfunction, or low CO
2. Post-op high grade block persisting >7d
3. Congenital 3rd degree block with wide QRS escape, complex ventricular ectopy, rate less than 55 bpm in infant, or rate less than 70 bpm in CHD
159
What does third letter of pacemaker notation mean?
I - inhibit, if pacemaker senses an event, will inhibit the pacing function
T - track/trigger, if pacemaker senses an event, will pace in response
D - dual, both
DDI - will pace at lower limit, but can't pace above which is helpful for not tracking atrial arrhythmia
160
What does third letter of pacemaker notation mean?
I - inhibit, if pacemaker senses an event, will inhibit the pacing function
T - track/trigger, if pacemaker senses an event, will pace in response
D - dual, both
161
Most common side effects of beta blockers in kids
Behavioral changes, depression, mood swings most common
Lightheadedness, tiredness, headache, nightmares, difficulty sleeping, heartburn, diarrhea, constipation
162
Define AH jump
Change of >50ms with 10ms change in premature stimulus
(suggests dual AV node pathways)
163
Genetic cause of CPVT
Ryanodine receptor gene (RYR2) or calsequestrin 2 gene (CASQ2)
164
Adverse effects of amiodarone
Photosensitivity
Thyroid dysfunction
Weakness/peripheral neuropathy
Corneal microdeposits
Elevated hepatic enzymes
165
Which electrolyte derangements make digoxin toxicity worse?
Hypokalemia
Hypomagnasemia
166
Treat digoxin toxicity
SVT - beta blocker (esmolol)
VT - lidocaine or phenytoin
Antibody
Correct hypoK and hypoMg
Temporary pacing
167
What is rate responsiveness in pacemaker?
Increases minimum rate with activity
168
What is effect of magnet over ICD?
Disables shock therapy, does not change pacing (only as long as magnet is in place)
Different from magnet over pacemaker which enables asynchronous pacing
169
Causes of left axis deviation on ECG?
AVSD (or primum ASD)
Tricuspid atresia
WPW
170
Impact of hypercalcemia on ECG?
Short QTc
171
Normal HV interval?
35 to 70 ms
Short HV is <25 ms, indicates an alternative method of conduction other than AV node
172
Prognostic value of loss of preexcitation in a single beat on exercise test
Accessory pathway is less likely to rapidly conduct to the ventricle in afib, less likely to cause sudden death
173
Treatment for Brugada patients with syncope or sudden death
ICD placement
174
Irregular wide complex tachycardia
Think atrial fibrillation with BBB or preexcitation
Avoid adenosine - don't want to promote conduction down the accessory pathway
175
What is a Mahaim fiber tachycardia?
antegrade only accessory pathway
176
Provocation of Brugada syndrome?
High lead placement (V1, V2)
Procainamide
Fever
177
Class I indications for biventricular pacing (resynchronization)
LBBB, QRS > 150ms, NYHA class II or worse
Usually only beneficial when EF < 35%, only indicated after medical therapy is optimized
178
NKX2.5
Hear block and ASD
179
PKP2
Arrhythmogenic right ventricular cardiomyopathy
180
NOTCH1 mutations
Left-sided disease - aortic valve stenosis, bicuspid aortic valve, coarctation of the aorta, HLHS
181
Transvenous pacing system contraindicated in what CHD?
Fontan - risk of thrombus in systemic circulation
182
Ebstein's ECG finding without pre-excitation
RBBB (atresia, short length, narrow caliber, fibrosis)
183
Why is carvedilol good for heart failure?
Non-selective - block alpha activity too
184
What is Ashman phenomenon?
Aberrancy during tachycardia due to decreased cycle length
185
What happens to risk of atrial fibrillation if you ablate WPW pathway?
Decreases - it’s thought that their atrial fib starts as svt and degenerates
186
Risk factors for atrial fibrillation
Thyroid abnormalities
Drugs of abuse
First degree relative with a fib
Prolonged PR at baseline
Obesity
OSA in adults
187
What is post-ligation cardiac syndrome?
After PDA closure, depressed LV function/hypotension due to:
1. Sudden increase in afterload
2. Sudden decrease in preload
188
Cath vs. echo peak gradient
Echo peak instantaneous pressure gradient higher than cath peak to peak (echo mean typically correlates more closely with cath peak to peak)
189
Indications for pediatric aortic valvuloplasty
Catheter gradient >50mmHg
Catheter gradient >40mmHg with symptoms, ST changes, pregnancy
Infants with depressed LV systolic function or critical AS (requiring PDA) regardless of measured gradient
Asymptomatic Doppler mean >40 mmHg
Desire to participate in competitive sports or contemplating pregnancy, Doppler mean >30 mmHg
Catheter peak to peak >60 mmHg
Desire to play competitive sports or become pregnant, peak to peak >50 mmHg
Symptoms or ECG changes peak to peak >50 mmHg
Not recommended for asymptomatic with peak-to-peak <40 mmhg unless cardiac output is impaired
190
Most common genes implicated in familial hypercholesterolemia
LDLR
apolipoprotein-b (apo-B)
pro-protein convertase (PCSK9)
LDL receptor adapter protein (LDLRAP1)
191
Criteria for clinical diagnosis of homozygous familial hypercholesterolemia
-Genetic confirmation
-Untreated LDL>500, treated LDL>300
-Cutaneous or tendon xanthomas before age 10
-Untreated elevated LDL in both parents
192
Therapies for homozygous familial hypercholesterolemia
Usual treatment (statins, ezetimibe, bile acid binding resins) are often ineffective
Lomitapide (microsomal transport protein inhibitor)
Mipomersen (antisense oligonucleotide against apo(B) mRNA)
Evolocumab (PCSK 9 inhibitor)
Inclisiran (small interfering RNA inhibits PCSK9)
Evinacumab (monoclonal antibody inhibits angiopoietin-like protein 3)
Nothing FDA approved for kids under 10years
193
Cardiac manifestations of NF1
Pulmonary valve stenosis
Mitral valve prolapse
Septal defects
Hypertrophic cardiomyopathy
194
Cardiac manifestations of alpha galactosidase A mutation
Fabry disease - LVH, valvular abnormalities, systemic hypertension, atherosclerotic disease of coronary and cerebral arteries
195
Vascular ring components with RAA
RAA, left-ductus, and aberrant left subclavian IF ductus inserts on the proximal descending aorta (with mirror image branching and insertion at base of L subclavian, not a ring)
Prenatal diagnosis: 80% RAA-ALSCA, 20% double aortic arch
Postnatally/surgically DAA increased proportion because more symptomatic
33% of RAA-ALSCA will be symptomatic, 75% of DAA will be
196
Catheter courses
Just anterior to spine - venous (IVC, azygous most posterior OVER vertebrae)
If catheter passes through pulm or aortic valve and aortic is leftward of pulmonary, think ccTGA
197
Effect of azygous vein on Glenn
Basically a giant venovenous collateral - cyanosis
198
Scimitar Syndrome
- Presentation during late adolescence or adulthood may not require surgery becuase small amount of blood shunting through hypoplastic lung so small right to left shunt
- May present in infancy with tachypnea and RV hypertension
- Transcatheter coiling of AP collaterals to improve PH
199
What is aorta/pulmonary valve position in ccTGA?
aorta is anterior and leftward of pulmonary valve
200
LSVC derives from what embryologic structure?
Left anterior cardinal vein
201
Left and right vitelline veins fuse to form what?
Portal vein
202
Mild/moderate/severe for regurgitation fractions on MRI?
<20% mild
20-40% moderate
>40% severe
retrograde volume / antegrade volume
203
What is normal Right/Left lung perfusion percent?
55% to right, 45% to left
Think 3 lobes vs. 2 lobes
204
Is lung perfusion scan safe with a right-to-left shunt?
Yes
205
Caveats for lung perfusion scan
- Can't tell you about bilateral PA stenosis (just relative R vs. L)
- Streaming issue with Fontan
- R-L shunt and AP collaterals (think PA MAPCAs)
206
First line and adjunctive therapies for PPHN?
First: iNO
Second: milrinone if LV dysfunction, sildenafil, inhaled iloprost
207
KCNH2 loss of function
Long QT type 2
Treat with nadolol
In patients with aborted arrest consider ICD and/or sympathectomy
208
Echo findings in sickle cell
LV dilation and diastolic dysfunction (high output, chronic ischemia)
209
Differential for bidirectional VT
1. CPVT
2. Long QT type 7 (Andersen-Tawil)
3. Digoxin toxicity
210
KCNJ2
Long QT type 7, Andersen-Tawil - periodic muscle paralysis and dysmorphic features
211
ECG findings in ARVC
- Epsilon waves
- T wave inversion in right precordial leads (V1 to V3)
212
Normal PDA Doppler over first days of life
Initially bidirectional
Low velocity L-R
High velocity L-R
213
How does Valsalva break SVT?
Increased intrathoracic pressure > stimulation of aortic arch/carotid sinus baroreceptors > increased parasympathetic tone
Initially decreased preload and parasympathetic stimulation but then have reflex sympathetic response
214
What are Howell Jolly bodies
Remnants of RBC nuclei normally removed by spleen - presence on smear indicates splenic dysfunction
May not be accurate in kids under 2 years - some advocate universal antibiotic prophyaxis
215
MYH7
myosin heavy chain 7, mutations associated with hypertrophic cardiomyopathy
216
Use pulmonary regurgitation Doppler to estimate PA pressures
End diastolic regurg velocity reflects pressure difference at end diastole (difference between PA and RVEDP = RA) so use Bernoulli and add RA pressure
Peak diastolic regurg velocity reflects MEAN PA pressure when added to RA pressure
RVSP = PASP in absence of pulmonary stenosis
217
Sports restriction
Define risk by static (pressure load) and dynamic (volume load), I/II/III, also consider impact/collision
Mildly to moderately dilated aorta (Z-score 2-3.5) and no features of connective tissue disorder can do low and moderate static and dynamic sports
218
Where are the 4 critical isthmuses for VT in repaired TOF?
Between VSD patch or transannular patch/ventriculotomy and TV or PV annulus
If terminates with ICD shock, likely reentrant (isthmus) as opposed to ectopic focus
219
Class I recommendations for heart failure management?
1. Diuretics to achieve euvolemia
2. Titrate ACEi to max tolerated dosing for symptomatic LV dysfunction
3. Consider mineralocorticoid antagonist
(B blocker is class IIa rec)
220
ALCAPA echo findings
-Coronary flow INTO the PA
-Reversal of flow in the left coronary
-Dilation of the RCA
-Mitral regurgitation, echobright papillary muscles
-LV dysfunction/dilation
221
Definition of accelerated ventricular rhythm
Within 10-15% of sinus rate. In a newborn with no other issues it's benign, resolves spontaneously within the first year
222
What is range ambiguity in Doppler?
Goes along with continuous wave - can't tell where the specific velocity came from (high PRF or high Nyquist limit) can't tell location
223
Grades of diastolic dysfunction on AV valve and pulm vein Doppler
1) Abnormal relaxation: E/A reversal, S/D reversal (S higher), increasing A-wave reversal
2) Mild-mod decreased compliance: pseudonormalization with short and narrow E wave, pulm vein with worse A wave reversal, near return to baseline between S and D
224
Contrast echo
With CONTRAST agent:
- Designed to pass through pulmonary capillary bed and opacify left heart
- Typical size of microspheres is 1-10 microns
- Acoustic impedance of contrast agents much lower than blood
- Contrast effect lasts for 3-5 minutes
With AGITATED SALINE:
- Don't pass through pulmonary capillaries (10-100 microns)
- Helpful to identify R-L shunt (stroke or cyanosis)
- In presence of an intracardiac shunt, 1-2 cardiac cycles; in presence of an intrapulmonary shunt, 3-5 cardiac cycles
225
Goals of acute HF management
- Decrease afterload (lower SVR as BP permits)
- Increase inotropy
- Optimize preload with diuretics if able
- PPV
- Decrease metabolic demand
226
Dose-dependent effects of dopamine
<10 microgram/kg/min, mostly B1 and B2 (chronotropy, inotropy)
High dose alpha1 and conversion to norepi
227
Infectious causes of heart block
Lyme
Rheumatic carditis
Endocarditis
Chagas
Viral myocarditis
228
Signs of shunt occlusion in Norwood
-Decrease in end tidal CO2 (ventilation without perfusion)
-Hypoxemia (no Qp)
Treatment:
1) Increase SVR - epi, norepi, phenylephrine
2) Increase O2 - supplemental O2
3) Heparinization (50-100 U/kg)
4) Reduce O2 use - sedation, paralysis
229
How do you find preexcitation on EP intracardiac tracing?
His - ventricle <30ms (measured to earliest QRS on surface ECG)
Look for first ventricular activation, can tell you where an accessory pathway is
230
Pathogenesis of ARVC
Mutations in cardiac desmosome (desmoplakin, plakophilin 2, desmogelin 2, desmocollin 2) leading to replacement of RV myocardium with fibrofatty tissue
Usually autosomal dominant
231
What is typical PVC/VT morphology for AVRC?
Superior axis (negative in II, III, aVF) and LBBB (positive in aVL)
Can have other morphologies
232
Exercise in AVRC?
Restriction from all competitive sports with possible exception of low-intensity class 1A sports because can lead to disease progression
233
Takayasu arteritis
-Inflammatory vasculitis of medium and large arteries, predominantly aorta and its branches
-Granulomatous lesions in vessels lead to stenosis, aneurysm, thrombosis, or combination
Three stages:
1) Vague symptoms for years, most commonly presents with HTN
2) Acute vasculitis
3) Chronic fibrosis of vessels "burnt-out stage," pulseless disease, may have renal artery HTN
**Can cause aortic obstruction and proximal coronary artery stenosis
234
Fontan follow up schedule
- Echo: annually
- End-organ testing by age group: preteen Q3-4y, adolescent Q1-3y, adults Q1-2y
235
Duration of afib after which have to anticoagulate/look for thrombus?
48 hours
236
Normal lipid values
Total cholesterol: acceptable <170, high >200
LDL: acceptable <110, high >130
apolipoprotein B: acceptable <90, high >110
TG: acceptable <75-90 depending on age, high >100-130
237
Management of familial hypercholestrolemia
For LDL >130 and <250, try 6mo of lifestyle change
At 6mo if <130 OR 130-190 with no FHx or other risk factors, continue
If 130-190 with risk factors or >190 initiate statin
Only treat over age 8 years
238
Guidelines to review
https://www.ahajournals.org/doi/10.1161/CIR.0b013e31821b1f10
https://www.ahajournals.org/doi/full/10.1161/CIR.0000000000000458
CARPREG (Cardiac Disease in Pregnancy)
239
Highest risk factors in pregnancy and CHD
5 general predictors:
- poor functional class or cyanosis
- prior event
- high risk valve disease/LVOTO
- systemic ventricular dysfunction
- no prior cardiac interventions
4 lesions:
- mechanical valve
- PH
- coronary disease
- aortopathy
Late pregnancy assessment
240
Dose response curve for partial agonist and noncompetitive antagonist
Noncompetitive antagonist (isoproterenol, propranolol) shifts curve to right -- higher dose for same effect
Partial agonist shifts curve to right and decreases efficacy
241
Interpretation of respiratory exam findings
Grunting - collapsed airways (consider left heart enlargement and bronchial compression)
Wheezing - narrowed small airways, which can occur from edema
Retractions - high airway resistance, negative intrapleural pressure
Tachypnea - bad compliance or metabolic acidosis
Stridor - inspiratory obstruction
242
Type 1 vs. type 2 LPA sling
Type 1 (30%) - normal trachea and bronchial bifurcation pattern, symptoms are result of airway narrowing
Type 2 - complete cartilaginous tracheal rings and bronchial tree maldevelopment (e.g. long segment tracheal stenosis, narrowing of an intermediate bronchus; much higher morbidity and mortality
Because of potentially complex bronchial anatomy need CT after diagnose on echo
243
Cardiopulmonary interactions
PPV effect on RV - consider in right sided disease, Glenn, Fontan:
- Decreased preload
- Increased afterload
- Over or under inflation can also increase afterload through higher PVR
PPV effect on LV:
- Decreases wall stress so better for LV dysfunction, mitral or aortic regurg
- Over or under distension may transiently impair LV preload
244
Pediatric aortic balloon valvuloplasty indications
1) Infant with critical AS or LV dysfunction (any gradient)
2) Catheter peak to peak >40 with symptoms
3) Catheter peak to peak >50 without symptoms
For echo correlate use mean (eg mean >50 without symptoms)
245
Genetic mutations for LQT types 1, 2, and 3
KCNQ1 (loss) - gain = short QT
KCNH2 (loss)
SCN5A (gain) - loss = Brugada
246
Nondihydropyridine CCB
Verapamil, diltiazem
Act both on heart and peripheral vasculature, in kids accidental ingestion can cause bradycardia, block, severe hypotension
See hyperglycemia because islet cells that release insulin have L-type Ca channels
Treat with insulin and dextrose, calcium, lipids to bind CCB, atropine / pacing, vasoactives, ECMO
247
Echo for chest pain indications
Always: exertional, abnormal ecg, 1st degree relative with sudden death or cm
Maybe: recent fever, family hx premature coronary disease
Don’t: reproducible with palpation, normal or no ecg
248
Flecainide toxicity
Wide QRS, long QT from wide QRS, long PR
Give lipids and sodium as sodium bicarbonate
249
Management of isolated ventricular ectopy
If >10% of beats on Holter, at risk for developing ventricular dysfunction - needs follow up echos
Outflow tract PVCs (LBBB, inferior axis meaning positive in II, III, aVF) tend to have a benign course
250
Treatment for infant with verapamil sensitive VT?
beta blocker - don't use CCB in infant
251
How to perform atrial electrogram?
1. Unipolar: connect right arm lead to atrial pacing wire, then lead I and II amplify atrial depolarization
2. Bipolar: connect right arm and left arm to atrial pacing wire, then lead I is across atrium (size?), lead II and lead III have unipolar atrial signal
252
Determine ECG rate based on small boxes
1 small box = 40 ms
Rate = 60,000 / (#sm box * 40)
253
How fast to pace for flutter
110% to 130% of flutter rate
254
Hemodynamic consequences of OSA
- Pulmonary hypertension
- Systemic hypertension
- Increased HR variability
- LV hypertrophy
255
Gorlin equation
valve area = cardiac output / (systolic ejection period x HR x 44.3 * sqrt(mean pressure gradient))
256
What are neural crest cells responsible for?
Septation of arterial trunk, semilunar valves
257
What line changes on ventricular pressure volume loop with inotropy?
ESPVR slope increases or decreases with inotropy; where you are on the line changes with afterload
258
Most common cause of death/need for early intervention in infantile marfan?
Mitral valve disease
Average life expectancy <2 years
259
Non-fasting lipid screening for patients with risk factors
Total cholesterol>200, HDL<45, or non-HDL>145 requires a follow-up fasted sample
260
Highest risk cholesterol patients
Kawasaki with aneurysm and transplant with coronary disease - threshold for statin is LDL>130; moderate risk is LDL>160
261
Treatment for fetal heart block caused by SSA/SSB antibodies
In utero steroids
IVIG
Hydroxychloroquine being investigated
262
In mom with SSA/SSB antibodies, what is risk of fetus with CHB?
2% in first pregnancy
20% in second if first baby had it
263
Treatment for newborn with complete heart block
Pacemaker if:
HR<50 without CHD
HR<60-70 with CHD
Low cardiac output/dysfunction
Complex ectopy, wide QRS
Most will need pacemaker by adolescence
264
HCM vs. athlete's heart
Ventricular dilation: athletes heart >55mm (usually smaller cavity in HCM <45mm)
LA dilation: both, but more pronounced in HCM (abnormal diastolic function)
Supranormal O2 consumption: athletes
Less septal hypertrophy in elite female athletes, so septum >13mm in female (or white athlete) much more likely abnormal; septal hypertrophy more common in endurance sports (rowing, cycling) than in isometric activities
Gray zone of LV wall thickness 13-15mm
265
Most frequent genetic mutation in patient's with TOF?
22q11 (20% of TOF)
266
Genetic cause of Alagille syndrome?
JAG1 and NOTCH2
267
Differential for short RP tachycardia
AVRT
JET
Typical AVNRT
268
Treatment for congenital JET in infancy
Amiodarone first line
Often need B-blocker and/or felcainide too
269
What embryologic processes result in criss-cross AV valves and superior-inferior ventricles?
Twisting - criss cross valves
Tilting - superior inferior ventricles
270
Stages of hypertension
Normal: <90th percentile for kids under 13, <120/80 for 13+
Elevated BP: 90-95th% or 120/80 to 95th%, for 13+ 120/80 to 129/80
Stage 1: 95th% to 95th% +12, 130/80 to 139/89
Stage 2: >95th +12 or >140/90
271
Management of HTN
All patients: UA, chem, lipids, renal US if UA/chem abnormal or <6yrs
In obese: HgbA1c, AST, ALT, lipid panel
Optional: fasting glucose, TSH, drug screen, sleep study, CBC
ABPM for elevated BP>1yr, stage 1 x3 measurements, stage 2 x2 measurements
Antihypertensives for:
-Symptomatic
-Persistent HTN despite lifestyle modification
-Stage 2 without modifiable risk factor
-LVH, CKD, diabetes
Initial followup after starting treatment Q4-6wks, then 3-4mo
272
Definition of LVH by mass
>115g/BSA (boys), >95 g/BSA (girls)
>51 g/m2
273
Differential diagnosis for wide complex tach in infant
1. Antidromic SVT
2. VT
3. SVT with aberrancy
4. Sinus/atrial/junctional tach with BBB or preexcitation
274
Hockeystick appearance of anterior mitral valve leaflet
Rheumatic heart disease
275
Tamponade criteria
TV inflow E-wave increases by >50% during inspiration
MV inflow E wave decreases by >30% during inspiration
(normal pattern - inspiration have negative intrathoracic pressure which draws blood into the right heart but in tamponade the collapse with expiration exaggerates the normal finding)
276
Nyquist limit and pulse repetition frequency
Nyquist limit = PRF/2 (highest velocity that can be accurately resolved), can increase with lower frequency transducer
277
Assessment of severity of aortic insufficiency on echo
1. Vena contracta width and ratio of vena contracta to aortic valve annulus (best - quantitative)
2. LV dilation helpful but also depends on chronicity
3. How far jet goes helpful but depends on LVEDP and jet eccentricity
4. Diastolic flow reversal and forward to reverse TVI ratio both helpful
278
Formula for myocardial performance index (Tei index)
(isovolumetric contraction time + isovolumetric relaxation time) / ventricular ejection time
279
Echo findings constrictive pericarditis
-Increased respiratory variation in mitral inflow Doppler velocity >25%; increased hepatic venous atrial systole flow reversal with EXPIRATION
-Increased E:A ratio, short E decel time
-Lateral mitral tissue Doppler normal
280
Restrictive LV physiology
E:A >2
Decel time <160ms
Decreased E'
Increased E/E' >15
281
Rate-corrected velocity of circumferential fiber shortening
VCFc = (shortening frac/ejection time)/sqrt(RR); preload independent
Stress-velocity index: relationship of VCFc to the end systolic wall stress (ESWS), load independent
282
Most common type of VSD associated with coarctation of the aorta?
Perimembranous
Next most common is posterior malalignment VSD
283
Single inlet / common inlet
Refers to the atria - single means just one atrium empties (e.g. tri atresia), common means one AVV empties both atria
284
Size of brachiocephalic/innominate vein with LSVC?
Small or absent - inversely proportional to size of coronary sinus
285
Reminder to distinguish between:
1. Diastolic dysfunction
2. Restrictive physiology
3. Constrictive pericarditis
4. Tamponade
Constrictive - exaggerated mitral filling, exaggerated A wave reversal in hepatic vein with expiration
286
What number is considered for surgical decision making in HOCM?
PEAK instantaneous Doppler in LVOT - due to late peak tends to correlate well with peak to peak in cath lab (different from fixed obstruction when use mean)
287
List TEE vs. TTE maneuvers
TEE:
multiplane
anteflexion/retroflexion
advance/retract
clockwise/counterclockwise rotate
TTE:
Tilt
Slide
Angle
Rock
288
What devices cause most artifact on MRI?
Stainless steel: PDA coil, occluder devices
Less artifact: pacemaker lead, PDA clip, nonferromagnetic stent
289
MRI sequences
Spin echo - black-blood, good for tissue characterization, image acquisition over several cardiac cycles, less artifact than other techniques
Gradient echo - faster acquisition, less contrast, more susceptible to artifact, bright-blood images
Delayed enhancement - best technique for assessing myocardial viability (fibrosis and necrotic areas appear bright)
Phase-contrast (velocity-encoded) cine - best for Qp:Qs, does not require contrast administration
T2 weighted black blood - best for edema
T1 - fatty infiltrates
T2* - best for iron overload
290
Delayed myocardial enhancement in infarct vs. myocarditis
Infarct - subendocardial or transmural
Myocarditis - subepicardial or midmyocardial, patchy
291
Best imaging for constrictive pericarditis?
Non-contrast CT
292
First and second line treatment for Long QT type 3
1. Nadolol
2. Mexilitine
293
What class of antiarrhythmics are contraindicated in long QT syndrome?
Class III (potassium channel blockers) - lengthen the QTc
294
Type A vs. Type B dissection
A involves ascending aorta (most common), B is all other types
295
Post-coarctectomy syndrome
Most common after surgical coarct repair but can happen with transcatheter - HTN from rebound sympathetic activtion, mesenteric arteritis and abdominal pain
296
POTS - postural orthostatic tachycardia syndrome
Palpitations/syncope with prolonged standing
Gradual improvement with rest
Generalized fatigue, nausea, mental clouding, dependent bluish discoloration of lower extremities
Increase in HR (>30bpm adults, >40bpm kids) without decrease in BP >20mmHg within 10min of standing
297
Risk factors for poor outcomes in Ebstein
pulmonary regurgitation on fetal echo
pulmonary atresia (functional or anatomic)
severe RA and atrialized RV dilation
increased cardiothoracic ratio
hydrops
larger TV annulus
LV dysfunction
low TR jet velocity
298
Celermajer index
(area of RA + area of atrialized RV) / (area of RV + area of left heart)
Higher number is worse
299
Associations with RAA with aberrant L subclavian
Extracardiac - gastrointestinal, urinary tract
22q11 deletion (10% of RAA patients)
Mostly asymptomatic at birth, 80% have not had surgery by 2 years of age
300
Minimum criteria for acute kidney injury
Increase in creatinine >0.3 from baseline or 1.5x baseline, UOP < 0.5 mL/kg/hr
301
Management of PVCs
>10% burden requires follow up
Exercise testing for those who can cooperate (for CPVT and ARVC) - class 1 for complex ectopy, 2a with simple ectopy
If any concerns for ARVC, MRI (2a rec) - epsilon, V1-3 T wave inversion, RV wall motion abnormality, RVOT enlargement, ectopy with LBBB and superior axis, family history
302
In patients with R-L shunt, EtCO2/PaCO2 is equal to what?
Qp:Qs
303
Aortic root dilation indication for surgery
>5.5cm or >5cm at center for excellence or with risk factors for early dissection
304
Severe aortic regurg indications for intervention if asymptomatic
LVSD >5cm, LVEDD >6.5cm
305
Exam findings with Type B interrupted arch and aberrant right subclavian
Both arms and legs desaturated (typically interrupted arch has differential cyanosis)
306
Contraindications for PDA closure with ibuprofen or indomethicin
NEC
Grade 3 or 4 IVH
Clinical bleeding
Platelet <60K
UOP<1 mL/kg/h
Cr>1.5, BUN>20
307
Holt-Oram syndrome
TBX5
Upper limb abnormalities
ASD or VSD
Conduction defects
Autosomal dominant
308
Max VO2 indexing (mL/min vs. mL/kg/min)
On treadmill, weight matters, mL/kg/min correlates better with fitness and lean person outperforms heavier person; on cycle, mL/min correlates better and heavier person outperforms lean person
309
Highest peak VO2 achieved during which type of exercise stress test?
Treadmill
310
How much does each thing increase with exercise:
HR
SV
minute ventilation
RR
VO2
HR - 2-3x
SV - 1.5-2x (least)
minute ventilation - 10x
RR - 2-3x
VO2 - 10-20x
311
Blood pressure change with isometric exercise (constant muscle length - holding a weight)
SBP and DBP both increase significantly
312
Blood pressure change with isotonic exercise
DBP decreases at least 10 mmHg
Higher BP generally with cycle ergometry (hand grip on bars)
313
Normal VO2 max?
11yo 35 mL/kg/min
Post pubertal male 45-50
Post pubertal female 35-40
Anemic patients lower
Treadmill > cycle > arm crank > hand grip
314
Expected max HR
For patients age 5 to 20, 195-215 bpm
For older: 220-age
315
Changes in max RR, tidal volume, and peak minute ventilation with age
Tidal volume and max minute ventilation increase
Max RR same (or slight decrease)
316
Power and work equations
On exercise test, usually report power
Power = work/time
1 Watt = 6.12 kilopond meters/min
MET is expression of power based on rate of oxygen consumption
1 MET = 3.5 mL of O2 consumed/kg of body weight/min
Arm cycle exercise requires 1.5x VO2 per Watt as does leg cycling
317
Risk level of regurgitant lesions with exercise testing
All regurgitant lesions are low risk
318
Explain VE/VCO2
VE = minute ventilation
VCO2 = end tidal CO2
VE/VCO2 = amount of air that must be ventilated to exhale 1L CO2
If ventilation is more efficient (less dead space), don't have to breathe as much to exhale 1L, slope decreases -- early exercise
If have R-L shunt with end tidal to PaCO2 gradient, will have to breathe more, slope rises
Slope rises with dead space or hyperventilation
319
Acetylene-helium rebreathing
Technique for measuring cardiac output (Fick principle), measures effective pulmonary blood flow
Depends on even distribution of inspired gas through the lungs (so not good in V/Q mismatch or big intracardiac shunt)
Easier to perform on cycle than treadmill
Better tolerated than CO2 rebreathing
320
Blood pressure cuff size
Bladder length 80% of arm circumference
Width 40% of arm circumference
321
Rule of nine in exercise ventilation
For every 25 watts increase in power, minute ventilation increases by 9 L/min
322
Prediction of MVV based on FEV1
FEV1 x 35 or 40
Max exertional minute ventilation is 60-80% of MVV
323
Work equation
Work = Force x Distance
unit is Newton-meter (J)
Force = Mass x Acceleration
Power = Work/Time
324
CO2 rebreathing technique
Along with acetylene-helium is a technique for measuring CO noninvasively
Vd/Vt = (PaCO2 - PeCO2) / PaCO2
Not super well tolerated, have to adjust for patient's size and exercise intensity, have to account for dead space (mouth piece, tubing)
325
What happens in pacemaker Wenckebach
Upper rate limit set at 180 bpm, once atrial rate reaches that, won't pace, so "drop beats"
326
Most common benign murmur in teenagers?
Pulmonary flow murmur - II/VI systolic ejection murmur at second left intercostal space
327
Activity restrictions in Kawasaki Disease
If normal coronaries, no restrictions in physical activity beyond 6-8 weeks from diagnosis
328
Return to sports after myocarditis including MISC
Echo, Holter and exercise ECG 3-6mo after initial illness
329
MISC vs. KD
Hypotension/shock more common in MISC (50-80%) than KD (<5%)
Coronary artery aneurysms and desquamation can occur in both
Steroids and GI symptoms more common treatment in MISC
330
Lab diagnosis of KD
Hypoalbuminemia <3
Anemia for age
ALT elevation
Platelet count >450K (after 7d)
WBC >15K
UA with >10 WBC and no bacteria
331
Aortopathy genes
FBN1
TGFBR1
COL3A1
ADAMTS10
ACTA2
FBN1 - Marfan
TGFBR1 - Loeys-Dietz and familial thoracic aortic aneurysm
COL3A1 - Ehlers-Danlos
ADAMTS10 - Weill-Marchesani
ACTA2 - familial thoracic aortic aneurysm
332
Highest risk endocarditis (need for prophylaxis)
- Prosthetic valve or prosthetic material used for valve
- Prior IE
- Unrepaired cyanotic CHD
- Prosthetic material in first 6 months post-op
- Residual defect next to prosthetic
- Valvulopathy in transplanted heart
Dental procedures, NOT respiratory procedures unless biopsy, NOT GI or GU procedures unless active infection
333
IE prophylaxis in penicillin allergic
PO azithromycin, cephalexin, clindamycin, clarithromycin
334
Toxic anthracycline dose
Within 6 years of treatment, 65% of kids who received 228 to 550 mg/m2 hav dysfunction
335
Regurgitant murmurs
Aortic insufficiency - high pitched, early diastole, best heard with diaphragm, left midsternal border, radiates to apex, decrescendo
Pulmonary insufficiency - LOW pitched, early diastole, best heard diaphragm, left midsternal or upper sternal border, radiates down sternal border; with significant PI can have to-fro
AVV regurg - mid or late diastolic, low pitched, heard with bell
336
Neurodevelopmental outcomes in CHD
Cyanotic lesions do worse, duration of cyanosis is important; single ventricle worst outcomes
Visual spatial skills are an area of specific weakness
High risk:
-Requiring surgery
-Cyanotic not requiring infant surgery (e.g. TOF, Ebstein)
-Prematurity, devel delay, genetic syndrome, hx mechanical support, need for CPR, heart transplant, postop hospitalization >14d, peri-op seizures, neuro imaging abnormalities
IQ mean TGA and TOF 2-3 points below mean, HLHS 12 points below mean
337
Signs of flecainide toxicity
Prolonged PR, signs of heart failure
Look for it at 12 months when transitioning off formula
338
Vein of Galen
More common in males
Associated lesions: none, sinus venosus defect, coarctation
339
Indications for lipid screening before typical age 9-11
1. Parent, grandparent, aunt/uncle, or sibling with MI/stroke <55 in males or <65 in females
2. Parent with total cholesterol >240
3. Diabetes, HTN, BMI>95th, smokes
4. Moderate or high risk medical condition - CKD, KD, heart tx, chronic inflammatory disease, HIV, nephrotic syndrome
340
Warfarin for non-cardiac procedures and mechanical valve
Ok to stop 48-72 hours (up to 5 days) before and resume the night after
Aortic valve doesn't require a bridge, all other valves should bridge with heparin; continue aspirin through
Risk factors could bridge: prior venous thromboembolism, atrial fibrillation, solid organ malignancy
341
Systolic clicks
Bicuspid aortic valve - click is at apex, no respiratory variation
Pulmonary stenosis - click at left upper sternal border, gets softer with increasing severity
Mitral prolapse - moves earlier with standing (decreased peload), moves later with squatting (increased preload), decreased LV contractility and increased afterload move it later
342
Brugada syndrome
More common in males, more arrhythmic events in men - higher testosterone thought to contribute
Inheritance is autosomal dominant
343
Marfan diagnosis and management
Revised Ghent criteria - positive family history with aortic root z-score >3 is enough to diagnose
Need echo at 6mo intervals; baseline MRI/CT is reasonable
If dilated, should start beta blocker or ACEi
344
Alagille syndrome facies
Broad forehead, deep-set eyes, small pointed chin
Bile duct paucity, heart disease, skeletal (butterfly vertebrae) and ocular abnormalities
NOTCH2 gene
345
Things that give higher yield for genetic testing in HCM
Younger age (<45y)
Reverse curve morphology
Wall thickness >20mm
Family history of HCM or sudden death
346
Risk factors for sudden death in HCM
History of syncope
Septal thickness >30mm or z-score .6
Family history of sudden cardiac death from HCM
Hypotension with exercise
Ventricular tachycardia
Delayed enhancement on cMRI
*ICD placement for 2+ risk factors
LVOTO severity not necessarily associated
347
Calcium disorders in genetic diagnoses
Williams syndrome: hypERcalcemia
DiGeorge syndrome: hypOcalcemia
348
Medications during acute myocarditis
NSAIDs - don't use (worsening inflammation, necrosis)
Ok: ACEi, ARB, spironolactone, CCB
349
Dietary impact of healthy food on warfarin
brussel sprouts, collard greens, spinach, kale rich in vitamin K, will decrease INR
Low vitamin K (no impact): banana, avocado, corn, fruit, green and red peppers
Amiodarone, levothyroxine, propranolol, and sertraline can increase warfarin effect; St. John's wort can decrease warfarin effect
350
Recreational drug use and cardiac effects
Cocaine, amphetamines, and marijuana cause MI (synthetic cannabinoids like K2 or spice may be even more potent)
Cocaine and MDMA cause coronary thrombosis
351
Incidence of endocarditis in Melody valve patients?
2.4% per patient year
352
Indication to stop an exercise test
SBP>250 mmHg, DBP>125 mmHg
Fall in SBP >10 mmHg
SpO2 <90% or fall more than 10 points from baseline
>2 mm flat or downsloping ST depression
SVT
Increasing ventricular ectopy
New BBB
New AV block
Dyspnea, angina
353
VACTERL association
Vertebral anomalies
Anal atresia
Cardiac defects (40-80% of patients, VSD most common)
TracheoEsophageal fistula
Renal anomalies
Limb abnormalities
Single umbilical artery often seen
354
CHARGE syndrome
CHD7 gene in 65-74%
Coloboma
Heart defects (TOF, DORV, arch anomalies)
Atresia of the choanae
Retartdation of growth
Genital abnormalities
Ear anomalies and hearing loss
355
Risk factors for rehospitalization, re-operation, and death in TOF/absent pulm valve
Prematurity/low weight at operation
Neonatal repair
Longer bypass
Pre-op respiratory distress (more likely malacia)
356
Sinus bradycardia pacemaker indications
Resting HR <40 bpm or pauses >3sec IIa in CHD or IIb in biventricular repair of CHD
357
Factors that increase endothelial-derived nitric oxide production
Bradykinin
Serotonin
Vasopressin
Histamine
NG-monomethyl-L-arginine (L-NMMA) decreases
358
Two most common arrhythmias after repair of AVSD
JET
Complete AV block
359
Common late complication after Norwood presenting with low cardiac output
Distal arch obstruction
360
Post-op complications from TAPVR repair
High LA filling pressures
Decreased pulmonary compliance
High PVR, RV dysfunction
Pulmonary edema
JET
361
Effect of positive pressure ventilation on coronary flow
Decreases RV/aortic pressure gradient, decreasing RV coronary flow
362
Formula for chylous effusion
Low in long chain triglycerides
High in medium chain triglycerides
363
Risk factors for seizure after cardiopulmonary bypass
Long periods of deep hypothermic circulatory arrest
Younger age
Preexisting CNS pathology
364
What size child has the best correlation between NIRS and true jugular venous saturation?
<10kg
365
Concerning chest tube output
>10 mL/kg/hr in first hour, >5 mL/kg/hr after two hours
366
Clinical signs of NEC
Thrombocytopenia, temperature instability, bradycardia, lethargy, apnea
Normal lactate doesn't rule out NEC
Watery stool more indicative of malabsorption
367
Aortic valve prolapse occurs in which types of VSD?
Perimembranous and outlet
368
Natural history of VSDs include spontaneous closure in how many?
75-80%
369
Malalignment VSD has malalignment of what structures?
Outlet septum and muscular septum (outlet septum is posteriorly - interrupted arch - or anteriorly - TOF - deviated)
370
Most common heart defect in T21
VSD
PDA
371
Heart disease in Ellis-van Creveld
Common atrium / AVSD
372
Rastelli Classification
Anterior bridging leaflet is ...
A - divided and attached to crest of septum
B - partially divided, papillary muscle on RV side of septum
C - not divided, papillary muscle on RV free wall
373
Types of AP window
Type I - proximal
Type II - distal
Type III - complete
374
Diastolic flow reversal in abdominal aorta and left heart dilation
Look for AP window
375
Berry Syndrome
Interrupted aortic arch Type A and AP window
376
Diastolic flow reversal in arch, dilated SVC, high output heart failure
Extracardiac AVM (L-R shunt)
377
Most common cause of extensive pulmonary AVMs
Hereditary hemorrhagic telangiectasia (if patient has multiple AVMs, 80% chance they have HHT)
Other issues: hepatopulmonary syndrome (hepatic failure)
With pulmonary AVMs, total cardiac output is not increased and PVR measures normal (decreased in AVMs, increased elsewhere)
378
Indications for fetal echo referrals ranked by frequency
- Suspicion of CHD on OB US (CHD confirmed in 40-50%)
- Fetal rhythm abnormality <10% of referrals
379
Retinoic acid associated with what cardiac defects?
Conotruncal defects, aortic arch anomalies
380
Normal fetal cardiothoracic ratio
25-35%
381
At term, what percent of fetal cardiac output perfuses the lungs?
10%
382
Retrograde flow in aortic arch is seen in what types of fetal echo?
Left sided obstructive lesions (e.g. critical aortic stenosis)
383
Teratogens and associated fetal diagnoses
Lithium: Ebstein, ASD, AVV atresia
Alcohol: maybe no increased risk of CHD, dTGA (hockey stick palmar crease, smooth philtrum, railroad track ears), VSD, ASD, coarctation, conotruncal
Indomethacin: ductal constriction
Phenytoin: coarctation/LVOTO
Retinoids: conotruncal, dTGA, 22q11
384
Most common cause of fetal complete heart block
Maternal SSA/SSB antibodies
Other causes: long QT, ccTGA, heterotaxy
385
Incidence of CHD in maternal diabetes and family history of CHD
Maternal diabetes: 4-10% (dTGA, truncus, TOF)
Family history: 2-4%
386
When is maternal hyperoxia test performed?
HLHS restrictive septum, look at pulsatility index in PAs - no change indicates pulmonary vasculature that is not reactive, higher risk
No change in PI: 100% sensitivity, 94% specificity for predicting need for immediate intervention on interatrial septum
387
Effect of bilirubin on NIRS
Bilirubin absorbs light at wavelength similar to that emitted by NIRS - falsely low reading
388
Effect of air bubbles in blood gas
pO2 falsely high, pCO2 falsely low
389
First line therapy for heart disease in Duchenne
ACEi
May be beneficial to start even before decline in function (fibrosis on MRI) - start when HF symptoms OR imaging shows abnormalities
390
Williams syndrome
7q11 - elastin (ELN) gene
Elfin facies supravalvar aortic and/or pulmonary stenosis, developmental and growth delay, ebullient personality, strength in language skills, deficits in visuospatial tasks
Hypercalcemia, digestive issues, urinary problems, abnormalities of connective tissue
Aortic stenosis tends to progress while pulmonary stenosis improves with time
391
Risk stratification for WPW
Exercise stress test - sudden disappearance of preexcitation at high heart rate - poor anterograde conduction, lower risk
If can't demonstrate that, should undergo EP study: shortest preexcited RR interval between two preexcited beats (SPERRI), <250 ms dangerous
392
ACHD indications for ASD closure
Right heart enlargement, no PH
- grade 2A to close if asymptomatic
- grade 1 to close if symptomatic
393
ACHD indications for pulmonary valve replacement in TOF
Asymptomatic with at least moderate PI, replacement reasonable with any two of:
- Mild or moderate RV or LV systolic dysfunction
- RVEDV >160 mL/m2
- RVESV >80 mL/m2
- RVEDV/LVEDV >2
- RVSP >2/3 systemic
- Progressive reduction in exercise tolerance
394
What is histological finding in pulmonary vein stenosis?
Fibromyxoid intimal proliferation in the VEINS
Muscularization in the pulmonary ARTERIOLES +/- arterial intimal fibrosis
395
Echo features of double chamber RV
Muscle bundles either in RV body or just below infundibulum
Normal size infundibulum (vs. hypoplastic in TOF)
Can have discrete PS or aortic stenosis
80-90% associated with VSD though can develop after VSD closure
Repair if peak gradient >40 mmHg or symptomatic
396
Criteria for LVNC
end systolic endocardial to myocardial ratio of 2:1 (echo)
end diastolic ratio of 2.3 to 1 (MRI)
LVNC is a different disease in kids than adults, with much higher morbidity/mortality in kids (60% of patients who have undergone transplant for LVNC were kids)
397
Most common complication after Glenn?
Phrenic nerve paralysis (4.7%)
398
How will fetal PVC appear on inflow/outflow Doppler?
lower peak velocity and smaller VTI because ventricle hasn't had time to fill
beat afterward may be larger (more time to fill)
may not re-set sinus node so atrial activity continues in a regular manner
399
Management of fetal PVCs
1. Weekly fetal heart rate measurement by OB
2. Monthly fetal echo to look for secondary causes
3. Postnatal cardiac assessment if doesn't resolve
400
Technical performance score
Echo evaluation of residual cardiac lesions
Class 1 - optimal
Class 2 - minor residual lesion
Class 3- major residual lesion
Class 3 has been associated with worse neurodevelopmental outcomes, increased resource utilization, higher mortality
401
Bundle branch block and SVT
If cycle length shortens with resolution of bundle branch block, accessory pathway USES that bundle branch - ipsilateral side
402
NOTCH1 and SMAD6 associated with what lesions?
Bicuspid aortic valve, aortic stenosis, and coarctation of the aorta
403
Jag1 and GATA6 associated with what lesion?
Tetralogy of Fallot
404
What genes are associated with TAPVR?
Ankyrin repeat domain
Platelet-derived growth factor receptor alpha
405
PA/IVS repair
Group A: tripartite RV, TV Z score > 2.5, no major sinusoids -- valve perforation (great survival)
Group B: TV z score -2.5 to -4.5, bipartite, patent RVOT ... can do valve perforation if no RV dependent coronaries
Group C: TV z score <-5 and unipartite usually RV dependent coronary (outcomes worse than HLHS - 5yr 22%)
406
DILV where is aorta and conduction system?
Aorta usually anterior and leftward
L-looped RV conduction is ANTERIOR to the pulmonary outflow tract
D-looped RV conduction is LATERAL to pulmonary outflow tract
407
Tricuspid atresia with transposed great arteries risk factor for late obstruction
bulboventricular foramen < 2cm2/m2
408
SVR Trial
1 year - transplant free survival better with Sano (74% vs. 64%)
3 years - No survival advantage, Sano had worse RV function and had more catheter interventions
6 years - No survival advantage
409
Enalapril in infants with single ventricle
Negative trial for enalapril in single ventricle patients
410
Normal Fontan pressure and transpulmonary gradient?
<15 mmHg
Transpulmonary gradient <10 mmHg (normal 5-8 mmHg)
411
Five year survival for Fontan with PLE?
88%
412
Treatments for PLE?
Budesonide (inflammation of GI tract)
Low fat/high protein diet
MCT
Octreotide
Zinc
413
Differential diagnosis for end tidal CO2 to PaCO2 gradient (>5 mmHg)
Dead space ventilation
- Remains in airways doesn't reach alveoli
- Blood doesn't reach the alveoli (low cardiac output, poor pulmonary blood flow); the latter also has shunt
*Hyperventilation
*Mechanical problem - ETT, pneumothorax
*PH or arrest
*Decreased pulm blood flow (e.g. Norwood shunt thrombosis)
414
Management of PH crisis
1. Relax pulmonary arterioles
- Correct acidosis
- Pulmonary vasodilators - iNO, O2
- Sedation/NMB
2. Increase SVR to shift septum rightward and maintain coronary perfusion (e.g. vasopressin)
415
Epidemiology of bicuspid aortic valve
1-2% of population (most common CHD)
R-L fusion most common (70%), then R-non
- Half of patients have BAV as isolated finding, with or without valve dysfunction (usually mild)
- Ascending aorta dilation occurs in about half
- Half of patients with coarctation have a BAV
416
Most important variable for accepting or rejecting donor heart in predicting post-transplant outcome?
Ejection fraction
417
Treatment for post-pericardiotomy syndrome
Class I: NSAIDs
Class IIa: colchicine
Diuretics can also help
Aspirin not recommended in kids
Steroids have fallen out of favor - side effects, higher recurrence (but do have a role when underlying autoimmune or connective tissue disease)
418
Progression of V1 over time
Newborn: upright T wave, R wave predominance
Infant: inverted T wave, R wave predominance
Child: inverted T wave, equal R/S waves
Adult: upright T wave, S wave predominance
419
Mutations in hamartin and tuberin cause what?
Tuberous sclerosis (hamartin = TSC1, tuberin = TSC2)
Can use sirolimus/everolimus (mTOR inhibitors) for treatment
Require long term monitoring even without tumors (arrhythmia)
420
HRAS mutation
Costello syndrome
Arrhythmias and hypertrophic cardiomyopathy
421
Abnormal coagulation studies in a Fontan patient not on Warfarin are most likely due to what?
Fontan associated liver disease
422
Criteria for Kawasaki Disease
CRASH and Burn
- Fever >= 5d PLUS 4/5:
1. Bilateral limbic-sparing CONJUNCTIVITIS
2. Oropharyngeal changes (STRAWBERRY)
3. Maculopapular RASH
4. HAND and feet changes
5. LympADENOPATHY
Coronary Z-score >2.5, aneurysm, or 3+ other suggestive features (decreased LVEF, MR, effusion, z-score >=2)
423
Familial supravalvar aortic stenosis
- No other dysmorphic features or syndromic associations
- Typically supravalvar AS, may also be at risk of vascular stenoses elsewhere (aorta, pulmonary, coronary, renal arteries)
- Elastin gene mutation (vs. Williams which is microdeletion of 7q11.23 involves elastin gene)
424
Anticoagulation for mechanical valves in pregnancy
- Dose <=5mg continue Warfarin until delivery, switch to IV UFH (PTT 2x control) 1 week before planned delivery
- Can add aspirin to Warfarin in 2nd/3rd trimester
- Dose >5mg: heparin (LMWH goal 0.8-1.2) through 1st trimester and at delivery, Warfarin in between
- Delivery should always be UFH, stop 6h before planned vaginal delivery
- If labor begins or urgent delivery required on warfarin, reverse and perform c-section
425
What is Ashman phenomenon?
Refractory period of the bundles depends on the preceding R-R interval, so at the start of tachycardia, longer refractory period and aberrancy
Then as tachycardia continues, refractoriness adjusts and QRS narrows
426
Anticoagulation recommendations for mechanical valve
1. Mechanical heart valve, minor procedure where bleeding easily controlled: continue warfarin
2. Mechanical aortic valve and no other thromboembolic risk factors with invasive procedure: ok to pause warfarin briefly with no bridge
3. Mechanical aortic valve and no other risk factors: warfarin to INR 2.5
4. Mechanical aortic valve and other risk factors OR mechanical mitral valve: warfarin to INR 3.0
Can add aspirin if there is an indication for antiplatelet therapy and no concerns for bleeding
427
Most likely diagnosis for irregularly irregular wide complex tachycardia in otherwise healthy patient?
Pre-excited atrial fibrillation
*Don't want to block AV node - adenosine, digoxin, diltiazem, beta blocker
Rx: cardioversion or amiodarone
428
Palivizumab (Synagis) prophylaxis criteria
<= 12 months with hemodynamically significant congenital heart disease
- Acyanotic heart disease being treated for CHF and who will require cardiac surgery
- Moderate to severe pulmonary hypertension
- No specific recommendations for palliated or unrepaired cyanotic heart disease
Primary benefit = decrease in RSV-associated hospitalization
429
Treatment of pericarditis
Ibuprofen/aspirin - most commonly used, don't alter the natural history of disease
Steroids - higher risk of relapse
Colchicine should be considered x4-6wks especially if haven't responded after 1wk NSAIDs
430
Treatment of refractory KD
Persistence or recurrence of fever 36h after one dose of IVIG:
- Second dose 2 g/kg
- Methylprednisolone 20-30 mg/kg IV x3d
431
Treatment of acute rheumatic fever
Mild to moderate carditis: aspirin 80-100 mg/kg/d in four divided doses
Severe carditis: Add prednisone
432
Immunosuppressive agents
Sirolimus - blocks transcription of lymphocyte activation genes, may be less nephrotoxic over the long term; associated with bone marrow suppression
Tacrolimus and cyclosporine - calcineurin inhibitors, available for IV use, no survival advantage for either over the other
Tacrolimus more often causes diabetes mellitus than cyclosporine (higher tacro levels, HLA-DR mismatch, and older age at transplant predisposes)
433
Duration of antibiotic prophylaxis after acute rheumatic fever?
Residual heart disease: At least 10 years or until age 40 years whichever is longer
No residual heart disease: At least 10 years or until 21 years of age, whichever is longer
Never had cardiac involvement: 5 years or until age 21, whichever is longer
434
Lidocaine toxicity
CNS - tremors, lightheadedness, ataxia, dysarthria, mood/personality changes, hallucinations, seizures
435
Most common side effect from rabbit ATG?
Fever
Shaking/chills
436
Phenytoin as an antiarrhythmic is like what other medications?
Lidocaine and mexiletine
Class IB sodium channel blockers
QTc can be slightly shortened
437
Medications for atrial flutter
Diltiazem - slow RVR, good choice
IV beta blocker or sotalol other options
Flecainide - can slow atrial rate (fast flutter 2:1 becomes slow flutter 1:1)
Disopyramide - has anticholinergic activity so may enhance AV node conduction, so could worsen situation alone
Best to administer flecainide/disopyramide WITH an AV node blocking agent
438
Sotalol clearance
Renal - should be dose adjusted in severe renal dysfunction
439
Atropine vs. isoproterenol
Atropine - anticholinergic/vagolytic so only works if AV block or sinus slowing is due to excessive vagal tone
Isoproterenol selective beta 1 - chronotropy and inotropy
440
Losartan has what impact on TGF-B
Angiotensin type 1 receptors increases expression of TGF-B ligands ... losartan blocks this pathway
441
Amiodarone drug interactions
Inhibits cytochrome P450, increases levels of:
cyclosporine
digoxin
warfarin
442
Amiodarone mechanism of action
Mostly potassium channel blockade
Also:
- Sodium channel and Ca channel blockade
- Beta blockade
- Decreases AV node conduction
Hypokalemia exacerbates pro-arrhythmic potential
443
Mechanism of heparin-induced thrombocytopenia with thrombosis (HITT)
Heparin + platelet factor 4 -- makes immunogenic complex
Antibodies result in formation of platelet aggregates which cause vaso-occlusion and immune-mediated platelet destruction
444
Differences between beta blockers
Nonselective: Nadolol, Propranolol, Sotalol
Selective: Atenolol, Metoprolol (less risk of bronchospasm)
Metoprolol more lipid soluble than atenolol so can cross blood-brain barrier
445
Location of action for antiarrhythmics
Procainamide - atrial myocardium, His-Purkinje, ventricular myocardium (no effect on SA or AV node)
Diltiazem - slow AV conduction
Lidocaine/mexilitine - ventricular muscle
446
Best antiarrhythmic for IART in ACHD patient with ventricular dysfunction who failed ablation attempt
Amiodarone or dofetilide
447
Dofetilide mechanism of action
Class III antiarrhythmic, inhibits rapid component of delayed rectifier potassium current - can prolong QTc
May have to dose adjust for renal disfunction
448
Clinical trial phases
0 - subtherapeutic 10-15 for pharmacokinetics
I - dosing and side effects in <100
II - up to 300, doesn't yet have a presumed therapeutic effect
III - large clinical trial, RCT 300-3,000
IV - long term follow up after FDA approval
449
Efficacy/potency of a drug
most efficacious is the one with the LARGEST desired response regardless of dose required
potency is based on drug concentration that produces 50% of maximum effect
450
How many half lives to steady state?
Four to five
451
Irritability and tremulousness are side effects of what immunosuppressive medication?
Tacrolimus
452
Side effects of immunosuppressants
Tacrolimus - tremulousness, seizure
Azathioprine, MMF - leukopenia, GI upset
Sirolimus - diarrhea, mouth sores
Prednisone - mood changes, appetite, hyperglycemia, weight gain, Cushingoid appearance, osteoporosis
453
Potts shunt vs. atrial septectomy
Potts:
- Doesn't decrease CVP
- Doesn't increase LV preload
- Increases LV afterload
- Advantage: lower risk of stroke, central saturation maintained
ASD:
- Does decrease CVP
- Increases LV preload
454
Interaction of calcineurin inhibitors and antifungal medications
Antifungal will increase calcineurin inhibitor levels (e.g. fluconazole creates tacrolimus toxicity)
Other meds that can increase calcineurin inhibitor level:
- Amiodarone
- Macrolides
- CCB
- Metoclopramide
Decrease calcineurin inhibitor level:
- Octreotide
- Some anticonvulsants (phenytoin, phenobarb, cabamazepine)
- Some antibiotics (nafcillin, IV Bactrim)
455
Risk factors for sudden cardiac death in dilated cardiomyopathy
LVEDD z-score >2.6
Age at diagnosis <14y
LV posterior wall thickness to LVEDD <0.14 (big/thin)
Presence of congestive heart failure at diagnosis
456
Stages of rejection
Grade 1R - interstitial and/or perivascular infiltrates, up to 1 focus of myocyte damage
Grade 2R - two or more foci of infilatrate with associated myocyte damage
Grade 3R - diffuse infiltrate with multifocal myocyte damage, wtih or without edema, hemorrhage, or vasculitis
457
Inheritance pattern of most familial dilated cardiomyopathy?
Autosomal dominant
458
Approach for EBV mismatch (donor +, recipient -) in transplant to decrease risk of PTLD?
Safely minimize immunosuppression
459
Treatment for transplant associated coronary vasculopathy
Aspirin
Statin
Switch calcineurin inhibitor to mTOR inhibitor (sirolimus)
Re-transplant
460
Mechanism of action of amiloride
Like spironolactone: blocks Na resorption in distal convoluting tubule, decreasing excretion of K and H
461
Side effects of PH therapies
Sildenafil: vision changes
Treprostinil: urinary retention, thrombocytopenia
Bosentan: hepatic dysfunction, lower extremity edema, anemia (other ERAs too)
462
Cat Eye Syndrome
Tetrasomy 22p
Rectoanal anomalies, coloboma, genitourinary anomalies, preauricular pits/tags
463
Risk of CHD based on indication for fetal echo
Suspicion on OB screening (40-50%)
Maternal CHD 3-7%
Maternal pregestational diabetes 3-5%
Sibling 3%
Paternal CHD 2-3%
IVF 1-3%
Fetal echo not warranted:
Second-degree relative 1-2%
Third degree relative - normal risk
Gestational diabetes - normal risk
464
Treatment for pseudoaneurysm post-cath
Risk factors: obesity, female sex, thrombocytopenia, underlying coagulopathy, systemic hypertension, arterial calcification, age >75y, large sheath, use of antiplatelet/anticoagulant drugs, emergency procedures, puncture below common femoral artery
Rx:
- If small (<2cm) without symptoms can observe
- US guided compression
- US guided thrombin injection - best procedural success rate
- Surgical repair in patients with life-threatening symptoms
465
Factors to precipitate early lipid screening (age 2-8 years) or 12-16 years
Parent, grandparent, aunt/uncle, or sibling with:
- MI, angina, stroke, angioplasty at <55y M or <65y F
- Parent with TC>=240 or known dyslipidemia
- Child with diabetes, hypertension, BMI>95th%, smokes cigarettes, moderate or high risk medical condition
466
What is universal lipid screening age 9-11?
Non-fasting lipid panel, total - HDL = non-HDL
If non-HDL >=145 and/or HDL <40, get FLP twice
OR
Fasting LDL >=130 +/- non-HDL >=145 +/- HDL <40 +/- triglycerides >=100 (130 if over 10)
Then repeat and average results
*Screen again age 17-21
467
Acetazolamide
Blocks activity of carbonic anhydrase
Acetazolamide effect is reduce alkalosis and increase NaCl, (HCO3- out, keep Cl-), increases effectiveness of the other diuretics
468
Metolazone
Thiazide diuretic, blocks NaCl reabsorption in distal convoluted tubule
469
PDA closure criteria
Routine follow-up Q3-5y for small PDA without evidence of left-sided overload
Percutaneous closure for:
- Left sided enlargement
- PH with net L-R shunt
- Prior case of endarteritis
470
LVOTO in AVSD more common in what scenario
Separate AVV orifices (partial > complete)
Can be caused by attachments of superior bridging leaflet to septum, extension of AL papillary muscle into LVOT
10% of patients will require re-operation for LVOTO
Most common indication for re-operation is LAVV regurgitation or stenosis
471
S2 with VSD
Usually normal
Some patients may have wide splitting of S2
472
Process of closure of the ductus arteriosus
Stage 1 (12 hours): contraction and cellular migration of medial smooth muscle in the wall
Stage 2 (2-3 weeks): infolding of endothelium, fragmentation of internal elastic lamina, proliferation of subintimal layers, hemorrhage and necrosis in subintimal region
473
Indomethicin vs. ibuprofen for PDA
Similar: rate of successful closure, risk of IVH
Ibuprofen better: less effect on renal function, cerebral blood flow/vasculature
Ibuprofen worse: higher risk of pulmonary HTN
474
Symptoms of sinus of Valsalva
75% male
65% right aortic sinus, 25% noncoronary sinus
50% associated with VSDs
Most common rupture is right sinus aneurysm to RV in setting of outlet VSD
Sinus of Valsalva fistula to right side: left to right shunt, wide pulse pressure, collapsing pulse, LV hyperactivity, may also have RV hyperactivity
Sinus of Valsalva fistula to left side: to-and-fro murmur like AI
475
Outcomes of embolization for pulmonary AVMs
Improves hypoxemia, orthodeoxia, TIAs/stroke
Does not reduce risk of brain abscess
To minimize risk of embolization use coil or umbrella not glue or beads
Goal is to raise systemic arterial oxygen tension >60 mmHg
476
Distinguish between large PDA and AVM in neonate
Systemic venous saturation
477
Kawashima
Glenn but with interrupted IVC with azygous continuation so ends up being close to Fontan
EXCEPT hepatic drainage still bypasses lungs so get AVMs
478
Unilateral pulmonary artery stenosis
Severity of stenosis likely underestimated because other side accommodates the extra flow (diastolic pressure difference is proportional)
Balloon diameter 3-4x the narrowest segment
Acute success rate is 50-60%
Recurrent stenosis rate 15-20%
479
What percent of patients with PA/IVS have RV-dependent coronary circulation?
<10%
(45% have ventriculo-coronary connections but aren't RV dependent)
480
Goal of TOF/PA/MAPCAS repair
Incorporate at least 14 pulmonary arterial segments into connection with RV
Central PA size at least 50% normal
RV pressure <70% LV - if higher, re-open VSD
481
Location of bundle of His in TOF/PA/MAPCAS
Normal sinus node, normal AV node
Bundle of His lies along the left ventricular aspect of the posteroinferior rim of the VSD
482
Waterston vs. Potts shunt
Waterston - ascending aorta to RPA
Potts - descending aorta to LPA
483
Continuous murmur more common in TOF/PA/MAPCAS or truncus?
TOF/PA/MAPCAS
484
Difference between isolated mitral valve cleft and cleft seen in AVSD
Isolated: directed anteriorly toward outflow septum/LVOT
AVSD: posteriorly directed toward inlet septum
485
Criteria for single- or bi-V repair with LV hypoplasia
Single V:
-Non-apex forming LV
-AoV annulus <5mm
-MV annulus <9mm
-Most predictive: aortic root dimension indexed to BSA, ratio of long axis of LV to long axis of heart, indexed MV area
*Rhode's criteria?
486
Sports participation for aortic stenosis
Mild: Doppler mean <25 mmHg, CW Doppler <40 mmHg, cath peak to peak <30 mmHg
Moderate: Doppler mean 25-40 mmHg, cath peak to peak 30-50 mmHg, CW Doppler 30-50 mmHg
Severe: Doppler mean >40 mmHg, cath peak to peak >50 mmHg, CW Doppler >70 mmHg
Mild and asymptomatic - can participate in all
Moderate, mild or less LVH, no repol abnormality, normal exercise test can do low static and low to moderate dynamic sports (moderate static and low dynamic if no arrhythmia)
Severe - no sports
487
When to operate on coarctation in asymptomatic child without severe upper extremity hypertension?
Age 2-3 years - less risk of late recurrence compared with surgery before age 1
488
What causes exercise induced upper extremity hypertension following coarctation repair?
Increased flow across relatively non-distensible coarctation repair site, can use beta blocker
489
ccTGA ECG
Q waves present in right precordial leads, III, aVF, absent in left
Left axis deviation
490
Best surgery for DORV with subpulmonary VSD
Arterial switch (transposition physiology)
With pulmonary stenosis: REV, Rastelli, or Nikaidoh
491
Pericardial defects
Best way to assess is MRI
80% of defects occur on left
Most are asymptomatic
Symptoms can include syncope, chest pain, arrhythmia, death
Bad outcomes from: herniation of LA appendage, torsion of GAs, constriction of coronary at rim of defect
CXR may have leftward displacement of cardiac border, "enlarged MPA" is actually herniated LA appendage
492
Follow up schedule for family history HCM
Without ECG findings or significant hypertrophy, Q12-18mo until 21 years
Then Q5y
493
Loffler endocarditis (hypereosinophilic syndrome)
Temperate climates, adult males
Hypereosinophilia usually involving other organs
Fever, weight loss, rash, cough, heart failure
Treat hypereosinophilia: steroids, hydroxyurea, or vincristine
Cardiac treatment: diuretics, afterload reduction, anticoag, digoxin
494
ECG in Duchenne
Deep Q waves in I, aVL, V5, V6
PR may be short
Resting sinus tach, loss of circadian rhythm, reduced HR variability
495
Most common arrhythmia in pediatric restrictive cardiomyopathy?
Atrial flutter
Next most common: high-grade second degree AV block, third degree block
496
Indications for closure of asymptomatic perimembranous VSD
- Progressive aortic regurgitation
- RV muscle bundle hypertrophy (DCRV)
- Bacterial endocarditis
497
Hemodynamic measures predicting success after Glenn
LVEDP <12 mmHg
TPG <10 mmHg
mPAP <16 mmHg
498
Late formation of aneurysm is associated with which technique for aortic coarctation repair?
Prosthetic patch
499
What is a hemi-Fontan?
Glenn without disconnecting SVC from RA
Advantage: later expeditious Fontan, but unlike Glenn it does require bypass
500
Goal for training LV in planned anatomic repair of ccTGA
70-80% systemic pressure, repair within first 2-3 years of life
501
"Circle Sign" on echo (small circle anterior to aortic valve in parasternal long)
High, anterior, leftward origin of RCA
Anomalous origin of RCA from left or LCA from right
502
Whale's tail sign suggests what
TAPVR to coronary sinus
503
Risk of congenital heart disease in situs inversus totalis?
<1%, same as general population
504
What is Carney complex?
Atrial myxomas
Spotty pigmentation
Endocrine over-reactivity
Autosomal dominant (PRKAR1A), 70% familial
505
Gorlin syndrome is associated with what cardiac tumor?
Fibroma
506
Classic x-rays:
Figure of 3
Egg on a string
Boot shaped heart
Basketball
Snowman
Figure of 3 - coarctation
Egg on a string - dTGA
Boot shaped heart - TOF
Basketball - Ebstein
Snowman - supracardiac TAPVR
507
Chiari network is remnant of what?
Incomplete embryonic absorption of thebesian valve
508
Frequency of echo in KD
Repeat twice/week until coronary dimensions have stopped progressing
509
Early vs. late Lyme disease
Early:
Erythema migrans
Facial palsy
1st degree AV block
Conjunctivitis
Late:
Large joint arthritis
510
What is vasculitis associated with HIV?
Vasculitis of aorta and pulmonary arteries that can lead to diffuse stenosis
511
MISC first line treatment
IVIG, methylprednisolone
512
Describe neonatal enterovirus
Common cause of acquired neonatal myocarditis and encephalitis
Family history of diarrheal type illness
Includes coxsackievirus
*IVIG commonly used though limited supportive data
513
Most common cause of acute purulent pericarditis
Staph aureus
Also: Strep pneumo
Tuberculosis in developing world
Empiric treatment: vanc and ceftriaxone, should consider cefepime for psuedomonas in postsurgical
514
Causes of HIV-related dilated cardiomyopathy
Zidovudine (AZT) associated with early DCM in teens and young adults
515
Congenital Rubella
Cataracts
Microcephaly
Blueberry muffin rash (petechiae or purpura)
Hepatosplenomegaly
Pulmonary stenosis / PDA
516
Tuberculous pericarditis
Adenine deaminase level very high (>40 U/l)
Also send: lymphocyte to neutrophyil ratio and pericardial IFN-gamma
Effusions associated with neoplasm may also have elevated ADA
AFB stain and mycobacterial culture should be sent but are unlikely to be positive from pericardial fluid
517
Chagas disease
Caused by Trypanosoma cruzi
Acute phase 2-3 months - mild flu like symptoms +/- meningoencephalitis and/or acute myocarditis
Chronic phase is lifelong, low-level parasitemia, mostly asymptomatic
25% develop progressive symptoms which can involve cardiomyopathy and ventricular arrhythmias
Consider reactivation in immunosuppression
Screening: IgG T. cruzi
518
Jervell and Lange-Nielsen Syndrome
Biallelic mutations in KCNQ1 (autosomal recessive)
Hearing loss and long QT
519
Features of cri-du-chat syndrome
Microcephaly, round face, hypertelorism, micrognathia, epicanthal folds, low set ears, hypotonia, severe psychomotor and mental retardation
high-pitched cat like cry
Cardiac: VSD, ASD, PDA, TOF
520
ASD and conduction abnormality WITHOUT limb anomalies has mutation. inwhat?
NKX2.5
521
Most common genetic cause of familial dilated cardiomyopathy
LMNA mutation
Cardiac conduction disease, dilated cardiomyopathy
522
Features of Ellis-van Creveld syndrome
Amish community
Skeletal: short stature, short limbs,
Ectodermal: neonatal or small teeth, hypoplastic or dysplastic fingernails
Cardiac: large ASD or common atrium
EVC and EVC2 genes on chromosome 4
523
Jacobsen syndrome
Wide-spaced eyes, ptosis, small ears, short stature, thrombocytopenia
VSD, left-sided lesions involving mitral and aortic valve
11q23 deletion
524
Percent that have CHD:
Trisomy 18
22q11
Trisomy 21
Turner
5p-
Trisomy 18: 95%
22q11: 80%
Trisomy 21: 40%
Turner: 25%
5p-: 20%
525
What percent of patients with interrupted aortic arch type B have 22q11 deletion?
Half
526
Genetic testing recommendations in coarctation
Every female should have a karyotype
527
Morphology of HCM with highest yield on genetic testing
Reverse curve
528
Marfan-like features with intellectual disability
Homocysteinuria (autosomal recessive), CBS gene
Recurrent thromboembolism, lens dislocation is downward
529
Types of tests for each type of data
Compare means (parametric):
2 groups = T-test
paired = Paired t-test
3+ groups = ANOVA
Compare medians (non-parametric):
2 groups = Mann-Whitney, Wilcoxon Rank Sum
paired = Wilcoxon Signed
3+ = Kruskal Wallis
Compare proportions:
Chi square/Fisher exact
paired = Mcnamara
530
What test is used to analyze difference between Kaplan-Meier curves?
Log-rank test
531
What happens to PPV and NPV as prevalence increases?
PPV increases
NPV decreases
532
What is a type III error?
Produces the right answer to the wrong question
533
What is the p-value?
Probability that an observed difference occurred only by chance
534
What factors increase the power of a study?
Power = 1- beta, probability of rejecting the null when it is false, of not committing a type II error
Bigger sample size
Bigger effect size
Less variability
Willingness to accept bigger alpha
535
Cost-effectiveness, utility, etc
Cost-effectiveness: per unit of health outcome
Cost-utility: quality of life expressed as utilities (QALY, disability-adjusted life year)
Cost-benefit: weigh everything in monetary terms (lost earnings)
536
Advantage/disadvantage of meta analysis
Advantage: refinement and reduction, efficiency, generalizability and consistency, reliability, power, precision
Disadvantage: publication bias, clinical heterogeneity, quality differences, lack of independence of study subjects
537
What is the standard error of the mean?
SEM = SD / sqrt(n)
How close the sample mean is likely to be to the population mean
538
accurate, precise, reliable, valid
Accurate and valid (measures like the gold standard)
- Accurate - closeness to quantity's true or accepted value
- Valid - extent to which it measures what it's intended to (systematic error or bias)
Precise and reliable (same value on repeated measures)
- Precise - closeness of two or more measurements (SEM)
- Reliable - consistency of set of measurements/tool, repeatability, inversely related to random error
Accurate/precise are about the tool, valid/reliable are about the research design?
539
What is degrees of freedom (chi square)
Number of independent comparisons that can be made between members of the sample, used with X2 to calculate p-value
540
Correlation coefficients
-1 strong negative correlation, 0 no correlation, 1 strong positive correlation
Can't use for non-linear relationship or multiple outliers
Pearson correlation (normal) or Spearman (not normal)
541
Types of bias
Observer: observer inaccurately assesses variable
Confounding: spurious association from another variable
Selection: subjects not representative of true population
Information: measurements incorrectly recorded
Publication: only positive results are published
Recall bias: inaccurate remembering of facts
Allocation bias: systematic difference in how subjects are assigned
Lead-time/length bias: looks like test improves survival but really detected earlier in course
542
Three principles of ethics from Belmont report
1. Respect for persons (autonomy, informed consent)
2. Beneficence (do no harm, maximize benefits and minimize risks)
3. Justice (reasonable, nonexploitative procedures administered fairly and equally)
543
How do you calculate sample size of study?
Power (usually 0.8)
Significance level (usually 0.01 or 0.05)
Variability of observations (SD)
Smallest effect of interest (standardized difference)
544
Levels of evidence by US Preventive Services Task Force
A - good (benefits >> risk)
B - fair (benefits > risk)
C - fair (benefit = risk)
D - fair (risk > benefit)
I - lacking, poor quality, conflicting
545
Define "censored" in survival analysis
Each participant is censored or noncensored
Censored = dropped out for reasons other than outcome of interest (withdrawal, lost to follow up, alive at end of study)
Noncensored = had the event of interest
546
What test to evaluate agreement between two groups when there's no gold standard?
Kappa statistic
-1: negative association
1: positive association
0: no association
547
When is odds ratio a good approximation of relative risk?
When probability of event of interest is small
548
When do you use Cochran-Mantel-Haenszel test?
Association between dichotomous exposure and dichotomous outcome accounting for confounder variables (think chi-square adjusted)
549
Systemic to PA shunt with highest risk of developing PHTN?
Potts/Waterston (can't regulate flow)
Central and BTTS more resistance
550
Biggest risk factor for left AVV regurg after AVSD repair?
Severe preop AVV regurgitation
551
Nakata index
Used to determine sufficient PA size for PA/VSD
LPA area (mm2) + RPA area (mm2) + MAPCA area (mm2) / BSA (m2)
>200 good candidate for complete repair
552
Dyspnea/cyanosis on standing
Pulmonary AVMs (most occur at basal region of lung)
553
Conduction risk with subaortic membrane resection
LBBB
554
Left heart distention after bypass
Suggests ongoing pulmonary venous return to LA implying systemic to pulmonary shunt - AP collaterals
Could also be bad AI
555
Most common late complication in Scimitar syndrome?
Pulmonary venous obstruction
556
How would retrograde coarctation present in hybrid?
Poor coronary perfusion - dysfunction, ST segment changes, irritability
557
Surgical palliation of TOF absent valve
LeCompte and PA plication
558
When to consider surgical ASD closure over cath lab
Deficiency of posterior-inferior rim
Other abnormalities that would benefit from surgical repair (tricuspid regurg)
559
What is the only surgical intervention for Ebstein anomaly that shows improvement in functional capacity?
ASD/PFO closure
560
Most common indication for re-operation after Ross
Pulmonary homograft failure
561
High filling pressure with normal function and no effusion
Re-open the chest
562
ACHD criteria for pulmonary valve replacement in TOF
Moderate or more PR AND:
1. Symptoms (class I)
2. Any two of (class IIa):
- At least mild ventricular dysfunction
- RVEDI >160, RVESV >80, RVEDV>2xLVEDV
- RVSP > 2/3 systemic
- Progressive reduction in exercise tolerance
3. Sustained tachyarrhythmia (class IIb)
563
Risk factors for sudden death in adult TOF
-LV dysfunction
-Nonsustained VT
-QRS >180
-Extensive RV fibrosis by CMR
564
Indications for surgery in severe AI
LVESV >50 mm (>25 mm/m2)
LV dysfunction (EF<55%)
565
Indications for surgery in bicuspid valve with ascending aorta dilation
>55 mm without risk factors
>50 mm with risk factors (family history, rapid growth)
566
Sotalol leads to more QTc prolongation (higher risk) with what electrolyte abnormalities?
Hypokalemia
Hypomagnasemia
567
Earliest sign of cirrhosis in Fontan?
Thrombocytopenia
568
Marker of hepatocellular cardinoma in Fontan?
Alpha-fetoprotein level
569
LMWH vs. UFH
LMWH = enoxaparin = Lovenox (subQ)
UFH = heparin (IV infusion or subQ)
570
Why is iron deficiency a concern for patients with Eisenmenger?
Microcytosis in patients with secondary erythrocytosis increases risk of thromboembolic events (stroke)
571
Patients with Senning/Mustard should have what evaluation prior to transvenous pacemaker placement?
Cath to assess for baffle leak or stenosis that would complicate lead placement or put patient at risk for paradoxical embolus
572
Evidence for medical therapy to treat failing systemic RV? (e.g. Mustard/Senning dTGA)
No evidence
573
Hepatitis C
Risk factors include intranasal and IV illicit drugs
No universal screening before 1992
5% of patients with CHD who had surgery before 1992 may be infected
574
Serum BNP >140 correlated with poor long-term outcome in patients with what disorder
Eisenmenger syndrome
575
Familial ASD
Holt Oram
NKX 2.5
GATA IV
576
List the 10 high risk pregnancy features from CARPREG II
1. Prior cardiac events or arrhythmia
2. Baseline NYHA class III-IV or cyanosis
3. Mechanical valve
4. At least mild LV systolic dysfunction
5. Left sided valve disease or LVOT obstruction
6. Pulmonary hypertension
7. Coronary artery disease
8. High risk aortopathy
9. No prior cardiac intervention
10. Late pregnancy assessment
577
ACE in pregnancy
ACE and ARB contraindicated (early teratogen, late renal dysfunction)
Replace with hydralazine and nitrate
578
Treatment for Loeys Dietz
ARB first line (effect on TGFB signaling)
579
Aortic stenosis in pregnancy
Calcified valve - can't do balloon or percutaneous valve
Surgery feasible
580
What is platypnea-orthodeoxia syndrome?
Hypoxemia and dyspnea with standing from supine
R-L shunting at PFO exacerbated by change in septal position with upright position
581
Cardiac considerations with antidepressants
Sertraline - low risk
Citalopram/amitriptyline - long QT
Venlafaxine and bupropion - norepi increase
582
Dabigatran use
Oral direct thrombin inhibitor
Approved for atrial fibrillation, not for mechanical valves
583
Simvastatin has drug-drug interaction with what antihypertensive?
Amlodipine increases risk of myopathy
584
Atenolol and pregnancy
Class D - positive evidence of fetal risk (a study demonstrated lower birth weight infants)
585
Who needs pre-op coronary angiography?
Men >=35years
Women >=35years with coronary risk factors
Women after menopause
586
Adult indications for high-intensity statin
Age 40-75 with diabetes, risk factors for atherosclerosis (hyperlipidemia, prior smoking, positive family history, HTN, coarct)
WITH diabetes don't have to do other work up
587
ICD placement in patient with cyanosis with intracardiac shunt should be what type?
Epicardial (increased risk of thromboemboli if intravenous placed)
588
What 3 categories of ACHD patients should be referred to ACHD center for non-cardiac surgery?
1. Complex or cyanotic CHD
2. PAH
3. Malignant arrhythmia
