Cardiology Boards

Question 1

What fetal finding is thought to lead to TOF/absent pulmonary valve?

Answer 1

Premature PDA closure/absent PDA

Question 2

List the 4 causes of long RP tachycardia

Answer 2

1. Sinus tachycardia
2. Atrial tachycardia
3. Atypical AVNRT
4. PJRT

Question 3

Which valve is Libman-Sacks lesion associated with? What disease is Libman-Sacks lesion associated with?

Answer 3

Mitral valve, SLE

Question 4

Adenosine causes what action on the coronary vessels?

Answer 4

Vasodilation (low O2 > adenosine monophosphate (can’t generate ATP) > adenosine > vasodilation)

Question 5

NO > cGMP and prostaglandin both cause smooth muscle what?

Answer 5

Smooth muscle relaxation

Question 6

Which receptors release Ca from the sarcoplasmic reticulum?

Answer 6

Ryanodine receptor

Question 7

Which receptors allow Ca into the myocyte?

Answer 7

L-type voltage gated Ca channel

Question 8

What’s the most common form of bicuspid aortic valve?

Answer 8

Fusion of R-L > R-non > L-non

Question 9

List two equations for resistance.

Answer 9

R = deltaP/Q
R = (8xlengthxviscosity)/pi*R^4

Question 10

Which of the aortic arches is each structure derived from:
carotid
arch
PAs/PDA
subclavian

Answer 10

carotid (III)
arch (IV)
PAs/PDA (VI)
subclavian (VII)

Question 11

What percent of TOF patients have an ASD/PFO? Right aortic arch?

Answer 11

80% and 25%

Question 12

Most common coronary arrangements in TGA?

Answer 12

Normal (70%)
LCx from RCA (16%)
Single right, inverted RCA/LCx

Question 13

Where is the transverse sinus?

Answer 13

Between great vessels and atrial walls (looks like LCA)

Question 14

Where is the oblique sinus?

Answer 14

Between posterior LA and reflections of SVC and pulmonary veins

Question 15

What is the normal ratio of septal to LV free wall thickness?

Answer 15

1.1 (increases with age to 1.2)
Ratio of LV:RV thickness is 3

Question 16

Most likely structure ruptured in trauma?

Answer 16

RV. If atrium is ruptured it’s typically the appendage.

Question 17

Repetitive exercise results in what changes for resting HR, resting BP, blood volume, stroke volume, myocardial O2 demand?

Answer 17

HR - decreased
BP - decreased
blood volume - increased
SV - increased
myocardial O2 demand - decreased

Question 18

How much displacement of the tricuspid valve is consistent with Ebstein anomaly?

Answer 18

8 mm/m2

Question 19

List AVSD types.

Answer 19

Complete
Intermediate - large ASD/VSD, separate
Transitional - small VSD, large ASD, separate
Partial - no VSD, large ASD, separate

Question 20

Most common form of HLHS?

Answer 20

MA/AA (36-46%)
MS/AA (20-30%)
MS/AS (13-26%)

Question 21

Name parts of a sarcomere

Answer 21

A band - thick (myosin) filament
I band - thin (actin) filament
H zone - central clearing

Question 22

Most common morphologies of truncal valve?

Answer 22

Tricuspid (70%)
Quadricuspid (20%)
Bicuspid (10%)

Always in continuity with mitral valve

Question 23

80% of Ebstein patients also have what?

Answer 23

ASD

Question 24

Which two structures have the lowest SaO2 in the fetus?

Answer 24

SVC and coronary sinus

Question 25

What is the most common great artery configuration in DORV?

Answer 25

Side-by-side with aorta rightward (2/3)

Question 26

What is deficient in Pompe disease?

Answer 26

acid ɑ glucosidase (GAA)

Question 27

Premature ductal constriction in the fetus results in what postnatal finding?

Answer 27

PPHN (RV hypertrophic and dilation)
Ductal pulsatility index <1.9 suggestive

Question 28

What are the major and minor criteria of acute rheumatic fever?

Answer 28

Polyarthritis, carditis, subcutaneous nodules, erythema marginatum, syndham chorea

CRP>3, ESR>60, fever, polyarthralgia, long PR

Question 29

List the three indications for surgical repair of double chambered RV

Answer 29

1. Pressure gradient of at least 40 mmHg
2. Worsening pressure gradient
3. Symptoms

Question 30

How often should adults with repaired coarctation undergo cross-sectional/3D imaging?

Answer 30

Every 5 years (looking for aneurysm)

Question 31

Ventricular dilation and low resting EF in otherwise healthy teen can be what?

Answer 31

Athletes heart (volume overload from high output leads to dilation)

Question 32

Differential for a change in QRS?

Answer 32

Intermittent preexcitation
Aberrant conduction
Ventricular rhythm
Twin AV nodes

Question 33

Ecg patterns with digoxin toxicity?

Answer 33

Slopes diffuse ST depression
AV block
Bidirectional VT

Question 34

Treatment for digoxin toxicity?

Answer 34

Antidote (often refractory to defibrillation)

Question 35

What happens to digoxin level with amiodarone?

Answer 35

Increases

Question 36

When is an upright T wave in V1 normal?

Answer 36

1st week of life
Adolescence and beyond

Question 37

Severity of pulmonary valve stenosis by peak velocity/gradient?

Answer 37

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

Question 38

What are the anatomic borders of the aortic isthmus?

Answer 38

Left subclavian and ductus arteriosus

Question 39

Most common coronary abnormality in TOF?

Answer 39

Conal branch (accessory LAD) from RCA 10-15%
LAD from RCA coursing anteriorly to RVOT 5%

Question 40

Most common great artery relationship in tricuspid atresia?

Answer 40

Normally related (75%)

Question 41

How is Ca2+ removed during myocyte relaxation?

Answer 41

Ca2+ ATPase SERCA pumps on sarcoplasmic reticulum

Question 42

What percent of patients with d-TGA have a VSD?

Answer 42

40-45%

Question 43

Which atria are the limbus and valve of the fossa ovalis in?

Answer 43

limbus - RA
valve - LA

Question 44

When is fetal hemoglobin (alpha/gamma) replaced with adult hemoglobin (alpha/beta)?

Answer 44

3 months of age
Fetal has higher affinity for O2

Question 45

How does norepinephrine activate beta 1 receptor?

Answer 45

Gs subunit > activates adenylate cyclase > ATP to cAMP > activates protein kinase a

Question 46

What causes an ostium primum ASD?

Answer 46

Endocardial cushion failure

Question 47

LSVC is persistence of what embryologic structure?

Answer 47

Left horn of the sinus venosus

Question 48

How much deoxyhemoglobin must be present for visible cyanosis?

Answer 48

5 g/dL (so may not be visible in anemic patients)

Question 49

What protein binds calcium allowing cross bridges to form?

Answer 49

Troponin C

Question 50

Living at altitude has what effect on PA pressure?

Answer 50

Elevated, especially with exercise (higher incidence of PDA)

Question 51

What does second heart sound like in d-TGA?

Answer 51

Loud, single (because of anterior aorta)

Question 52

DORV is most commonly associated with aortic or pulmonary stenosis?

Answer 52

Pulmonary stenosis (half)

Question 53

Contractile myocytes (not smooth muscle) are found in what vessels?

Answer 53

Pulmonary veins just before insertion into LA

Question 54

Most common type of truncus?

Answer 54

Type I (MPA from left/posterior side of truncus) 50-65%
Type II 30-45%
Type III 5-10%

RAA in 35%

Question 55

Which PA is most commonly absent in truncus and TOF?

Answer 55

TOF - opposite side from aortic arch
Truncus - same side from aortic arch

Question 56

Which embryologic aortic arch typically regresses?

Answer 56

Fifth

Question 57

Borders of the triangle of Koch?

Answer 57

Coronary sinus ostium, septal leaflet of TV, tendon of Todaro

Question 58

What is the valve at the junction between the great cardiac vein and the coronary sinus?

Answer 58

Vieussens valve

Question 59

What is the pH change for acute respiratory acidosis?

Answer 59

Decrease by 0.08 for each increase in PaCO2 by 10 mmHg

Question 60

Metabolic compensation for respiratory acidosis

Answer 60

Bicarb increase by 3.5*(change in PaCO2/10)

Question 61

What is negative neurodevelopment effect of loop diuretic?

Answer 61

Ototoxicity, related to peak level (slowing infusion may be useful), bumetanide less toxic

Question 62

What is responsible for the high PVR in fetus?

Answer 62

Low alveolar and blood oxygen tension

Question 63

What is the coronary supply of the AV node?

Answer 63

Right coronary artery

Question 64

What is the law of Laplace?

Answer 64

Wall Tension = pressure*radius for a thin-walled sphere or cylinder

Wall Stress = Wall Tension / 2thickness
Wall Stress = pressureradius / 2*thickness

Question 65

Steps of the actin/myosin power stroke?

Answer 65

-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

Question 66

When to suspect familial hypercholesterolemia

Answer 66

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

Question 67

Cardiac diagnoses associated with pectus excavatum

Answer 67

Mitral valve prolapse or aortic root dilation (isolated or with Margan, Loeys-Dietz, Ehlers-Danlos)

Question 68

What is the law of Laplace?

Answer 68

Wall Tension = pressure*radius for a thin-walled sphere or cylinder

Wall Stress = Wall Tension / 2thickness
Wall Stress = pressureradius / 2*thickness

Question 69

Steps of the actin/myosin power stroke?

Answer 69

-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

Question 70

When to suspect familial hypercholesterolemia

Answer 70

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

Question 71

Cardiac diagnoses associated with pectus excavatum

Answer 71

Mitral valve prolapse (isolated or with Margan, Loeys-Dietz, Ehlers-Danlos)

Question 72

What is Danon disease?

Answer 72

Glycogen storage disease IIb
Hypertrophic cardiomyopathy, intellectual disability, WPW, skeletal myopathy

LAMP2 mutation (lysosomal associated membrane protein)

Question 73

Fabry Disease

Answer 73

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

Question 74

In combination with R aortic arch, what causes complete vascular ring?

Answer 74

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

Question 75

What is the Belhassen VT pattern?

Answer 75

RBBB, left axis deviation, Q in 1 and aVL (left to right), inferior leads negative QRS (bottom to top)

Question 76

Electrocardiographic sign for ALCAPA?

Answer 76

Deep Q waves in I and aVL

Question 77

What is Danon disease?

Answer 77

Glycogen storage disease IIb
Hypertrophic cardiomyopathy

Question 78

Fabry Disease

Answer 78

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

Question 79

In combination with R aortic arch, what causes complete vascular ring?

Answer 79

Draw from the top in axial view: aberrant L subclavian and L PDA

Question 80

What is the ligament of Marshall?

Answer 80

Vestige of left cardinal vein, anterior to pulmonary veins, LPA

Question 81

If there is tricuspid-aortic continuity, what type of VSD is present?

Answer 81

perimembranous

Question 82

“Pentalogy” of Fallot adds what defect present in 80% of TOF patients?

Answer 82

ASD or PFO

Question 83

What is effect of ANP on kidney?

Answer 83

-Dilating the afferent arteriole and constricting the efferent arteriole
-Distal tubules to decrease sodium resorption

Question 84

Cardiac situs is determined by the position of what structure?

Answer 84

right atrium

Question 85

What is the ligament of Marshall?

Answer 85

Vestige of left cardinal vein, anterior to pulmonary veins, LPA

Question 86

Which great vessels are intra-pericardial?

Answer 86

Ascending aorta, MPA, terminal SVC

Question 87

Borders of the transverse sinus?

Answer 87

atria vs. great vessels

Question 88

Single, firm, intramural tumors involving the ventricular free wall or septum is likely to be what?

Answer 88

Fibroma

Question 89

HOCM murmur louder/softer

Answer 89

Louder: isoproterenol, nitroglycerin, exercise, standing from squat, straining portion of Valsalva
Softer: phenylephrine

Question 90

What CHD is phenylketonuria associated with?

Answer 90

Left-sided lesions (HLHS, coarct)
Septal defects
TOF

Question 91

Superior axis (left or NW) is associated with which CHD?

Answer 91

AVSD, tricuspid atresia

Question 92

Most common viral causes of myocarditis?

Answer 92

Coxsackie and adenovirus
Rare: CMV, parvo, fluA, HSV, EBV, HIV, RSV

Question 93

IAA without a VSD is associated with what CHD?

Answer 93

AP window

Type A interrupted arch (think of it like severe coarctation)
Type B most common in DiGeorge
Type C very rare

Question 94

Still’s mumur is best heard in what position?

Answer 94

supine

Question 95

What causes an ANTERIOR esophageal indentation?

Answer 95

PA sling

Question 96

Well-circumscribed, noncapsulated, intramural, or intracavitary nodules, bright appearance on echo

Answer 96

Rhabdomyoma

Question 97

Retinoic acid results in what category of defect?

Answer 97

Conotruncal

Question 98

Obstruction, emboli, and B symptoms attached to atrial septum?

Answer 98

Myxoma - most common adult cardiac tumor, second most common childhood cardiac tumor, 75% located in LA

Question 99

Heart disease associated with WPW?

Answer 99

Hypertrophic cardiomyopathy
LVNC
Ebstein anomaly
ccTGA

Question 100

What systolic murmur increases in intensity after PVC?

Answer 100

Aortic stenosis

Question 101

Most common CHD associated with ccTGA?

Answer 101

VSD (80%)

Question 102

Additional echo finding to look for in dTGA with VSD?

Answer 102

Sub-pulmonary stenosis (LVOTO) - needs REV or Nikaidoh

Question 103

Taussig-Bing anomaly

Answer 103

DORV, side by side GA, subpulmonary VSD (TGA physiology, cyanotic)

Question 104

Valsalva effect on murmurs

Answer 104

Forced exhale - decreases venous return (so most murmurs decrease, exception is HOCM and MR)

Question 105

Constrictive pericarditis vs. restrictive cardiomyopathy

Answer 105

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

Question 106

What portion of the aorta is at risk of enlargement with bicuspid aortic valve?

Answer 106

Ascending (root with Marfan/Loey’s Deitz)

Question 107

Natural history of rhabdomyomas in tuberous sclerosis?

Answer 107

Most have complete resolution

Question 108

Friction rub is loudest in what position?

Answer 108

Leaning forward

Question 109

Q waves appear where in ccTGA?

Answer 109

right precordial leads (as opposed to left precordial leads as in normal patient)

Question 110

What is most common CHD in congenital Rubella?

Answer 110

pulmonary stenosis and PDA

Question 111

Most common cause of sudden death in athletes?

Answer 111

HOCM (44%), coronary anomalies (17%)

Question 112

Giant cell myocarditis

Answer 112

• 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

Question 113

Consequences of premature ductal constriction?

Answer 113

RV dilation/hypertrophy
Hydrops
PVR up (more pulm flow)
RV hypoplasia/PS (right to left at asd)

Question 114

What is treatment for familial chylomicronemia syndrome?

Answer 114

Reduce fat intake to <10-15% of calories
No medications currently approved by FDA
Genetically mediated deficiency in lipoprotein lipase

Question 115

Natural history of SVT in infants

Answer 115

Most resolve after 1st year

Question 116

Differential diagnosis of short RP tachycardia

Answer 116

Atrioventricular reentrant tach
Typical AVNRT
Junctional tachycardia

Question 117

What causes increased a wave in RA and LA tracing?

Answer 117

Elevated RVEDP
Tricuspid stenosis
Complete heart block

Elevated LVEDP
Mitral stenosis
AV dyssynchrony

Question 118

Fick equation?

Answer 118

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]

Question 119

Fick equation?

Answer 119

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]

Question 120

Qeff

Answer 120

Qeff = VO2 / content in PV - MV

Question 121

Easy calculation for PVR/SVR when Qp:Qs = 1

Answer 121

Transpulmonary gradient - Transsystemic gradient

Question 122

Allergic reaction from Amplatzer device most likely caused by

Answer 122

Nickel allergy (titanium-nickel compound)

Question 123

When are you unable to calculate Q?

Answer 123

Multiple sources of flow with different saturations

Question 124

Patients taking NPH insulin are at risk of hypersensitivity to what medication used in cardiac surgery?

Answer 124

Protamine

Question 125

What is incidence of device erosion for Amplatzer ASD occluder?

Answer 125

1 / 1,000 (0.1%)
Gore has none

Question 126

Which has highest incidence of acute aortic wall injury as treatment for coarctation in older kids - balloon, stent, or surgery?

Answer 126

Balloon

Question 127

Restrictive vs constrictive

Answer 127

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

Question 128

Restrictive vs constrictive

Answer 128

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

Question 129

ACHD cath balloon valvuloplasty indications

Answer 129

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)

Question 130

Class I indications for pediatric pulmonary valvuloplasty

Answer 130

• Critical PS (cyanosis, PDA dependence)
• Peak gradient >= 40 mmHg (echo or cath)
• Significant stenosis with RV dysfunction

*<15% risk of recurrence

Question 131

Indications for transcatheter ASD closure

Answer 131

• Hemodynamically significant, suitable anatomy (class I)
• TIA/stroke or symptomatic cyanosis with transient R-L (class IIa)
• Hypercoagulable, transvenous pacing system (class II)

Question 132

What’s associated with 3-methylglutaconic aciduria type II (Barth syndrome)?

Answer 132

Neutropenia
Dilated cardiomyopathy / LVNC
Proximal skeletal myopathy
Growth failure
Endocardial fibroelastosis

tafazzin (TAZ) mutation, X-linked

Question 133

What’s associated with 3-methylglutaconic aciduria type II (Barth syndrome)?

Answer 133

Neutropenia
Dilated cardiomyopathy
Proximal skeletal myopathy/weakness
Hypotonia
Growth failure
Endocardial fibroelastosis
3-methylglutaconic aciduria

Question 134

22q11.2

Answer 134

Mutation in TBX1
DiGeorge
IAA-B, truncus, right aortic arch, TOF, conotruncal VSD

Question 135

JAG1

Answer 135

Alagille
Peripheral PA hypoplasia, TOF

Question 136

7q11, ELN mutation

Answer 136

Wiliams
Supravalvar AS, PPS

Question 137

PTPN11

Answer 137

Noonan
PS, HCM, PAPVR
Nuchal translucency, cystic hygroma, polyhydramnios

Question 138

Turner (XO)

Answer 138

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

Question 139

Right aortic arch is most often seen in which form of TOF?

Answer 139

TOF/PA/MAPCAS (PA with VSD)

Question 140

Most common post-op issue following sano shunt?

Answer 140

PA stenosis

Question 141

In what scenario do you worry about retrograde coarctation?

Answer 141

Ductal stent in hybrid procedure

Question 142

What is a classic Glenn?

Answer 142

RSVC to RPA, disconnect RPA from LPA

Question 143

What is a charged Glenn?

Answer 143

Same as pulsatile Glenn - pulm valve left patent

Question 144

LPA stent for Glenn

Answer 144

• Should only be considered if can be dilated to adult size except in extreme situations
• Stent placement in Glenn want gradient < 3-5 mmHg

Question 145

Treatment for fetal SVT

Answer 145

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)

Question 146

Treatment for fetal SVT

Answer 146

Digoxin first line, high level (2)
Fetal transfer decreases in presence of hydrops

Question 147

Risk factors for sudden death in HOCM

Answer 147

• Septal thickness >3cm
• Family history (?)
• Syncope, non-neurally mediated
• BP decrease or inadequate rise with exercise
• NSVT
• Late gadolinium enhancement on MRI

Question 148

Amiodarone increases effects of what medications?

Answer 148

Warfarin, digoxin, phenytoin, class I antiarrhythmics

Question 149

Treatment for Lyme disease

Answer 149

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

Question 150

What are symptoms of Kearns-Sayre Syndrome?

Answer 150

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

Question 151

Prognosis of Long QT with 2:1 AV block

Answer 151

Poor (50% mortality in infancy)
Beta blocker and pacemaker

Question 152

First line treatment for WPW with SVT?

Answer 152

Propranolol
Second line: flecainide

Question 153

First line treatment for WPW with SVT?

Answer 153

Propranolol

Question 154

Normal V1 T wave

Answer 154

positive at birth
negative 1 week - teenage
positive after teenage

Question 155

P-wave morphology in PJRT

Answer 155

Deeply negative p-waves in II, III, and aVF

Question 156

Most common sequelae of maternal amiodarone

Answer 156

Neonatal thyroid dysfunction

Question 157

Class I indications for pacemaker in kids

Answer 157

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

Question 158

Class I indications for pacemaker in kids

Answer 158

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

Question 159

What does third letter of pacemaker notation mean?

Answer 159

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

Question 160

What does third letter of pacemaker notation mean?

Answer 160

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

Question 161

Most common side effects of beta blockers in kids

Answer 161

Behavioral changes, depression, mood swings most common

Lightheadedness, tiredness, headache, nightmares, difficulty sleeping, heartburn, diarrhea, constipation

Question 162

Define AH jump

Answer 162

Change of >50ms with 10ms change in premature stimulus

(suggests dual AV node pathways)

Question 163

Genetic cause of CPVT

Answer 163

Ryanodine receptor gene (RYR2) or calsequestrin 2 gene (CASQ2)

Question 164

Adverse effects of amiodarone

Answer 164

Photosensitivity
Thyroid dysfunction
Weakness/peripheral neuropathy
Corneal microdeposits
Elevated hepatic enzymes

Question 165

Which electrolyte derangements make digoxin toxicity worse?

Answer 165

Hypokalemia
Hypomagnasemia

Question 166

Treat digoxin toxicity

Answer 166

SVT - beta blocker (esmolol)
VT - lidocaine or phenytoin
Antibody
Correct hypoK and hypoMg
Temporary pacing

Question 167

What is rate responsiveness in pacemaker?

Answer 167

Increases minimum rate with activity

Question 168

What is effect of magnet over ICD?

Answer 168

Disables shock therapy, does not change pacing (only as long as magnet is in place)

Different from magnet over pacemaker which enables asynchronous pacing

Question 169

Causes of left axis deviation on ECG?

Answer 169

AVSD (or primum ASD)
Tricuspid atresia
WPW

Question 170

Impact of hypercalcemia on ECG?

Answer 170

Short QTc

Question 171

Normal HV interval?

Answer 171

35 to 70 ms
Short HV is <25 ms, indicates an alternative method of conduction other than AV node

Question 172

Prognostic value of loss of preexcitation in a single beat on exercise test

Answer 172

Accessory pathway is less likely to rapidly conduct to the ventricle in afib, less likely to cause sudden death

Question 173

Treatment for Brugada patients with syncope or sudden death

Answer 173

ICD placement

Question 174

Irregular wide complex tachycardia

Answer 174

Think atrial fibrillation with BBB or preexcitation

Avoid adenosine - don’t want to promote conduction down the accessory pathway

Question 175

What is a Mahaim fiber tachycardia?

Answer 175

antegrade only accessory pathway

Question 176

Provocation of Brugada syndrome?

Answer 176

High lead placement (V1, V2)
Procainamide
Fever

Question 177

Class I indications for biventricular pacing (resynchronization)

Answer 177

LBBB, QRS > 150ms, NYHA class II or worse
Usually only beneficial when EF < 35%, only indicated after medical therapy is optimized

Question 178

NKX2.5

Answer 178

Hear block and ASD

Question 179

PKP2

Answer 179

Arrhythmogenic right ventricular cardiomyopathy

Question 180

NOTCH1 mutations

Answer 180

Left-sided disease - aortic valve stenosis, bicuspid aortic valve, coarctation of the aorta, HLHS

Question 181

Transvenous pacing system contraindicated in what CHD?

Answer 181

Fontan - risk of thrombus in systemic circulation

Question 182

Ebstein’s ECG finding without pre-excitation

Answer 182

RBBB (atresia, short length, narrow caliber, fibrosis)

Question 183

Why is carvedilol good for heart failure?

Answer 183

Non-selective - block alpha activity too

Question 184

What is Ashman phenomenon?

Answer 184

Aberrancy during tachycardia due to decreased cycle length

Question 185

What happens to risk of atrial fibrillation if you ablate WPW pathway?

Answer 185

Decreases - it’s thought that their atrial fib starts as svt and degenerates

Question 186

Risk factors for atrial fibrillation

Answer 186

Thyroid abnormalities
Drugs of abuse
First degree relative with a fib
Prolonged PR at baseline
Obesity
OSA in adults

Question 187

What is post-ligation cardiac syndrome?

Answer 187

After PDA closure, depressed LV function/hypotension due to:
1. Sudden increase in afterload
2. Sudden decrease in preload

Question 188

Cath vs. echo peak gradient

Answer 188

Echo peak instantaneous pressure gradient higher than cath peak to peak (echo mean typically correlates more closely with cath peak to peak)

Question 189

Indications for pediatric aortic valvuloplasty

Answer 189

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

Question 190

Most common genes implicated in familial hypercholesterolemia

Answer 190

LDLR
apolipoprotein-b (apo-B)
pro-protein convertase (PCSK9)
LDL receptor adapter protein (LDLRAP1)

Question 191

Criteria for clinical diagnosis of homozygous familial hypercholesterolemia

Answer 191

-Genetic confirmation
-Untreated LDL>500, treated LDL>300
-Cutaneous or tendon xanthomas before age 10
-Untreated elevated LDL in both parents

Question 192

Therapies for homozygous familial hypercholesterolemia

Answer 192

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

Question 193

Cardiac manifestations of NF1

Answer 193

Pulmonary valve stenosis
Mitral valve prolapse
Septal defects
Hypertrophic cardiomyopathy

Question 194

Cardiac manifestations of alpha galactosidase A mutation

Answer 194

Fabry disease - LVH, valvular abnormalities, systemic hypertension, atherosclerotic disease of coronary and cerebral arteries

Question 195

Vascular ring components with RAA

Answer 195

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

Question 196

Catheter courses

Answer 196

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

Question 197

Effect of azygous vein on Glenn

Answer 197

Basically a giant venovenous collateral - cyanosis

Question 198

Scimitar Syndrome

Answer 198

• 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

Question 199

What is aorta/pulmonary valve position in ccTGA?

Answer 199

aorta is anterior and leftward of pulmonary valve

Question 200

LSVC derives from what embryologic structure?

Answer 200

Left anterior cardinal vein

Question 201

Left and right vitelline veins fuse to form what?

Answer 201

Portal vein

Question 202

Mild/moderate/severe for regurgitation fractions on MRI?

Answer 202

<20% mild
20-40% moderate
>40% severe

retrograde volume / antegrade volume

Question 203

What is normal Right/Left lung perfusion percent?

Answer 203

55% to right, 45% to left

Think 3 lobes vs. 2 lobes

Question 204

Is lung perfusion scan safe with a right-to-left shunt?

Answer 204

Yes

Question 205

Caveats for lung perfusion scan

Answer 205

• 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)

Question 206

First line and adjunctive therapies for PPHN?

Answer 206

First: iNO
Second: milrinone if LV dysfunction, sildenafil, inhaled iloprost

Question 207

KCNH2 loss of function

Answer 207

Long QT type 2

Treat with nadolol
In patients with aborted arrest consider ICD and/or sympathectomy

Question 208

Echo findings in sickle cell

Answer 208

LV dilation and diastolic dysfunction (high output, chronic ischemia)

Question 209

Differential for bidirectional VT

Answer 209

1. CPVT
2. Long QT type 7 (Andersen-Tawil)
3. Digoxin toxicity

Question 210

KCNJ2

Answer 210

Long QT type 7, Andersen-Tawil - periodic muscle paralysis and dysmorphic features

Question 211

ECG findings in ARVC

Answer 211

• Epsilon waves
• T wave inversion in right precordial leads (V1 to V3)

Question 212

Normal PDA Doppler over first days of life

Answer 212

Initially bidirectional
Low velocity L-R
High velocity L-R

Question 213

How does Valsalva break SVT?

Answer 213

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

Question 214

What are Howell Jolly bodies

Answer 214

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

Question 215

MYH7

Answer 215

myosin heavy chain 7, mutations associated with hypertrophic cardiomyopathy

Question 216

Use pulmonary regurgitation Doppler to estimate PA pressures

Answer 216

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

Question 217

Sports restriction

Answer 217

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

Question 218

Where are the 4 critical isthmuses for VT in repaired TOF?

Answer 218

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

Question 219

Class I recommendations for heart failure management?

Answer 219

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)

Question 220

ALCAPA echo findings

Answer 220

-Coronary flow INTO the PA
-Reversal of flow in the left coronary
-Dilation of the RCA
-Mitral regurgitation, echobright papillary muscles
-LV dysfunction/dilation

Question 221

Definition of accelerated ventricular rhythm

Answer 221

Within 10-15% of sinus rate. In a newborn with no other issues it’s benign, resolves spontaneously within the first year

Question 222

What is range ambiguity in Doppler?

Answer 222

Goes along with continuous wave - can’t tell where the specific velocity came from (high PRF or high Nyquist limit) can’t tell location

Question 223

Grades of diastolic dysfunction on AV valve and pulm vein Doppler

Answer 223

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

Question 224

Contrast echo

Answer 224

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

Question 225

Goals of acute HF management

Answer 225

• Decrease afterload (lower SVR as BP permits)
• Increase inotropy
• Optimize preload with diuretics if able
• PPV
• Decrease metabolic demand

Question 226

Dose-dependent effects of dopamine

Answer 226

<10 microgram/kg/min, mostly B1 and B2 (chronotropy, inotropy)

High dose alpha1 and conversion to norepi

Question 227

Infectious causes of heart block

Answer 227

Lyme
Rheumatic carditis
Endocarditis
Chagas
Viral myocarditis

Question 228

Signs of shunt occlusion in Norwood

Answer 228

-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

Question 229

How do you find preexcitation on EP intracardiac tracing?

Answer 229

His - ventricle <30ms (measured to earliest QRS on surface ECG)

Look for first ventricular activation, can tell you where an accessory pathway is

Question 230

Pathogenesis of ARVC

Answer 230

Mutations in cardiac desmosome (desmoplakin, plakophilin 2, desmogelin 2, desmocollin 2) leading to replacement of RV myocardium with fibrofatty tissue

Usually autosomal dominant

Question 231

What is typical PVC/VT morphology for AVRC?

Answer 231

Superior axis (negative in II, III, aVF) and LBBB (positive in aVL)

Can have other morphologies

Question 232

Exercise in AVRC?

Answer 232

Restriction from all competitive sports with possible exception of low-intensity class 1A sports because can lead to disease progression

Question 233

Takayasu arteritis

Answer 233

-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

Question 234

Fontan follow up schedule

Answer 234

• Echo: annually
• End-organ testing by age group: preteen Q3-4y, adolescent Q1-3y, adults Q1-2y

Question 235

Duration of afib after which have to anticoagulate/look for thrombus?

Answer 235

48 hours

Question 236

Normal lipid values

Answer 236

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

Question 237

Management of familial hypercholestrolemia

Answer 237

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

Question 238

Guidelines to review

Answer 238

https://www.ahajournals.org/doi/10.1161/CIR.0b013e31821b1f10

https://www.ahajournals.org/doi/full/10.1161/CIR.0000000000000458

CARPREG (Cardiac Disease in Pregnancy)

Question 239

Highest risk factors in pregnancy and CHD

Answer 239

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

Question 240

Dose response curve for partial agonist and noncompetitive antagonist

Answer 240

Noncompetitive antagonist (isoproterenol, propranolol) shifts curve to right – higher dose for same effect

Partial agonist shifts curve to right and decreases efficacy

Question 241

Interpretation of respiratory exam findings

Answer 241

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

Question 242

Type 1 vs. type 2 LPA sling

Answer 242

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

Question 243

Cardiopulmonary interactions

Answer 243

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

Question 244

Pediatric aortic balloon valvuloplasty indications

Answer 244

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)

Question 245

Genetic mutations for LQT types 1, 2, and 3

Answer 245

KCNQ1 (loss) - gain = short QT
KCNH2 (loss)
SCN5A (gain) - loss = Brugada

Question 246

Nondihydropyridine CCB

Answer 246

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

Question 247

Echo for chest pain indications

Answer 247

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

Question 248

Flecainide toxicity

Answer 248

Wide QRS, long QT from wide QRS, long PR

Give lipids and sodium as sodium bicarbonate

Question 249

Management of isolated ventricular ectopy

Answer 249

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

Question 250

Treatment for infant with verapamil sensitive VT?

Answer 250

beta blocker - don’t use CCB in infant

Question 251

How to perform atrial electrogram?

Answer 251

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

Question 252

Determine ECG rate based on small boxes

Answer 252

1 small box = 40 ms
Rate = 60,000 / (#sm box * 40)

Question 253

How fast to pace for flutter

Answer 253

110% to 130% of flutter rate

Question 254

Hemodynamic consequences of OSA

Answer 254

• Pulmonary hypertension
• Systemic hypertension
• Increased HR variability
• LV hypertrophy

Question 255

Gorlin equation

Answer 255

valve area = cardiac output / (systolic ejection period x HR x 44.3 * sqrt(mean pressure gradient))

Question 256

What are neural crest cells responsible for?

Answer 256

Septation of arterial trunk, semilunar valves

Question 257

What line changes on ventricular pressure volume loop with inotropy?

Answer 257

ESPVR slope increases or decreases with inotropy; where you are on the line changes with afterload

Question 258

Most common cause of death/need for early intervention in infantile marfan?

Answer 258

Mitral valve disease
Average life expectancy <2 years

Question 259

Non-fasting lipid screening for patients with risk factors

Answer 259

Total cholesterol>200, HDL<45, or non-HDL>145 requires a follow-up fasted sample

Question 260

Highest risk cholesterol patients

Answer 260

Kawasaki with aneurysm and transplant with coronary disease - threshold for statin is LDL>130; moderate risk is LDL>160

Question 261

Treatment for fetal heart block caused by SSA/SSB antibodies

Answer 261

In utero steroids
IVIG
Hydroxychloroquine being investigated

Question 262

In mom with SSA/SSB antibodies, what is risk of fetus with CHB?

Answer 262

2% in first pregnancy
20% in second if first baby had it

Question 263

Treatment for newborn with complete heart block

Answer 263

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

Question 264

HCM vs. athlete’s heart

Answer 264

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

Question 265

Most frequent genetic mutation in patient’s with TOF?

Answer 265

22q11 (20% of TOF)

Question 266

Genetic cause of Alagille syndrome?

Answer 266

JAG1 and NOTCH2

Question 267

Differential for short RP tachycardia

Answer 267

AVRT
JET
Typical AVNRT

Question 268

Treatment for congenital JET in infancy

Answer 268

Amiodarone first line
Often need B-blocker and/or felcainide too

Question 269

What embryologic processes result in criss-cross AV valves and superior-inferior ventricles?

Answer 269

Twisting - criss cross valves
Tilting - superior inferior ventricles

Question 270

Stages of hypertension

Answer 270

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

Question 271

Management of HTN

Answer 271

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

Question 272

Definition of LVH by mass

Answer 272

> 115g/BSA (boys), >95 g/BSA (girls)

> 51 g/m2

Question 273

Differential diagnosis for wide complex tach in infant

Answer 273

1. Antidromic SVT
2. VT
3. SVT with aberrancy
4. Sinus/atrial/junctional tach with BBB or preexcitation

Question 274

Hockeystick appearance of anterior mitral valve leaflet

Answer 274

Rheumatic heart disease

Question 275

Tamponade criteria

Answer 275

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)

Question 276

Nyquist limit and pulse repetition frequency

Answer 276

Nyquist limit = PRF/2 (highest velocity that can be accurately resolved), can increase with lower frequency transducer

Question 277

Assessment of severity of aortic insufficiency on echo

Answer 277

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

Question 278

Formula for myocardial performance index (Tei index)

Answer 278

(isovolumetric contraction time + isovolumetric relaxation time) / ventricular ejection time

Question 279

Echo findings constrictive pericarditis

Answer 279

-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

Question 280

Restrictive LV physiology

Answer 280

E:A >2
Decel time <160ms
Decreased E’
Increased E/E’ >15

Question 281

Rate-corrected velocity of circumferential fiber shortening

Answer 281

VCFc = (shortening frac/ejection time)/sqrt(RR); preload independent

Stress-velocity index: relationship of VCFc to the end systolic wall stress (ESWS), load independent

Question 282

Most common type of VSD associated with coarctation of the aorta?

Answer 282

Perimembranous

Next most common is posterior malalignment VSD

Question 283

Single inlet / common inlet

Answer 283

Refers to the atria - single means just one atrium empties (e.g. tri atresia), common means one AVV empties both atria

Question 284

Size of brachiocephalic/innominate vein with LSVC?

Answer 284

Small or absent - inversely proportional to size of coronary sinus

Question 285

Reminder to distinguish between:
1. Diastolic dysfunction
2. Restrictive physiology
3. Constrictive pericarditis
4. Tamponade

Answer 285

Constrictive - exaggerated mitral filling, exaggerated A wave reversal in hepatic vein with expiration

Question 286

What number is considered for surgical decision making in HOCM?

Answer 286

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)

Question 287

List TEE vs. TTE maneuvers

Answer 287

TEE:
multiplane
anteflexion/retroflexion
advance/retract
clockwise/counterclockwise rotate

TTE:
Tilt
Slide
Angle
Rock

Question 288

What devices cause most artifact on MRI?

Answer 288

Stainless steel: PDA coil, occluder devices

Less artifact: pacemaker lead, PDA clip, nonferromagnetic stent

Question 289

MRI sequences

Answer 289

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

Question 290

Delayed myocardial enhancement in infarct vs. myocarditis

Answer 290

Infarct - subendocardial or transmural

Myocarditis - subepicardial or midmyocardial, patchy

Question 291

Best imaging for constrictive pericarditis?

Answer 291

Non-contrast CT

Question 292

First and second line treatment for Long QT type 3

Answer 292

1. Nadolol
2. Mexilitine

Question 293

What class of antiarrhythmics are contraindicated in long QT syndrome?

Answer 293

Class III (potassium channel blockers) - lengthen the QTc

Question 294

Type A vs. Type B dissection

Answer 294

A involves ascending aorta (most common), B is all other types

Question 295

Post-coarctectomy syndrome

Answer 295

Most common after surgical coarct repair but can happen with transcatheter - HTN from rebound sympathetic activtion, mesenteric arteritis and abdominal pain

Question 296

POTS - postural orthostatic tachycardia syndrome

Answer 296

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

Question 297

Risk factors for poor outcomes in Ebstein

Answer 297

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

Question 298

Celermajer index

Answer 298

(area of RA + area of atrialized RV) / (area of RV + area of left heart)

Higher number is worse

Question 299

Associations with RAA with aberrant L subclavian

Answer 299

Extracardiac - gastrointestinal, urinary tract

22q11 deletion (10% of RAA patients)

Mostly asymptomatic at birth, 80% have not had surgery by 2 years of age

Question 300

Minimum criteria for acute kidney injury

Answer 300

Increase in creatinine >0.3 from baseline or 1.5x baseline, UOP < 0.5 mL/kg/hr

Question 301

Management of PVCs

Answer 301

> 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

Question 302

In patients with R-L shunt, EtCO2/PaCO2 is equal to what?

Answer 302

Qp:Qs

Question 303

Aortic root dilation indication for surgery

Answer 303

> 5.5cm or >5cm at center for excellence or with risk factors for early dissection

Question 304

Severe aortic regurg indications for intervention if asymptomatic

Answer 304

LVSD >5cm, LVEDD >6.5cm

Question 305

Exam findings with Type B interrupted arch and aberrant right subclavian

Answer 305

Both arms and legs desaturated (typically interrupted arch has differential cyanosis)

Question 306

Contraindications for PDA closure with ibuprofen or indomethicin

Answer 306

NEC
Grade 3 or 4 IVH
Clinical bleeding
Platelet <60K
UOP<1 mL/kg/h
Cr>1.5, BUN>20

Question 307

Holt-Oram syndrome

Answer 307

TBX5
Upper limb abnormalities
ASD or VSD
Conduction defects

Autosomal dominant

Question 308

Max VO2 indexing (mL/min vs. mL/kg/min)

Answer 308

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

Question 309

Highest peak VO2 achieved during which type of exercise stress test?

Answer 309

Treadmill

Question 310

How much does each thing increase with exercise:
HR
SV
minute ventilation
RR
VO2

Answer 310

HR - 2-3x
SV - 1.5-2x (least)
minute ventilation - 10x
RR - 2-3x
VO2 - 10-20x

Question 311

Blood pressure change with isometric exercise (constant muscle length - holding a weight)

Answer 311

SBP and DBP both increase significantly

Question 312

Blood pressure change with isotonic exercise

Answer 312

DBP decreases at least 10 mmHg

Higher BP generally with cycle ergometry (hand grip on bars)

Question 313

Normal VO2 max?

Answer 313

11yo 35 mL/kg/min
Post pubertal male 45-50
Post pubertal female 35-40
Anemic patients lower

Treadmill > cycle > arm crank > hand grip

Question 314

Expected max HR

Answer 314

For patients age 5 to 20, 195-215 bpm

For older: 220-age

Question 315

Changes in max RR, tidal volume, and peak minute ventilation with age

Answer 315

Tidal volume and max minute ventilation increase

Max RR same (or slight decrease)

Question 316

Power and work equations

Answer 316

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

Question 317

Risk level of regurgitant lesions with exercise testing

Answer 317

All regurgitant lesions are low risk

Question 318

Explain VE/VCO2

Answer 318

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

Question 319

Acetylene-helium rebreathing

Answer 319

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

Question 320

Blood pressure cuff size

Answer 320

Bladder length 80% of arm circumference

Width 40% of arm circumference

Question 321

Rule of nine in exercise ventilation

Answer 321

For every 25 watts increase in power, minute ventilation increases by 9 L/min

Question 322

Prediction of MVV based on FEV1

Answer 322

FEV1 x 35 or 40

Max exertional minute ventilation is 60-80% of MVV

Question 323

Work equation

Answer 323

Work = Force x Distance
unit is Newton-meter (J)

Force = Mass x Acceleration

Power = Work/Time

Question 324

CO2 rebreathing technique

Answer 324

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)

Question 325

What happens in pacemaker Wenckebach

Answer 325

Upper rate limit set at 180 bpm, once atrial rate reaches that, won’t pace, so “drop beats”

Question 326

Most common benign murmur in teenagers?

Answer 326

Pulmonary flow murmur - II/VI systolic ejection murmur at second left intercostal space

Question 327

Activity restrictions in Kawasaki Disease

Answer 327

If normal coronaries, no restrictions in physical activity beyond 6-8 weeks from diagnosis

Question 328

Return to sports after myocarditis including MISC

Answer 328

Echo, Holter and exercise ECG 3-6mo after initial illness

Question 329

MISC vs. KD

Answer 329

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

Question 330

Lab diagnosis of KD

Answer 330

Hypoalbuminemia <3
Anemia for age
ALT elevation
Platelet count >450K (after 7d)
WBC >15K
UA with >10 WBC and no bacteria

Question 331

Aortopathy genes
FBN1
TGFBR1
COL3A1
ADAMTS10
ACTA2

Answer 331

FBN1 - Marfan
TGFBR1 - Loeys-Dietz and familial thoracic aortic aneurysm
COL3A1 - Ehlers-Danlos
ADAMTS10 - Weill-Marchesani
ACTA2 - familial thoracic aortic aneurysm

Question 332

Highest risk endocarditis (need for prophylaxis)

Answer 332

• 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

Question 333

IE prophylaxis in penicillin allergic

Answer 333

PO azithromycin, cephalexin, clindamycin, clarithromycin

Question 334

Toxic anthracycline dose

Answer 334

Within 6 years of treatment, 65% of kids who received 228 to 550 mg/m2 hav dysfunction

Question 335

Regurgitant murmurs

Answer 335

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

Question 336

Neurodevelopmental outcomes in CHD

Answer 336

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

Question 337

Signs of flecainide toxicity

Answer 337

Prolonged PR, signs of heart failure

Look for it at 12 months when transitioning off formula

Question 338

Vein of Galen

Answer 338

More common in males
Associated lesions: none, sinus venosus defect, coarctation

Question 339

Indications for lipid screening before typical age 9-11

Answer 339

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

Question 340

Warfarin for non-cardiac procedures and mechanical valve

Answer 340

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

Question 341

Systolic clicks

Answer 341

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

Question 342

Brugada syndrome

Answer 342

More common in males, more arrhythmic events in men - higher testosterone thought to contribute

Inheritance is autosomal dominant

Question 343

Marfan diagnosis and management

Answer 343

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

Question 344

Alagille syndrome facies

Answer 344

Broad forehead, deep-set eyes, small pointed chin

Bile duct paucity, heart disease, skeletal (butterfly vertebrae) and ocular abnormalities

NOTCH2 gene

Question 345

Things that give higher yield for genetic testing in HCM

Answer 345

Younger age (<45y)
Reverse curve morphology
Wall thickness >20mm
Family history of HCM or sudden death

Question 346

Risk factors for sudden death in HCM

Answer 346

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

Question 347

Calcium disorders in genetic diagnoses

Answer 347

Williams syndrome: hypERcalcemia
DiGeorge syndrome: hypOcalcemia

Question 348

Medications during acute myocarditis

Answer 348

NSAIDs - don’t use (worsening inflammation, necrosis)

Ok: ACEi, ARB, spironolactone, CCB

Question 349

Dietary impact of healthy food on warfarin

Answer 349

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

Question 350

Recreational drug use and cardiac effects

Answer 350

Cocaine, amphetamines, and marijuana cause MI (synthetic cannabinoids like K2 or spice may be even more potent)

Cocaine and MDMA cause coronary thrombosis

Question 351

Incidence of endocarditis in Melody valve patients?

Answer 351

2.4% per patient year

Question 352

Indication to stop an exercise test

Answer 352

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

Question 353

VACTERL association

Answer 353

Vertebral anomalies
Anal atresia
Cardiac defects (40-80% of patients, VSD most common)
TracheoEsophageal fistula
Renal anomalies
Limb abnormalities

Single umbilical artery often seen

Question 354

CHARGE syndrome

Answer 354

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

Question 355

Risk factors for rehospitalization, re-operation, and death in TOF/absent pulm valve

Answer 355

Prematurity/low weight at operation
Neonatal repair
Longer bypass
Pre-op respiratory distress (more likely malacia)

Question 356

Sinus bradycardia pacemaker indications

Answer 356

Resting HR <40 bpm or pauses >3sec IIa in CHD or IIb in biventricular repair of CHD

Question 357

Factors that increase endothelial-derived nitric oxide production

Answer 357

Bradykinin
Serotonin
Vasopressin
Histamine

NG-monomethyl-L-arginine (L-NMMA) decreases

Question 358

Two most common arrhythmias after repair of AVSD

Answer 358

JET
Complete AV block

Question 359

Common late complication after Norwood presenting with low cardiac output

Answer 359

Distal arch obstruction

Question 360

Post-op complications from TAPVR repair

Answer 360

High LA filling pressures
Decreased pulmonary compliance
High PVR, RV dysfunction
Pulmonary edema
JET

Question 361

Effect of positive pressure ventilation on coronary flow

Answer 361

Decreases RV/aortic pressure gradient, decreasing RV coronary flow

Question 362

Formula for chylous effusion

Answer 362

Low in long chain triglycerides
High in medium chain triglycerides

Question 363

Risk factors for seizure after cardiopulmonary bypass

Answer 363

Long periods of deep hypothermic circulatory arrest
Younger age
Preexisting CNS pathology

Question 364

What size child has the best correlation between NIRS and true jugular venous saturation?

Answer 364

<10kg

Question 365

Concerning chest tube output

Answer 365

> 10 mL/kg/hr in first hour, >5 mL/kg/hr after two hours

Question 366

Clinical signs of NEC

Answer 366

Thrombocytopenia, temperature instability, bradycardia, lethargy, apnea

Normal lactate doesn’t rule out NEC
Watery stool more indicative of malabsorption

Question 367

Aortic valve prolapse occurs in which types of VSD?

Answer 367

Perimembranous and outlet

Question 368

Natural history of VSDs include spontaneous closure in how many?

Answer 368

75-80%

Question 369

Malalignment VSD has malalignment of what structures?

Answer 369

Outlet septum and muscular septum (outlet septum is posteriorly - interrupted arch - or anteriorly - TOF - deviated)

Question 370

Most common heart defect in T21

Answer 370

VSD
PDA

Question 371

Heart disease in Ellis-van Creveld

Answer 371

Common atrium / AVSD

Question 372

Rastelli Classification

Answer 372

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

Question 373

Types of AP window

Answer 373

Type I - proximal
Type II - distal
Type III - complete

Question 374

Diastolic flow reversal in abdominal aorta and left heart dilation

Answer 374

Look for AP window

Question 375

Berry Syndrome

Answer 375

Interrupted aortic arch Type A and AP window

Question 376

Diastolic flow reversal in arch, dilated SVC, high output heart failure

Answer 376

Extracardiac AVM (L-R shunt)

Question 377

Most common cause of extensive pulmonary AVMs

Answer 377

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)

Question 378

Indications for fetal echo referrals ranked by frequency

Answer 378

• Suspicion of CHD on OB US (CHD confirmed in 40-50%)
• Fetal rhythm abnormality <10% of referrals

Question 379

Retinoic acid associated with what cardiac defects?

Answer 379

Conotruncal defects, aortic arch anomalies

Question 380

Normal fetal cardiothoracic ratio

Answer 380

25-35%

Question 381

At term, what percent of fetal cardiac output perfuses the lungs?

Answer 381

10%

Question 382

Retrograde flow in aortic arch is seen in what types of fetal echo?

Answer 382

Left sided obstructive lesions (e.g. critical aortic stenosis)

Question 383

Teratogens and associated fetal diagnoses

Answer 383

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

Question 384

Most common cause of fetal complete heart block

Answer 384

Maternal SSA/SSB antibodies

Other causes: long QT, ccTGA, heterotaxy

Question 385

Incidence of CHD in maternal diabetes and family history of CHD

Answer 385

Maternal diabetes: 4-10% (dTGA, truncus, TOF)
Family history: 2-4%

Question 386

When is maternal hyperoxia test performed?

Answer 386

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

Question 387

Effect of bilirubin on NIRS

Answer 387

Bilirubin absorbs light at wavelength similar to that emitted by NIRS - falsely low reading

Question 388

Effect of air bubbles in blood gas

Answer 388

pO2 falsely high, pCO2 falsely low

Question 389

First line therapy for heart disease in Duchenne

Answer 389

ACEi

May be beneficial to start even before decline in function (fibrosis on MRI) - start when HF symptoms OR imaging shows abnormalities

Question 390

Williams syndrome

Answer 390

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

Question 391

Risk stratification for WPW

Answer 391

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

Question 392

ACHD indications for ASD closure

Answer 392

Right heart enlargement, no PH
- grade 2A to close if asymptomatic
- grade 1 to close if symptomatic

Question 393

ACHD indications for pulmonary valve replacement in TOF

Answer 393

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

Question 394

What is histological finding in pulmonary vein stenosis?

Answer 394

Fibromyxoid intimal proliferation in the VEINS
Muscularization in the pulmonary ARTERIOLES +/- arterial intimal fibrosis

Question 395

Echo features of double chamber RV

Answer 395

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

Question 396

Criteria for LVNC

Answer 396

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)

Question 397

Most common complication after Glenn?

Answer 397

Phrenic nerve paralysis (4.7%)

Question 398

How will fetal PVC appear on inflow/outflow Doppler?

Answer 398

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

Question 399

Management of fetal PVCs

Answer 399

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

Question 400

Technical performance score

Answer 400

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

Question 401

Bundle branch block and SVT

Answer 401

If cycle length shortens with resolution of bundle branch block, accessory pathway USES that bundle branch - ipsilateral side

Question 402

NOTCH1 and SMAD6 associated with what lesions?

Answer 402

Bicuspid aortic valve, aortic stenosis, and coarctation of the aorta

Question 403

Jag1 and GATA6 associated with what lesion?

Answer 403

Tetralogy of Fallot

Question 404

What genes are associated with TAPVR?

Answer 404

Ankyrin repeat domain
Platelet-derived growth factor receptor alpha

Question 405

PA/IVS repair

Answer 405

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%)

Question 406

DILV where is aorta and conduction system?

Answer 406

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

Question 407

Tricuspid atresia with transposed great arteries risk factor for late obstruction

Answer 407

bulboventricular foramen < 2cm2/m2

Question 408

SVR Trial

Answer 408

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

Question 409

Enalapril in infants with single ventricle

Answer 409

Negative trial for enalapril in single ventricle patients

Question 410

Normal Fontan pressure and transpulmonary gradient?

Answer 410

<15 mmHg
Transpulmonary gradient <10 mmHg (normal 5-8 mmHg)

Question 411

Five year survival for Fontan with PLE?

Answer 411

88%

Question 412

Treatments for PLE?

Answer 412

Budesonide (inflammation of GI tract)
Low fat/high protein diet
MCT
Octreotide
Zinc

Question 413

Differential diagnosis for end tidal CO2 to PaCO2 gradient (>5 mmHg)

Answer 413

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)

Question 414

Management of PH crisis

Answer 414

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)

Question 415

Epidemiology of bicuspid aortic valve

Answer 415

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

Question 416

Most important variable for accepting or rejecting donor heart in predicting post-transplant outcome?

Answer 416

Ejection fraction

Question 417

Treatment for post-pericardiotomy syndrome

Answer 417

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)

Question 418

Progression of V1 over time

Answer 418

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

Question 419

Mutations in hamartin and tuberin cause what?

Answer 419

Tuberous sclerosis (hamartin = TSC1, tuberin = TSC2)

Can use sirolimus/everolimus (mTOR inhibitors) for treatment

Require long term monitoring even without tumors (arrhythmia)

Question 420

HRAS mutation

Answer 420

Costello syndrome
Arrhythmias and hypertrophic cardiomyopathy

Question 421

Abnormal coagulation studies in a Fontan patient not on Warfarin are most likely due to what?

Answer 421

Fontan associated liver disease

Question 422

Criteria for Kawasaki Disease

Answer 422

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)

Question 423

Familial supravalvar aortic stenosis

Answer 423

• 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)

Question 424

Anticoagulation for mechanical valves in pregnancy

Answer 424

• 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

Question 425

What is Ashman phenomenon?

Answer 425

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

Question 426

Anticoagulation recommendations for mechanical valve

Answer 426

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

Question 427

Most likely diagnosis for irregularly irregular wide complex tachycardia in otherwise healthy patient?

Answer 427

Pre-excited atrial fibrillation

*Don’t want to block AV node - adenosine, digoxin, diltiazem, beta blocker

Rx: cardioversion or amiodarone

Question 428

Palivizumab (Synagis) prophylaxis criteria

Answer 428

<= 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

Question 429

Treatment of pericarditis

Answer 429

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

Question 430

Treatment of refractory KD

Answer 430

Persistence or recurrence of fever 36h after one dose of IVIG:
- Second dose 2 g/kg
- Methylprednisolone 20-30 mg/kg IV x3d

Question 431

Treatment of acute rheumatic fever

Answer 431

Mild to moderate carditis: aspirin 80-100 mg/kg/d in four divided doses

Severe carditis: Add prednisone

Question 432

Immunosuppressive agents

Answer 432

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)

Question 433

Duration of antibiotic prophylaxis after acute rheumatic fever?

Answer 433

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

Question 434

Lidocaine toxicity

Answer 434

CNS - tremors, lightheadedness, ataxia, dysarthria, mood/personality changes, hallucinations, seizures

Question 435

Most common side effect from rabbit ATG?

Answer 435

Fever
Shaking/chills

Question 436

Phenytoin as an antiarrhythmic is like what other medications?

Answer 436

Lidocaine and mexiletine
Class IB sodium channel blockers
QTc can be slightly shortened

Question 437

Medications for atrial flutter

Answer 437

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

Question 438

Sotalol clearance

Answer 438

Renal - should be dose adjusted in severe renal dysfunction

Question 439

Atropine vs. isoproterenol

Answer 439

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

Question 440

Losartan has what impact on TGF-B

Answer 440

Angiotensin type 1 receptors increases expression of TGF-B ligands … losartan blocks this pathway

Question 441

Amiodarone drug interactions

Answer 441

Inhibits cytochrome P450, increases levels of:
cyclosporine
digoxin
warfarin

Question 442

Amiodarone mechanism of action

Answer 442

Mostly potassium channel blockade
Also:
- Sodium channel and Ca channel blockade
- Beta blockade
- Decreases AV node conduction

Hypokalemia exacerbates pro-arrhythmic potential

Question 443

Mechanism of heparin-induced thrombocytopenia with thrombosis (HITT)

Answer 443

Heparin + platelet factor 4 – makes immunogenic complex

Antibodies result in formation of platelet aggregates which cause vaso-occlusion and immune-mediated platelet destruction

Question 444

Differences between beta blockers

Answer 444

Nonselective: Nadolol, Propranolol, Sotalol
Selective: Atenolol, Metoprolol (less risk of bronchospasm)

Metoprolol more lipid soluble than atenolol so can cross blood-brain barrier

Question 445

Location of action for antiarrhythmics

Answer 445

Procainamide - atrial myocardium, His-Purkinje, ventricular myocardium (no effect on SA or AV node)

Diltiazem - slow AV conduction

Lidocaine/mexilitine - ventricular muscle

Question 446

Best antiarrhythmic for IART in ACHD patient with ventricular dysfunction who failed ablation attempt

Answer 446

Amiodarone or dofetilide

Question 447

Dofetilide mechanism of action

Answer 447

Class III antiarrhythmic, inhibits rapid component of delayed rectifier potassium current - can prolong QTc

May have to dose adjust for renal disfunction

Question 448

Clinical trial phases

Answer 448

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

Question 449

Efficacy/potency of a drug

Answer 449

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

Question 450

How many half lives to steady state?

Answer 450

Four to five

Question 451

Irritability and tremulousness are side effects of what immunosuppressive medication?

Answer 451

Tacrolimus

Question 452

Side effects of immunosuppressants

Answer 452

Tacrolimus - tremulousness, seizure
Azathioprine, MMF - leukopenia, GI upset
Sirolimus - diarrhea, mouth sores
Prednisone - mood changes, appetite, hyperglycemia, weight gain, Cushingoid appearance, osteoporosis

Question 453

Potts shunt vs. atrial septectomy

Answer 453

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

Question 454

Interaction of calcineurin inhibitors and antifungal medications

Answer 454

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)

Question 455

Risk factors for sudden cardiac death in dilated cardiomyopathy

Answer 455

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

Question 456

Stages of rejection

Answer 456

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

Question 457

Inheritance pattern of most familial dilated cardiomyopathy?

Answer 457

Autosomal dominant

Question 458

Approach for EBV mismatch (donor +, recipient -) in transplant to decrease risk of PTLD?

Answer 458

Safely minimize immunosuppression

Question 459

Treatment for transplant associated coronary vasculopathy

Answer 459

Aspirin
Statin
Switch calcineurin inhibitor to mTOR inhibitor (sirolimus)
Re-transplant

Question 460

Mechanism of action of amiloride

Answer 460

Like spironolactone: blocks Na resorption in distal convoluting tubule, decreasing excretion of K and H

Question 461

Side effects of PH therapies

Answer 461

Sildenafil: vision changes
Treprostinil: urinary retention, thrombocytopenia
Bosentan: hepatic dysfunction, lower extremity edema, anemia (other ERAs too)

Question 462

Cat Eye Syndrome

Answer 462

Tetrasomy 22p
Rectoanal anomalies, coloboma, genitourinary anomalies, preauricular pits/tags

Question 463

Risk of CHD based on indication for fetal echo

Answer 463

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

Question 464

Treatment for pseudoaneurysm post-cath

Answer 464

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

Question 465

Factors to precipitate early lipid screening (age 2-8 years) or 12-16 years

Answer 465

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

Question 466

What is universal lipid screening age 9-11?

Answer 466

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

Question 467

Acetazolamide

Answer 467

Blocks activity of carbonic anhydrase

Acetazolamide effect is reduce alkalosis and increase NaCl, (HCO3- out, keep Cl-), increases effectiveness of the other diuretics

Question 468

Metolazone

Answer 468

Thiazide diuretic, blocks NaCl reabsorption in distal convoluted tubule

Question 469

PDA closure criteria

Answer 469

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

Question 470

LVOTO in AVSD more common in what scenario

Answer 470

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

Question 471

S2 with VSD

Answer 471

Usually normal
Some patients may have wide splitting of S2

Question 472

Process of closure of the ductus arteriosus

Answer 472

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

Question 473

Indomethicin vs. ibuprofen for PDA

Answer 473

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

Question 474

Symptoms of sinus of Valsalva

Answer 474

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

Question 475

Outcomes of embolization for pulmonary AVMs

Answer 475

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

Question 476

Distinguish between large PDA and AVM in neonate

Answer 476

Systemic venous saturation

Question 477

Kawashima

Answer 477

Glenn but with interrupted IVC with azygous continuation so ends up being close to Fontan

EXCEPT hepatic drainage still bypasses lungs so get AVMs

Question 478

Unilateral pulmonary artery stenosis

Answer 478

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%

Question 479

What percent of patients with PA/IVS have RV-dependent coronary circulation?

Answer 479

<10%

(45% have ventriculo-coronary connections but aren’t RV dependent)

Question 480

Goal of TOF/PA/MAPCAS repair

Answer 480

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

Question 481

Location of bundle of His in TOF/PA/MAPCAS

Answer 481

Normal sinus node, normal AV node
Bundle of His lies along the left ventricular aspect of the posteroinferior rim of the VSD

Question 482

Waterston vs. Potts shunt

Answer 482

Waterston - ascending aorta to RPA
Potts - descending aorta to LPA

Question 483

Continuous murmur more common in TOF/PA/MAPCAS or truncus?

Answer 483

TOF/PA/MAPCAS

Question 484

Difference between isolated mitral valve cleft and cleft seen in AVSD

Answer 484

Isolated: directed anteriorly toward outflow septum/LVOT
AVSD: posteriorly directed toward inlet septum

Question 485

Criteria for single- or bi-V repair with LV hypoplasia

Answer 485

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?

Question 486

Sports participation for aortic stenosis

Answer 486

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

Question 487

When to operate on coarctation in asymptomatic child without severe upper extremity hypertension?

Answer 487

Age 2-3 years - less risk of late recurrence compared with surgery before age 1

Question 488

What causes exercise induced upper extremity hypertension following coarctation repair?

Answer 488

Increased flow across relatively non-distensible coarctation repair site, can use beta blocker

Question 489

ccTGA ECG

Answer 489

Q waves present in right precordial leads, III, aVF, absent in left
Left axis deviation

Question 490

Best surgery for DORV with subpulmonary VSD

Answer 490

Arterial switch (transposition physiology)
With pulmonary stenosis: REV, Rastelli, or Nikaidoh

Question 491

Pericardial defects

Answer 491

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

Question 492

Follow up schedule for family history HCM

Answer 492

Without ECG findings or significant hypertrophy, Q12-18mo until 21 years
Then Q5y

Question 493

Loffler endocarditis (hypereosinophilic syndrome)

Answer 493

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

Question 494

ECG in Duchenne

Answer 494

Deep Q waves in I, aVL, V5, V6
PR may be short
Resting sinus tach, loss of circadian rhythm, reduced HR variability

Question 495

Most common arrhythmia in pediatric restrictive cardiomyopathy?

Answer 495

Atrial flutter
Next most common: high-grade second degree AV block, third degree block

Question 496

Indications for closure of asymptomatic perimembranous VSD

Answer 496

• Progressive aortic regurgitation
• RV muscle bundle hypertrophy (DCRV)
• Bacterial endocarditis

Question 497

Hemodynamic measures predicting success after Glenn

Answer 497

LVEDP <12 mmHg
TPG <10 mmHg
mPAP <16 mmHg

Question 498

Late formation of aneurysm is associated with which technique for aortic coarctation repair?

Answer 498

Prosthetic patch

Question 499

What is a hemi-Fontan?

Answer 499

Glenn without disconnecting SVC from RA
Advantage: later expeditious Fontan, but unlike Glenn it does require bypass

Question 500

Goal for training LV in planned anatomic repair of ccTGA

Answer 500

70-80% systemic pressure, repair within first 2-3 years of life

Question 501

“Circle Sign” on echo (small circle anterior to aortic valve in parasternal long)

Answer 501

High, anterior, leftward origin of RCA
Anomalous origin of RCA from left or LCA from right

Question 502

Whale’s tail sign suggests what

Answer 502

TAPVR to coronary sinus

Question 503

Risk of congenital heart disease in situs inversus totalis?

Answer 503

<1%, same as general population

Question 504

What is Carney complex?

Answer 504

Atrial myxomas
Spotty pigmentation
Endocrine over-reactivity
Autosomal dominant (PRKAR1A), 70% familial

Question 505

Gorlin syndrome is associated with what cardiac tumor?

Answer 505

Fibroma

Question 506

Classic x-rays:
Figure of 3
Egg on a string
Boot shaped heart
Basketball
Snowman

Answer 506

Figure of 3 - coarctation
Egg on a string - dTGA
Boot shaped heart - TOF
Basketball - Ebstein
Snowman - supracardiac TAPVR

Question 507

Chiari network is remnant of what?

Answer 507

Incomplete embryonic absorption of thebesian valve

Question 508

Frequency of echo in KD

Answer 508

Repeat twice/week until coronary dimensions have stopped progressing

Question 509

Early vs. late Lyme disease

Answer 509

Early:
Erythema migrans
Facial palsy
1st degree AV block
Conjunctivitis

Late:
Large joint arthritis

Question 510

What is vasculitis associated with HIV?

Answer 510

Vasculitis of aorta and pulmonary arteries that can lead to diffuse stenosis

Question 511

MISC first line treatment

Answer 511

IVIG, methylprednisolone

Question 512

Describe neonatal enterovirus

Answer 512

Common cause of acquired neonatal myocarditis and encephalitis
Family history of diarrheal type illness
Includes coxsackievirus
*IVIG commonly used though limited supportive data

Question 513

Most common cause of acute purulent pericarditis

Answer 513

Staph aureus

Also: Strep pneumo
Tuberculosis in developing world
Empiric treatment: vanc and ceftriaxone, should consider cefepime for psuedomonas in postsurgical

Question 514

Causes of HIV-related dilated cardiomyopathy

Answer 514

Zidovudine (AZT) associated with early DCM in teens and young adults

Question 515

Congenital Rubella

Answer 515

Cataracts
Microcephaly
Blueberry muffin rash (petechiae or purpura)
Hepatosplenomegaly
Pulmonary stenosis / PDA

Question 516

Tuberculous pericarditis

Answer 516

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

Question 517

Chagas disease

Answer 517

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

Question 518

Jervell and Lange-Nielsen Syndrome

Answer 518

Biallelic mutations in KCNQ1 (autosomal recessive)
Hearing loss and long QT

Question 519

Features of cri-du-chat syndrome

Answer 519

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

Question 520

ASD and conduction abnormality WITHOUT limb anomalies has mutation. inwhat?

Answer 520

NKX2.5

Question 521

Most common genetic cause of familial dilated cardiomyopathy

Answer 521

LMNA mutation

Cardiac conduction disease, dilated cardiomyopathy

Question 522

Features of Ellis-van Creveld syndrome

Answer 522

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

Question 523

Jacobsen syndrome

Answer 523

Wide-spaced eyes, ptosis, small ears, short stature, thrombocytopenia

VSD, left-sided lesions involving mitral and aortic valve
11q23 deletion

Question 524

Percent that have CHD:
Trisomy 18
22q11
Trisomy 21
Turner
5p-

Answer 524

Trisomy 18: 95%
22q11: 80%
Trisomy 21: 40%
Turner: 25%
5p-: 20%

Question 525

What percent of patients with interrupted aortic arch type B have 22q11 deletion?

Answer 525

Half

Question 526

Genetic testing recommendations in coarctation

Answer 526

Every female should have a karyotype

Question 527

Morphology of HCM with highest yield on genetic testing

Answer 527

Reverse curve

Question 528

Marfan-like features with intellectual disability

Answer 528

Homocysteinuria (autosomal recessive), CBS gene
Recurrent thromboembolism, lens dislocation is downward

Question 529

Types of tests for each type of data

Answer 529

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

Question 530

What test is used to analyze difference between Kaplan-Meier curves?

Answer 530

Log-rank test

Question 531

What happens to PPV and NPV as prevalence increases?

Answer 531

PPV increases
NPV decreases

Question 532

What is a type III error?

Answer 532

Produces the right answer to the wrong question

Question 533

What is the p-value?

Answer 533

Probability that an observed difference occurred only by chance

Question 534

What factors increase the power of a study?

Answer 534

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

Question 535

Cost-effectiveness, utility, etc

Answer 535

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)

Question 536

Advantage/disadvantage of meta analysis

Answer 536

Advantage: refinement and reduction, efficiency, generalizability and consistency, reliability, power, precision

Disadvantage: publication bias, clinical heterogeneity, quality differences, lack of independence of study subjects

Question 537

What is the standard error of the mean?

Answer 537

SEM = SD / sqrt(n)
How close the sample mean is likely to be to the population mean

Question 538

accurate, precise, reliable, valid

Answer 538

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?

Question 539

What is degrees of freedom (chi square)

Answer 539

Number of independent comparisons that can be made between members of the sample, used with X2 to calculate p-value

Question 540

Correlation coefficients

Answer 540

-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)

Question 541

Types of bias

Answer 541

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

Question 542

Three principles of ethics from Belmont report

Answer 542

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)

Question 543

How do you calculate sample size of study?

Answer 543

Power (usually 0.8)
Significance level (usually 0.01 or 0.05)
Variability of observations (SD)
Smallest effect of interest (standardized difference)

Question 544

Levels of evidence by US Preventive Services Task Force

Answer 544

A - good (benefits&raquo_space; risk)
B - fair (benefits > risk)
C - fair (benefit = risk)
D - fair (risk > benefit)
I - lacking, poor quality, conflicting

Question 545

Define “censored” in survival analysis

Answer 545

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

Question 546

What test to evaluate agreement between two groups when there’s no gold standard?

Answer 546

Kappa statistic
-1: negative association
1: positive association
0: no association

Question 547

When is odds ratio a good approximation of relative risk?

Answer 547

When probability of event of interest is small

Question 548

When do you use Cochran-Mantel-Haenszel test?

Answer 548

Association between dichotomous exposure and dichotomous outcome accounting for confounder variables (think chi-square adjusted)

Question 549

Systemic to PA shunt with highest risk of developing PHTN?

Answer 549

Potts/Waterston (can’t regulate flow)
Central and BTTS more resistance

Question 550

Biggest risk factor for left AVV regurg after AVSD repair?

Answer 550

Severe preop AVV regurgitation

Question 551

Nakata index

Answer 551

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

Question 552

Dyspnea/cyanosis on standing

Answer 552

Pulmonary AVMs (most occur at basal region of lung)

Question 553

Conduction risk with subaortic membrane resection

Answer 553

LBBB

Question 554

Left heart distention after bypass

Answer 554

Suggests ongoing pulmonary venous return to LA implying systemic to pulmonary shunt - AP collaterals

Could also be bad AI

Question 555

Most common late complication in Scimitar syndrome?

Answer 555

Pulmonary venous obstruction

Question 556

How would retrograde coarctation present in hybrid?

Answer 556

Poor coronary perfusion - dysfunction, ST segment changes, irritability

Question 557

Surgical palliation of TOF absent valve

Answer 557

LeCompte and PA plication

Question 558

When to consider surgical ASD closure over cath lab

Answer 558

Deficiency of posterior-inferior rim
Other abnormalities that would benefit from surgical repair (tricuspid regurg)

Question 559

What is the only surgical intervention for Ebstein anomaly that shows improvement in functional capacity?

Answer 559

ASD/PFO closure

Question 560

Most common indication for re-operation after Ross

Answer 560

Pulmonary homograft failure

Question 561

High filling pressure with normal function and no effusion

Answer 561

Re-open the chest

Question 562

ACHD criteria for pulmonary valve replacement in TOF

Answer 562

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)

Question 563

Risk factors for sudden death in adult TOF

Answer 563

-LV dysfunction
-Nonsustained VT
-QRS >180
-Extensive RV fibrosis by CMR

Question 564

Indications for surgery in severe AI

Answer 564

LVESV >50 mm (>25 mm/m2)
LV dysfunction (EF<55%)

Question 565

Indications for surgery in bicuspid valve with ascending aorta dilation

Answer 565

> 55 mm without risk factors
50 mm with risk factors (family history, rapid growth)

Question 566

Sotalol leads to more QTc prolongation (higher risk) with what electrolyte abnormalities?

Answer 566

Hypokalemia
Hypomagnasemia

Question 567

Earliest sign of cirrhosis in Fontan?

Answer 567

Thrombocytopenia

Question 568

Marker of hepatocellular cardinoma in Fontan?

Answer 568

Alpha-fetoprotein level

Question 569

LMWH vs. UFH

Answer 569

LMWH = enoxaparin = Lovenox (subQ)
UFH = heparin (IV infusion or subQ)

Question 570

Why is iron deficiency a concern for patients with Eisenmenger?

Answer 570

Microcytosis in patients with secondary erythrocytosis increases risk of thromboembolic events (stroke)

Question 571

Patients with Senning/Mustard should have what evaluation prior to transvenous pacemaker placement?

Answer 571

Cath to assess for baffle leak or stenosis that would complicate lead placement or put patient at risk for paradoxical embolus

Question 572

Evidence for medical therapy to treat failing systemic RV? (e.g. Mustard/Senning dTGA)

Answer 572

No evidence

Question 573

Hepatitis C

Answer 573

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

Question 574

Serum BNP >140 correlated with poor long-term outcome in patients with what disorder

Answer 574

Eisenmenger syndrome

Question 575

Familial ASD

Answer 575

Holt Oram
NKX 2.5
GATA IV

Question 576

List the 10 high risk pregnancy features from CARPREG II

Answer 576

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

Question 577

ACE in pregnancy

Answer 577

ACE and ARB contraindicated (early teratogen, late renal dysfunction)

Replace with hydralazine and nitrate

Question 578

Treatment for Loeys Dietz

Answer 578

ARB first line (effect on TGFB signaling)

Question 579

Aortic stenosis in pregnancy

Answer 579

Calcified valve - can’t do balloon or percutaneous valve

Surgery feasible

Question 580

What is platypnea-orthodeoxia syndrome?

Answer 580

Hypoxemia and dyspnea with standing from supine

R-L shunting at PFO exacerbated by change in septal position with upright position

Question 581

Cardiac considerations with antidepressants

Answer 581

Sertraline - low risk
Citalopram/amitriptyline - long QT
Venlafaxine and bupropion - norepi increase

Question 582

Dabigatran use

Answer 582

Oral direct thrombin inhibitor
Approved for atrial fibrillation, not for mechanical valves

Question 583

Simvastatin has drug-drug interaction with what antihypertensive?

Answer 583

Amlodipine increases risk of myopathy

Question 584

Atenolol and pregnancy

Answer 584

Class D - positive evidence of fetal risk (a study demonstrated lower birth weight infants)

Question 585

Who needs pre-op coronary angiography?

Answer 585

Men >=35years
Women >=35years with coronary risk factors
Women after menopause

Question 586

Adult indications for high-intensity statin

Answer 586

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

Question 587

ICD placement in patient with cyanosis with intracardiac shunt should be what type?

Answer 587

Epicardial (increased risk of thromboemboli if intravenous placed)

Question 588

What 3 categories of ACHD patients should be referred to ACHD center for non-cardiac surgery?

Answer 588

1. Complex or cyanotic CHD
2. PAH
3. Malignant arrhythmia