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cardiology testing 123 > Board Review Mix > Flashcards

Board Review Mix Flashcards

1
Q

MC 2 gene HCM

A

genes are for sarcomeric proteins (thin and thick)
MYH7 (myosin heavy chain) MY Heavy Sevey
MYBPC 3: My Blood Pressure Contractility! 3
3+7=10

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2
Q

MYH7

A

MC gene HCM (myosin heavy chain) MY Heavy Sevey

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3
Q

MC cause SCD athletes adolescent

A

HCM (about half)

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4
Q

indications ICD placement in HCM (6)

A

2+ of the following:
septum > 3cm, prior SCD event (VT/VF), unexplained syncope, NSVT, abnl BP response exercise (<20mmHg), FamHx SCD in 1st deg relative

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5
Q

septal thickness to Dx HCM

A

> 15mm = 1.6 cm (think 16mm)

OR >13 mm with appropriate family history

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6
Q

traditional indexed PVR cutoff for HTx?

A

PVRI<6

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7
Q

doxyrubicin max dose

A

300 mg/m2

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8
Q

thiazide diuretic side effects that separate it from loops

A

hyperglycemia, hyperlipidemia

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9
Q

digoxin drug-drug interaction

A

amiodarone (less common beta blocker)

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10
Q

amio drug drug interaction

A

digoxin

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11
Q

ACE cough cause?

A

blocks breakdown of bradykinin

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12
Q

types of calcineurin inhibitors

A

tacrolimus and cyclosporin

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13
Q

immunosuppresant antimetabolite

A 
cellcept = mycophenolate (inhibits purine synthesis in lymphocytes)
imuran = azothioprine (same, is precursor to 6-MP)
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14
Q

side effect ATG

A

thrombocytopenia (also fever chills)

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15
Q

side effects tacro

A

nephro, HKalemia, Sz, glucose intolerance, l’il more PTLD

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16
Q

side effects cyclosporine

A

gum overgrowth, hair growth, little more rejection

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17
Q

typical Immunesupprasant combo

A

calcineurin and cellcept (tacro and cellcept)

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18
Q

MOA sirolimus

A

mTOR inhibitor, reduces T and B sensitivity to IL-2. Seriously Inhbits the TORO by putting limes on its 2 horns (used with renal/rejection, coronary

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19
Q

side effect cellcept

A

also with imuran GI distress and leukopenia (which is the whole point)

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20
Q

investigator intentionally misleads participants

A

deception (acceptible under only specific instances)

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21
Q

investigator witholds information about some aspect of research

A

incomplete disclosure (acceptible under only specific instances)

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22
Q

SFI (significatn Financial Interest) (4)

A

Renumeration >$5000 from an entity, holds equity interest, IP rights, reimbursement

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23
Q

making up data

A

fabrication

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24
Q

manipulate research process to get

A

falsification

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25
Q

using someone elses ideas/words

A

plagerism

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26
Q

investigator makes mistake because he is human

A

honest error (not research misconduct)

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27
Q

dispute about elements of study, stats

A

difference of opinion (not research misconduct)

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28
Q

belmont report written why and why?

A

1974, tuskegee syphilis study

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29
Q

3 principles of belmont report

A

respect for persons, beneficence justice

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30
Q

what is clinical equipoise

A

needed for a randomized study to continue. may only continue if uncertain that one therapy is better than another

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31
Q

uncertainty whether one therapy is better than another

A

clinical equipoise

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32
Q

research subject does not understand purpose of research

A

therapeutic misconception

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33
Q

threat to get subject to enroll in a study

A

coersion

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34
Q

when does a child become an adult?

A

depends on the state, check local law

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35
Q

when can you do a study involving study with greater than minimal risk?

A

1)direct benefit or 2) generalizable knowledge

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36
Q

cardiac crescent is from what turns into what

A

mesoderm, turns in pprimary and 2nd hear tube

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37
Q

primary heart tube is from what, turns into what

A

from cardiac cresent, turns into atrium and embryonic ventricle

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38
Q

atrial appendage forms from

A

primative atrium, from primary heart field, from cardiac crescent, from mesoderm

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39
Q

LV forms from

A

embryonic ventricle, from primary heart field, from cardiac crescent (mesoderm)

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40
Q

bulbus comes from

A

secondary heart field, from cardiac crescent

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41
Q

bulus cordis turns into

A

RV, conus, outflow tract

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42
Q

RV comes from

A

proxmal bulbus cordis, from seconadry hear tfield, from cardiac crescent

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43
Q

conus come from

A

mid bulbus cordis, from seconadry hear tfield, from cardiac crescent

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44
Q

truncoartic sac comes from

A

distal bulbus, from seconadry heart field,

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45
Q

outflow tract derived from

A

bulbus cordis AND neural crest cells

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46
Q

cardiac crescent and heart tube derived from

A

creschent, mesoderm ; heart tube endo and meso

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47
Q

time of V looping

A

d22-27 (think of a milenial “figuring themselves out” in mid-20s

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48
Q

cardiac timeline in terms of growing up

A

16 days crescent (driving a car), 19d tube (getting out of town), 22d beating (providing for themselvesafter colelge), 22-27d looping (figuring themselves out), 25d atria cephalad (head becomes centered), bulbus midline and indents (straightening up)

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49
Q

time of V septum closure and outflow tract closure

A

VSD 6 weeks (6 looks like a VSD), outflow 6-8 (8 looks like a PA and aorta divided)

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50
Q

straddlign of AVV through which VSD

A

TV through inlet VSD, MV through malaligned VSD

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51
Q

which arch becomes innominate artery

A

contralateral 4th

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52
Q

association RAA with ab left SC artery?

A

22q11

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53
Q

normal arch, what involutes

A

right dorsal aortic arch, right 6th

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54
Q

RAA arch w/ mirror, what involutes

A

left dorsal aortic arch, right 6th (left 6th usual stay around, but is not a ring)

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55
Q

findings of left atrium

A

CS bosy, pect muscles stay in appendage, thin primary septum on the left side

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56
Q

findings of the RA

A

pect muscles go to free wall, CS os, Limbus, thick septum secundum

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57
Q

RAA with ab L subclav, what involutes?

A

L 4th involutes, if left ducuts, ring

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58
Q

MC rings

A

doubale Ao arch, RAA with ab L subclavian

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59
Q

Type A IAA associated with?

A

AP window

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60
Q

normal ear sat, low arm and leg sat, Dx?

A

type B IAA with ab R subclavian A, both SC come from ductus, both carotids come from asc aorta

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61
Q

4 weak pulses, low RA and LA sat, normal ear sat

A

type B IAA with ab R subclavian A, both SC come from ductus, both carotids come from asc aorta

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62
Q

type B IAA with ab R subclavian A, sats?

A

carotids normal, subclavians low

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63
Q

anterior indentation on esophagram

A

LPA sling (only to go between trachea and esophagus)

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64
Q

vein embryology: R horn sinus venosus becomes

A

joins into RA

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65
Q

vein embryology: L horn sinus venosus becomes

A

coronary sinus

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66
Q

vein embryology: R cardinal vein becomes

A

SVC (male cardinal flies down)

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67
Q

vein embryology: L cardinal vein becomes

A

involutes, or LSVC to CS (female cardinal redness involvules)

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68
Q

venous structure from abnormal LA egress

A

levoatrial cardinal vein (not PAPVR, this is a decompressing vein from LUPV to innom vein)

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69
Q

Fetal % RV:LV

A

RV:LV 60:40

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70
Q

Fetal % RV flow

A

2/3 goes out ductus to the placenta (low resistance), < 1/3 goes to lungs = 20% total CO

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71
Q

% total fetal CO through isthmus

A

5-10% through isthmus, most LV output goes to the head and neck

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72
Q

left heart obstruction in fetal, what effect

A

low O2 delivery to brain, retrograde ductus

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73
Q

TGA in fetal, what effect

A

low O2 delivery to brain and coronary arteries

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74
Q

fetal myocardium differences

A

immature sarc ret, fewer contractile, dependent on HR, sensitive to change in afterload

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75
Q

metabolic demand change upon birth, cardiac output change

A

3fold inc in metabolism (temp regulation, breathing), CO increases by 50%

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76
Q

effect of cyanosis and increased pulmonary blood flow

A

very delayed drop in pulmonary vascular resistance (TGA/VSD)

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77
Q

highest risk for delayed drop in pulmonary vascular resistance

A

cyanosis with high pulmonary blood flow (TGA/VSD)

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78
Q

electron microscopy: sarcomere

A

z disc to z disc (Zarcomere)

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79
Q

troponin C vs. troponin I

A

C bind calcium, conformational change, I Inhibits by covering actin binding spot

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80
Q

thin vs. thick filament, which protein

A

thin = Actin, thick = myosin (M is thicker than A), myosin pulls the actin when ATP and Ca are available

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81
Q

how does calcium get into the cell

A

voltage gated L type Ca channel, Ca influx activates ryanodine receptor on the Sarc Ret

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82
Q

what pumps calcium away from myosin?

A

SERCA2 in sarcoplasmic reticulum (inhibited by phospholamban during systole LAMB = calm and docile), enhances diastolic function

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83
Q

how does milrinone work?

A

inhibits phosphidiesterase 3, increase cAMP, cAMP inhibits phospholamban, decreased inhibition of SERCA, SERCA more powerfully removes Calcium from the cells during diastole, decreased Ca, decrease myofibril bridging, also puts more Ca in the SR, so the next burst of Ca will be larger

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84
Q

what does Titan do?

A

passive stiffness of sarcomere, binds thin filament to actinin ( Z line)

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85
Q

2 ways that sarcomere is connected to cell membrane?

A

1) dystrophin glycoprotein complex (binds thin filament to ECM 2) titin to actinin (Z line) to Integrin on cell membrane

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86
Q

define length tension relationship

A

passive force generated by stretching a muscle

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87
Q

4 ways to alter PV loop to change Stroke volume

A

preload, diastolic function, afterload, contractility: preload climbs the floor ramp, diastolic drops the floor ramp, afterload descends the roof decline, contractility moves the roof to the left

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88
Q

sympathetic nervous system effect on heart muscle

A

most from circulating catecholamines, adults have some B1 innervation

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89
Q

thyroid effect on heart

A

increase adrenergic receptors, inc mitochondria, inc myocyte proteins

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90
Q

what does endothelin do to pulm vasculature

A

vasoconstricts, of course (endothelin receptor antagonists)

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91
Q

effect of sildenafil, MOA?

A

inhibits PDE-5, decreased NO breakdown, increased cGMP, vasodilation

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92
Q

MOA endothelin

A

ET A and B receptors, potent vasocontriction AND smooth muslce proliferation

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93
Q

MOA prostacyclin

A

prostaglandin, cAMP, vasodilation

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94
Q

mechanism of hypoxic vasoconsitrction

A

mitochondrial sensor: increased oxygen species in electron trsansport chain, K channel opens, decreases voltage gated Ca influx, vasodilation

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95
Q

what do baroreceptors respond to? what do they do?

A

respond to mechanical stretch (pressure), decrease sympathetic output and inc vagal tone, dec HR, vasodilation

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96
Q

4 subdivisions of fetal CO and percentages?

A

30% brain, 10% isthmus, 40% ductus, 20% lungs

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97
Q

which electron microscopy lines of a sarcomere shorten?

A

Z bands and H line shorten, M line is the myosin middle, A band is the myosin thick filament, I band is the actin element of thin feliment bisected by Z band

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98
Q

4 types of AVM causes

A

HHT, Abernathy syndrome, hepatopulmonary Glenn

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99
Q

HHT mutations

A

ACVRL1 (A to V, R to L in 1 sec), ENG (endoglin, ENGineering makes you bleed yours eyes out)

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100
Q

Portal disease, vs. Hepatic disease, what effects on the lungs?

A

portal causes PAH, hepatic disease causes AVMs

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101
Q

how does RA hypertension lead to pulmonary edema?

A

impaired pulm lymphatic drainage

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102
Q

how does pulmonary edema cause weezing?

A

perivascular cuffing of airways by engorged vessels, causes obstructive defecr

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103
Q

asociation of PA sling?

A

complete tracheal rings (50%)

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104
Q

what is FRC?

A

volume of air left in the lungs at the end of tidal volume

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105
Q

what are the 2 main effects of respiration on blood pressure?

A

preload of RV, afterload of LV

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106
Q

effect of normal negative pressure expiration on CO

A

inspiration -> dec pleural pressure -> dec RA pressure, -> inc RV stroke volume -> inc CO

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107
Q

effect of normal negative pressure expiration on CO

A

inspiration -> inc pleural pressure -> inc RA pressure, -> dec RV stroke volume -> dec CO

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108
Q

Equation LV systolic transmural pressure

A

LV systolic transmural pressure = intravascular systolic pressure - pleural pressure, implications during inspiration:
normal ventilation: lower pleural pressure, transmural pressure higher, inc LV afterload, dec LV stroke volume, dec CO
PPV: higher pleural pressure, LV transmural pressure dec, dec LV afterload, inc cardiac output

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109
Q

poiseuille equation

A

R proportional to viscocity * length / radius^4

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110
Q

normal PA - Pa gradient?

A

5mmHg

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111
Q

Alveolar gas equation

A

PAO2 = (Ptm - Po2) *FiO2 - PACO2/RQ

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112
Q

equation for oxygen content

A

CaO2 = SaO2 * Hgb * 1.34 +PaO2 * 0.003

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113
Q

What is a Libman-Sacks lesion?

A

endocarditis lesion in lupus, found on left sided valves MV>Ao Valve, 10% NEW DX LUPUS, IRREGULAR VEGETATIONS

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114
Q

lupus endocarditis

A

Libman-Sacks

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115
Q

L type calcium channels located on?

A

cell membrane (voLtage gated), ryanodine (Ca dependent) on the sarc ret

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116
Q

where and why is renin made?

A

kidney juxtaglomerular apparatus, in response to lowe kidney perfusion pressure

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117
Q

board andswer for pulmonary artery embryonic arch?

A

6th arch

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118
Q

Most common additional cardiac finding in tetralogy of fallot

A

ASD/PFO in 80% (pentalogy of fallot), RAA in 25%

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119
Q

MC coronary abnormality in D-TGA

A

circ off of RCA (16%, circle and circle), followed by single RCA, then inverted RCA and Cx (RCA off LAD, Circ off posterior)

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120
Q

space seen near LCA on echo

A

transverse sinus

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121
Q

where is ANP made and what does it do?

A

both atria in response to stretch. increases GFR by arteriolar glomerular effect, distal tubule sodium reabsorption is blocked

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122
Q

most reliable differentiating factor between MV and TV

A

TV offset apically at hinge point

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123
Q

when do you see left vs. right juxtaposition of atrial apendages?

A

left more common in complex heart lesions with abnormal VA connections, right in simpler lesions

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124
Q

MC great artery position in DORV

A

side-by-side = aorta right, PA left

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125
Q

what is mitral arcade

A

absent/abnormal chordal insertions

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126
Q

why is the second heart sound loud in TGA?

A

anterior aorta

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127
Q

absent pulmonary arteries in TOF vs. truncus?

A

in TOF, the absent PA is opposite the aortic arch. In truncus, the absent PA is on the same side as the arch

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128
Q

blood supply to AL and PM paps

A

AL from LAD and Circ, PM from right. AL is a Cheery round LAD. PaM is always right.

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129
Q

what is the thebesian valve and the vieussens valve?

A

Thebesian valve is located at the mouth of the CS. Vieussens is located where the cardiac vein merges with coronary sinus.

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130
Q

MC coronary abnormality in normal hearts?

A

Circ from the RCA

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131
Q

where do T tubules associate?

A

with the Z disc, continuation of sarcolemma

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132
Q

what is an intercalated disc?

A

junctions between myocytes, link myofibrils to the edge to transmit force from the sarcomere. LINK everything together

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133
Q

when is ATP used during the power stroke?

A

during the power stroke when the myosin bends, release of ADP allows the extension of the myosin head

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134
Q

indications for coarct repair (5)

A
  1. BP gradient > 20, 2. BP gradient <20mmHg with >50% narrowing at coarct level 3. hypertension 4. other cardiac lesions 5. elevated LVEDP
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135
Q

coarct repair from side or front?

A

all about the size of proximal arch, front if proximal arch small: -2 z score, 60% of asc ao, < weight in kg + 1

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136
Q

risk of subclavian flap

A

aneurysm

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137
Q

long term risks after coarct repair?

A

HTN in 30-90%, recoarct in 5-10%

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138
Q

long term complications after IAA repair?

A

LVOTO (30%), recoarct (8-30%)

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139
Q

1st 2nd most common coronary arrangements of D-TGA?

A

first is normal, 2nd is circ off the RCA. (circle and circle in paralell D-TGA)

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140
Q

4 options for TGA/LVOTO

A

1) switch with LVOT resection
2) Rastelli
3) Nikaidoh
4) REV

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141
Q

TGA/LVOTO surgery of choice if pulm valve very small

A

Nikaidoh (remember, risk of kinking CA, homer simpsons is big and walking around saying DOH!

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142
Q

risk of death after ASO, which factors?

A

abnl coronary pattern, multiple VSDs, older age

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143
Q

late complications of arterial switch

A

supravalvar PS, neo-aortic dilation, insufficiency

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144
Q

stage 1 treatment of chois if 1.5 kg

A

hybrid, then sano , then BTTS

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145
Q

10 yr survival after norwood

A

65%

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146
Q

Tri atresia, what are the types?

A 
I - normal (70%)
II - D transpo (30%)
III - L transpo (<5%)
A = atrtic or small PV
B = balanced
C = over Circulated
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147
Q

what is trussler’s rule?

A

how big is the inner diameter of a PA band? 21mm + weight (in kg)

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148
Q

when do people doa Mee shunt?

A

central PA shunt to asc aorta in MAPCAS or small PA

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149
Q

for the boards, best long term survival with which type fo fontan?

A

ECC

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150
Q

location of obstruction in supracardiac TAPVR? infracardiac

A

supra (25-50%):between left PA and L bronchus (vice) > vertical vein insertion
infra (99%): hepatic sinusoids, insertion to IVC, location of diaphragm

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151
Q

MC type of truncal valve (# cusps)

A

tri in 60, then quad

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152
Q

mortality of truncus IAA?

A

up to 50%

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153
Q

indications for AS surgery?

A

critical, asymptomatic >50 mmHg, symptomatic or ischemic ECG changes

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154
Q

which VSD most likely to cause AI?

A

juxta-arterial

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155
Q

location of conduction system in VSDs: perimembranous
inlet
muscular
doubly committed

A

perimembranous: inferior rim
inlet: inferior rim
muscular: less at risk
doubly committed: less at risk, caudal limb septal Y band

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156
Q

how do you handle the AVV in a one-patch repair of the AVSD?

A

incise them

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157
Q

rate of PV replacement for repaired tet at 20 years?

A

30%

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158
Q

taussig bing

A

TGA (TausiG) type DORV, subpulmonary VSD, risk of coarctation

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159
Q

TGA type DORV, subpulmonary VSD, risk of coarctation

A

taussig bing (TausiG) = TGA

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160
Q

gene for williams syndrome

A

elastin (ELN)

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161
Q

surgery for supra-valvar AS

A

Doty Y patch repair (or three patch)

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162
Q

indications for subas repair

A

40mmHg, symptoms, 50% if tunnel-like, or Aortic involvement with AI

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163
Q

what is modified in the modified konno?

A

resect septum and place VSD patch to make more room

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164
Q

cath indication for ASD closure?

A

QpQs 1.5:1, otherwise R dilation, lung diseases, R to L shunt

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165
Q

what determines the type of surgery in PAPVR?

A

height of the RUPV, if closure, do a intracardiac baffle from RUPV orifice to ASD. If high, do a Warden Procedure

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166
Q

which ASD repair has a high risk of sinus node dysfunction?

A

PAPVER repair with 2 patch repair

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167
Q

associated features of scimitar syndrome?

A

basic disease is R PVs go to the IVC/RA junction, associated:
AP collaterals to right lower lobe
right lung hypoplasia
+/- right diaphragmatic hernia

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168
Q

what is dysphagia lusoria?

A

LAA with ab right subclavian

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169
Q

what is innominate artery syndorme and how to you fix it?

A

innominate artery compresses the trachea anteriorly, fix by aortopexy or innominate artery reimplantation

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170
Q

what is the Takeuchi repair?

A

repair of ALCAPA, baffle from coronary OS through the PA to a window between the aorta and the PA

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171
Q

definition of RV-dependent coronary circulation?

A

myo perfusion from RV fistulae from obstruction of 2+ coronary branches OR ostial atresia

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172
Q

MC types of IE organisms

A

staph 43%, strep 40%, then beta hemolitic strep, enterococcus hacek

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173
Q

strep IE associations vs. staph

A

staph is acute, CHD< prosthetic, catheters, newborns (also candida). strep is subacute, non-chd, >1

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174
Q

candida IE

A

newborns

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175
Q

newborn IE orrganisms

A

staph, then candida

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176
Q

which IE organism only needs 1 blood culture?

A

Coxiella (Cox for blood Cx)

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177
Q

Duke criteria

A

2 major: 2+ culture and ECHO positive
5 minor: predisposing (like CHD, IVDU), fever, vascular findings, immonolgic findings, micro (culture that doesn’t meet major criteria)

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178
Q

what are the duke minor vascular findings?

A

arterial emboli, septic emboli, mycotic aneurysm, janeway lesion, conjunctival hemorrhage

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179
Q

what are the duke minor immunologic findings?

A

Roth spots, osler node, glomerulonephritis

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180
Q

HACEK organisms

A

Haemophilus, Aggregatibacter, Cardiomacterium, Eukenella, Kingella

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181
Q

empiric drugs to treat IE

A

beta lactam, aminoglycoside +/- vanc, rifamin if prosthetic valve

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182
Q

adjuvant antibiotic if the patient has a prosthetic valve?

A

rifampin

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183
Q

surgical indicatsion for IE (3 buckets)

A

vegetation reasons, valve reasons, perivalvar things

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184
Q

what are the vegetation reasons for surgical intervention for IE

A

persistent vegetation after embolism, anterior mitral vegetation, 2 emboli in 2 weeks, 3 eboli total

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185
Q

what are the valve reasons for surgical intervention for IE

A

valve regurgitation, flail, or heart failure

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186
Q

what are the perivalvar reasons for surgical intervention in IE

A

valvular dehiscence, heart block, large abscess

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187
Q

which gets IE prophylaxis?

A

prior IE, prosthetic valve, heart transplant with regurgitation, CHD that is cyanotic, 6 months after surgery, residual defect

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188
Q

when do you give IE prophylaxis

A

before dental procedures, before pulm procedures, when working with infected tissue, not before GI/GU surgeries, not for orthodontics

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189
Q

KD predominantly affects kids < __ years old

A

5

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190
Q

risk of KD aneurysm decreases from __ to __ with IVIG

A

25 to 4 %

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191
Q

recurrence rate of KD

A

3%

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192
Q

msot KD happens in which season in the US?

A

spring

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193
Q

pathogenesis of KD and difference between moderate and large aneurysms?

A

necrotizing arteritis of medial layer, small and moderate has vessel damage (may decrease in size after/remodel), large and giant, medial layer is destroyed (does not regress or remodel afterwards)

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194
Q

3 stages of KF pathogenesis

A

acute 2 weeks, neutrophil necrotic, subacute lyumphs months - years, chronic is luminal myofibroblastic proliferation

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195
Q

MC vessels affects in KD

A

LAD, RCA, LMCA (LMCA will almost always have other vessels affected)

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196
Q

Z score cutoffs in KD for vessel dilation

A

nml <2, dilation 2-2.5, mild 2.5-5 moderate 5-10, large >10

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197
Q

myocarditis findings in KD

A

subclinical dysfunction 50%, KD shock (7%)

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198
Q

valvitis in KD

A

MR (25%)

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199
Q

echocardiographic findings to support atypical KD

A

one of 3: LAD or RCA 2.5 or greater, coronary aneurysm, or dysfunction AND MR AND effusion AND LAD/RCA 2-2.5

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200
Q

indication for plavix in KD

A

in subacute disease, moderate dilation or chronic moderate/large dilation

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201
Q

indication for lovenox in KD

A

large/giant aneurysms or rapidly progressing aneurysms

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202
Q

adjucntive anticoagulants in subacte KD?

A

plavix if moderate, lovenox is large+

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203
Q

LMP

A

luminal myoproliferation

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204
Q

cardiac tumor associated with V arrythmia

A

fibroma

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205
Q

PKU in mother at risk for which CHD?

A

left sided obstructive lesions

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206
Q

chromosome in williams syndrome

A

7q11

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207
Q

7q11

A

williams syndrome

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208
Q

chromosome in jacobsen syndrome

A

11q23 (123)

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209
Q

11q23

A

chromosome in jacobsen syndrome (123)

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210
Q

what is LEOPARD syndrome

A 
Lentigenes
ECG conduction defects
Ocular hypertelorism
Pulmonary stenosis
Abnormal Genitals
Retarded growth
Deafness
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211
Q

gene in kabuki syndrome

A

MLL2

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212
Q

MLL2

A

kabuki syndrome

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213
Q

how to differentiate between Marfan and Loez Dietz on the boards?

A

Loez Dietz will have dilation of the extra-aortic vessels

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214
Q

WPW associated with which 2 forms of CHD?

A

1) Ebsteins 20-30%, 2) HCM (10%)

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215
Q

age of rheumatic fevere and rheumatic heart diseas

A

fever 5-15 years, RHD 25-45

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216
Q

what % of acute rheumatic fever gets severe valvulitis with 1 episode?

A

10%

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217
Q

MC clinical manifestation of ARF?

A

carditis in 5-070%, arthritis second, carditis defines as MR or AI

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218
Q

why is ARF a disease of the margins?

A

erythema marginatum, socioeconomic factors affect people at the MARGINS of society, margins of valves are affected by inflammation in acute phase

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219
Q

ARF More common in males/females?

A

females 2:1

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220
Q

what forms on valves in ARF?

A

verrucae of fibrin and cells on the margins of valves

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221
Q

jones criteria for diagnosis

A

Initial: 2 major or 1M 2min
Recurrent: 2 major or 1M 2min, or 3min

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222
Q

Jones criteria major and minor in low risk

A

Major: carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules
Minor: polyarthRALGIA, Fever >38.5, ESR60CRP3, prolonged PR

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223
Q

Jones criteria major and minor differences in high risk vs. low risk

A

in high risk, monoarthrritis is a major as well as polyarthralgia. monoarthralgia is a low risk. Fever 38 instead of 38.5, ESR 30 instead of 60

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224
Q

echocardiographic diagnosis of carditis in ARF?

A

MR: 2 views, 2cm jet, 3 m/sec pansystole
AI: 1cm jet, 2 views, 3m/sec, pandiastole

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225
Q

main difference between carditis in ARF and chronic RHD?

A

carditis = insufficiency, chronic is mitral stenosis and aortic insufficiency

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226
Q

mild MR murmur

A

high pitched, holosystolic at apex

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227
Q

high pitched, holosystolic at apex

A

mild MR murmur

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228
Q

harsh, holosystolic, loud S2, S3, mid-diastolic rumble?

A

severe MR (diastolic from relative MS)

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229
Q

murmur severe MR

A

harsh, holosystolic, loud S2, S3, mid-diastolic rumble?

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230
Q

classic MS murmur

A

loud S1, low pitched rumble, possible loud S2 (from P2 PH)

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231
Q

loud S1, low pitched rumble, possible loud S2 (from P2 PH)

A

classic MS murmur

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232
Q

CXR with curly B lines

A

LA hypertension

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233
Q

Tx ARF

A

high dose ASA, NSAIDs, PCN, treat family members

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234
Q

ARF prophylaxis meds and duration?

A

PCN G monthly, V BID, erythromycin,
no carditis 5 years or until 18
carditis 5 years or until 21, or longer if residual lesions

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235
Q

retinoic acid is associated with which CHD?

A

conotruncal defects (lets pretend it has to do with the movement of neural crest cells)

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236
Q

prevalence of elevated BP and HTN among youth?

A

elevated BP 10%, HTN 3%

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237
Q

rates of HTN with each of these co-morbidities: obesity, diabetes, OSA, CKD, left sided heart disease

A 
obesity: 25%
diabetes 6%
OSA 3-14%
CKD 50%
left sided >50% in coarct
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238
Q

cuff size:

A

get arm circumference: lenght is 80%, width is 40%

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239
Q

easy to remember BP screening cutoffs for 3 y/o, 11 y/o 13 y/o

A

this is the 90th percentile:
3 y/o: 100 mmHg
11 y/o: 110 mmHg
13 y/o: 120 mmHg

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240
Q

criteria to diagnose with HTN?

A

ausculatory BP reading >95%ile at 3 different visits

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241
Q

BP categories

A

1-12years: <90% = normal, 90-95% elevated BP, 95% - 95% +12 = stage I, >95% + 12 = stage II
>13 years: adult cutoffs: 120,130,140

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242
Q

MCC of HTN in age groups?

A 
>6 = essential HTN
<6 = renovascular disease
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243
Q

initial lab work up for HTN?

A

UA, renal, lipids. RENAL ultrasound if <6 or abnormal UA

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244
Q

when do you get an ECHO or EKG for elevated BP?

A

echo when startign drugs to assess for end-organ damage or for etiology. EKG not needed

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245
Q

when do you get a renal ultrasound in HTN?

A

<6 years old, abnromal UA, stage II, diastolic HTN, abnormal renal, or h/o kidney disease

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246
Q

treatment goals for HTN?

A

<13 years: <90 %ile

>13 years: 130/80

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247
Q

first line treatment for HTN:

A

ACE inhibitor, send is thiazide, then calcium channel blockers

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248
Q

walk thru the lipid metabolism

A

chylomicro metabolized by Lipprotein lpase to LDL, LDL receptor on the liver takes it up, or LDL goes to the vessels. HDL takes cholesterol away from vessels

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249
Q

which is the atherogenic apoprotein?

A

ApoB

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250
Q

causal genes for familial Hcholesterolemia?

A

LDL is the main one, then ApoB and PCSK9 (all cause decreased binding to LDL receptor, which causes poor feedback and liver keeps making LDL

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251
Q

range for common Hchol, hetero FH, homog FH?

A

common 130-200, hetero 160-400, homoz >400

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252
Q

clinical findings in hoozygous FH?

A

xanthomas, supra-AS, coronary abnl

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253
Q

what does lipoprotein A do?

A

makes LDL bond in a stickier fashion to vessel walls

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254
Q

range of hypertriglyceridemia in famililal and severe?

A

familil 200-100,severe > 1000, manage with very low fat diet

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255
Q

what is Tangier’s disease?

A

very low HDL with orange tonsils (Think of Tracy Jordan)

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256
Q

when to screen for FH?

A

9-11, or 17-19, age 2 with strong fmaily history or obesity other risk factors may screen earlier

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257
Q

treatment threshold for common Hcholestereol?

A

190 for everyone, 160 if risk factors, 130, if high risk. preceded by 6 months counceling and only if > 10 years

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258
Q

2 tests for screening for familial Hchol?

A

non-HLD = total cholesterol - HDL cholesterol, should be < 145
or fasting lipids

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259
Q

treatment of high trigluycerides

A
  1. lifestyle
  2. fenofibrate
  3. fish oil
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260
Q

treatment for hypercholesterolemia meds?

A
  1. lifestyle
  2. statins
  3. Ezetimibe
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261
Q

expected LDL drop with lifestlye and statins

A

lifestly 10-15%, statins up to 50%

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262
Q

contraindicated medication with statin?

A

gemfibrozil (common hypertriglyceride medication) think of a sharp GEM causing rhabdomyolisis of a static muscle

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263
Q

acute pancreatitis

A

hypertriglyceridemia (Lipoprotein lipase deficiency)

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264
Q

IF all else fails and you can’t remember inheritance, what general rule can you apply?

A

most CHD is autosomal dominant, most cardiomyopathy is autosomal recessive

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265
Q

orofacial cleft, micropthalmia, prominent nasal bridge, Dx?

A

Trisomy 13, associated with post-axial polydactyly, holoprosencephay
Card: VSD, TOF, complex, polyvalvar, Pulmn HTN
(LOC mid-BDRM)

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266
Q

prominent occiput, narrow bifrontal, short palpebral fissues

A

trisomy 18 - chromosomal

Card: polyvalvar disease, VSD< TOF, complex (LOC Mast Bath)

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267
Q

female, short stature, webbed neck

A

Turner 45 XO - chromosomal
left sided lesions, asc ao dilation, PAPVR, prolonged QT
LOC library

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268
Q

INT aortic arch B

A

22q11 microdeletion
Tubular nose, hypoplastic nasal aleae, bulbous nose tip, cleft palate velopharyngeal insufficiency,
Card: IAA-B, truncus, TOF, RAA
LOC downstair workshop

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269
Q

stellate irises

A

Williams-Beuron 7q11.23 microdeletion
Card: SVAS, PVAS, PPS, CoA, areteriopathy
Other: UNique cognitive profile hoarse voice
LOC (donstair gues bed)

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270
Q

coloboma, choanal atresia

A 
CHARGE -single gene
CHD7 = Charred (LOC side of house by grill)
Cooboma
Heart
choanal Atresia
Retarded growth
Genital 
Ear
TOF, DORV, IAA, AVSD, arch, VSD
LOC = side of house
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271
Q

triangular face, Hypertelorism, pointed chin

A 
Aligille - singel gene
JAG1, Notch 2 
branch PAS, TOF< VSD
live bile duct stuff, butterfly vert
LOC living room
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272
Q

pre-axial abnormality

A 
Holt Oram - single gene
TBX5
non-dysmprphic, arm abnormalities
progressive conduction disease
LOC entryway lovated by stairs, conduction disease
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273
Q

downslanting palpebral fissurees, epicanthal folds, ptosis, webbed neck

A

Noonan - single gene (RASopathy)
PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship
short stature, pectus, PS with dysplastic valve, HCM

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274
Q

Noonan’s like with deafness

A

LEOPARD = Noonans with multiple lentigenes (RASopathy)
PTPN11 (90%), dominant negative
lentigenes after puberty
LOC loft

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275
Q

cafe-au-lait, iris lisch nodules

A

NF1 - single gene (RASopathy)
rare cardiac Coarct, PS (2%)
learning diability, cerebral artery aneurysm, hypertension

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276
Q

arched eyebrows, broad thumbs

A

Rubenstein Taybi - single gene CREBBP or microdeletion 16
Cardiac: PDA, septal defects, CoA, PS
LOC stairs going down (creeping down the stairs)

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277
Q

bell shaped ribcage, short, polydactyly

A

Ellis Van Crevald - single gene autosomal recessive
gene EVC, EVC2 (Evacuate!)
Card: Common Atrium, CAVC
Think amish
LOC - common atrium with 2 garage doors, woodworking in the garage, Evacuate in your car!)

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278
Q

dextrocardia, situs inversus totalis

A 
Kartagener = single gene but autosomal recessive
DNAI1, DNAH5 - strands of DNA
nondysmorphic
frequent lung infections
LOC: small bathroom
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279
Q

malar hypoplasia, narrow face, micrognathia

A

Marfan = fibrillin single gene - autosomal dominant
FBN1
Card: aortic dilation, risk of dissection, MV prolapse
has eye findings (ectopia lentis)

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280
Q

Marfans with no eye stuff

A

Loez Dietz = single gene - auto dominant TGFB
TGFB1,2 (the greenest finest beans)
also bifud uvula,
BAV, dil ao root, arterial aneurysm, risk dissection, PDA
no eye involvement
LOC (side of driveway)

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281
Q

marfan with contractures

A

Beals disease = congenital contractural arachnodactyly
single gene FBN2 (fibrillin 2)
crumpled ears, tall, thin, contractures at birth
ao root dilation
no eye involvement
LOC walkway to driveway

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282
Q

bruising and scarring with thin skin

A

vascular ehlers danlos - single gene autodom
COL3A
spontaneour arterial rupture
no mitral valve prolapse or aortic root dilation
LOC bottom of driveway

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283
Q

7q11.23 microdeletion

A

Williams

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284
Q

11q23

A

Jacobsen

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285
Q

16p deletion

A

Rubenstein taybi (or CREBBS single gene) creep down stairs

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286
Q

CHD7

A

CHARGE CHD7 = Charred (LOC side of house by grill)

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287
Q

JAG1

A

Aligille - singel gene

JAG1, Notch 2

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288
Q

Notch 2

A

Aligille - singel gene

JAG1, Notch 2 Jagged 1 alligator with 2 Notched teeth

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289
Q

TBX5

A

Holt Oram - single gene

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290
Q

PTPN11

A

Noonan - single gene (RASopathy)
PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship

OR

LEOPARD (90%) dominant negative

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291
Q

SOS1 (10%)

A

Noonan - single gene (RASopathy)

PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship

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292
Q

NF1

A

Nerufibromatosis (RASopathy)

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293
Q

CREBBP

A

Rubenstein Taybi - single gene CREBBP or microdeletion 16

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294
Q

EVC, EVC2

A

Ellis Van Crevald - single gene autosomal recessive

gene EVC, EVC2 (Evacuate!)

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295
Q

DNAI1, DNAH5

A

Kartagener = single gene but autosomal recessive

DNAI1, DNAH5 - strands of DNA

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296
Q

TGFB1,2

A

Loez Dietz = single gene - auto dominant TGFB

TGFB1,2 (the greenest finest beans)

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297
Q

FBN2

A

Beals disease = congenital contractural arachnodactyly

single gene FBN2 (fibrillin 2)

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298
Q

COL3A

A

vascular ehlers danlos - single gene autodom

COL3A

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299
Q

gene for VACTERL

A

trick! its an association

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300
Q

cardiac rhabdo

A

tuberous sclerosis (auto dominant)
TSC1, TSC2
facial angiofibroma, ungual fibroma, retinal hamartoma,

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301
Q

TSC1, TSC2

A 
tuberous sclerosis (auto dominant)
TSC1, TSC2
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302
Q

atrial myxoma

A

7% familial
heterozygous mutation in PRKAR1A
syndromic form is Carney Syndrome
LOC back porch where we do ParKour! and stuff gets stuck to the screen door like the PFO

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303
Q

PRKAR1A

A

atrial myxoma (PARKOUR on the back PORCH)

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304
Q

5p

A

cri-duchat
round face, hypertelorism, low ears, prominent nasal bridge
Card: PDA, VAS, ASD< TOF
high pitched cry
LOC _downstairs TV room where cats cuddle

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305
Q

4p

A

wolf hirschorn
Shield face
4p deletion
LOC: pool room with chewbacca

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306
Q

MYBPC 3

A

MYBPC 3: My Blood Pressure Contractility! 3

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307
Q

gene mutation differences between Duchenne and Becker

A 
Duchenne = frameshift mutation causing absent protein
Becker = in-frame causing a shorter protein
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308
Q

when do DMD get cardiomyopathy by?

A

Dilated 100% by age 18, BMD in the teens as well, but live into their mid-40s

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309
Q

genetic causes of Emery Drifuss MD?

A

All nuclear proteins (in the shower bubble)
EMD and FHL (X linked girly soaps = emerald and fooh la la) with LMNA (Lamin A is washcloth) = Auto recessive
LOC up bathroom Emerald DRY fussy
at risk for conduction issue,s sudden death
all types of musc dystrophy
elbow and achiles contractures

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310
Q

elbow and achilles contractures

A

Emery Drifuss MD?
All nuclear proteins (in the shower bubble)
EMD and FHL (X linked girly soap = emerald X linnked girly shampoo = fooh la la) with LMNA (Lamin A is washcloth) = Auto recessive
LOC up bathroom Emerald DRY fussy
at risk for conduction issue,s sudden death with EMD and LMNA
all types of musc dystrophy
elbow and achiles contractures trouble with showering

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311
Q

mutation of proteins that associate the cell membrane with dytrophin

A

sarcoglycans
DES mutations, risk of SCD (for DESmin, life does not go on)
limb girdle defects
both girls and boys (Matt wearing a girdle)
LOC : bros room

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312
Q

mutation at risk for SCD in muscular dystrophy?

A

EMD (soap), LMNA (washcloth), DES (DESmin)

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313
Q

ataxia with HCM

A 
Friedrich's ataxia
Frataxin
FXN (FRAT, FRAT, FRAT!)
HCM -> DCM
LOC: my room
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314
Q

elevated lactate, ptosis, seizures, encephalopathy

A

mitochondrial diseases: MELAS, Kearnes Sayre
MELAS = MEALS (red ragged fiber)
LOC: inside seat of dining room table
Kearnes Sayre = Corn slayer
ptosis with conduction disease = Kearnes Sayre
LOC: window side of dining room table, need for pacemaker if diagnosed with Kearnes Sayre and high grade heart block

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315
Q

decompensation with fastinging, hypoglycemia, metabolic acidosis

A

fatty acid oxidation defect
HCM and arrythmia
LOC: downstairs desk

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316
Q

weakness, failure to thrive, hepatomegaly, giant ECG voltages

A 
GSD Pompe = acid maltase deficiency (GSDII)
GAA mutation (someone Pumping Iron and going GAAAAA)
HCM
Infiltration of conduction system causes short PR
LOC: weights downstairs

Dannon is GSD IIb ( differentiate normal alpha glucosidase level), LAMP2 mutation sitting at the desk with a LAMP

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317
Q

coarse facial features, skeletal and joint problems

A

mucopolysaccharidoses
Hurlers and Hunters
Hunters are X linked
Cards: thickening and stiffening of valves: MR and AI and HCM, risk of sudden death arrhythmia
LOC: kitchen sink (cleaning mucous off dishes)

318
Q

lipidoses with heart problems

A

Gaucher (Toaster) and Fabry (Stove)

319
Q

progressive neurological deterioration with HSmegaly, anemia, thrombocytopenia, bone pain

A 
Gaucher disease
GBA (Bad GPA)
Tea and Toast diet leads to anemia
glucocerebtosidase (grain brain)
LOC: Toaster
320
Q

GBA

A

Gaucher, bad GPA = GBA

321
Q

male with periodic crisis of severe extremity pain, angiokeratomas, corneal clouding

A 
Fabry Disease = X linked
mutation in GLA (GLAss stovetop)
corneal clouding is not glassy
CV: mitral valve disease, HCM, conduction infiltration and cerbrovascular disease
LOC: stovetop
322
Q

round face, tall forehead, deep set eyes, prominent ears

A 
Barth syndrome
TAZ = TAZmanian devil
neutropenia, skeletal myopathy
DCM, LVNC, arrythmia w/o conduction disease (basically the only inborn error of metaboism to have DCM instead of HCM)
LOC: Kendall Kitchen Table
323
Q

4 big categories of cardiomyopathies

A

Sarcomeric, Dystrophinopathy, Muscular dystrophies, Inborn Errors of Metabolism

324
Q

ACHD TOF, risk of PCOD?

A

Potts or Waterston shunt, direct connection causes enregulated pulm blood flow, risk of PHTN and then PCOD

325
Q

what is Nakata Index, how is it used?

A

NI = (RPA dimater+ LPA diameter + MAPCA diameter) / BSA
used in PA/VSD
If >200 -> close VSD with RV-PA conduit
If < 200 -> RV-PA conduit and leave VSD open

326
Q

which rim is most important to allow for device closure of an ASD?

A

posteroinferior

327
Q

which coarct repair has the highest risk of speudoaneurysm?

A

synthetic patch repair

328
Q

treatment f post-pericardiotomy syndrome

A

high dose aspirin for 4-6 weeks

329
Q

onc kid with sepsis

A

early form of anthracyclin-induced CMP

330
Q

LVNC, which syndrome to associate?

A

Barth - Tefazzin mutation (think about steak being cut up by tazmanian devil), X linked

331
Q

LGE location: epicardial

A

myocarditis (closest to the artery)

332
Q

LGE location: midwall

A

DCM

333
Q

LGE location: subendocardial

A

ischemia (farthest from the artery)

334
Q

LGE location: LV-RV junction

A

HCM (pulled the most in these regions)

335
Q

LGE location: none, instead aneurysm+clot

A

Chagas

336
Q

LV anuerysm disease

A

Chagas

337
Q

Prognosis of fulminant myocarditis

A

fast on and fast off. hemodynamic compromise with 2 weeks of presentation, Px better than acute which has a less distinct onset

338
Q

myocarditis that doesn’t get better after 2 weeks

A

suspect giant cell myocarditis, treat with steroids. poor prognosis, due to autoimmune most likely, 10% recurrence following transplant

339
Q

histo slide with multinucleated giant cell

A

giant cell myocarditis

340
Q

Tx lyme carditis w/ dysfunction

A

IV CTX 3 weeks

341
Q

lyme carditis, po or IV antibiotics?

A

If complete heart block -> IV
If PR>300 -> IV
If PR <300 -> po

342
Q

how to Dx lyme carditis?

A

lyme titers

343
Q

germ for lyme carditis?

A

borrelia burgdorferi (sounds like you are eating peanut butter)

344
Q

right sided myocarditis? disease? germ? Dx? Tx? associate?

A

Chagas disease, trypanasoma cruzi
Dx with ELISA test
Tx: bendazole
Associate LV aneurysm -> clot -> stroke

345
Q

incidence of DCM? 5 yr survivival?

A

0.57 / 1k

5 year survival = 50%

346
Q

nutritional deficience that causes DCM?

A

thiamine deficiency

347
Q

sarcomeric causes of DCM?

A

Titan TTN, MyH6,7

348
Q

Lamin defect causes?

A

DCM with conduction issues

349
Q

Tx asymptomatic DCM? then if symptomatic

A

betablock and ace

syptoms -> lasix, aldactone, digoxin

350
Q

tefazzin gene?

A

TAZ, barth syndrome, DCM that is X linked, also has LVNC (Think of meat being cut up on the dining room table by Tazmanian devil), also has neutropenia (CBK link)

351
Q

neutropenia

A

think of Barth syndrome

352
Q

desmosome mutations

A

ARVC, autosomal dominant

353
Q

Define HCM

A

LV hypertrophy without an identifiable cause (no increased afterload)

354
Q

Define DCM

A

dilation and systolic dysfunction in 1 or more ventricles

355
Q

MCC SCD in athletes

A

HCM

356
Q

incidence of HCM?

A

0.47 / 100k

357
Q

disarray in a biopsy micro?

A

HCM

358
Q

normal BP response to exercise?

A

> 20

in HCM, < 20 is an indication for ICD

359
Q

on HCM therapies that are proven to improve survival?

A

1) ICD
2) exercise restriction
beta blockers don’t!

360
Q

medical treatment of HF in HCM?

A

1) beta blocker
2) CCB (verapamil)
3) exercise restriction

361
Q

indications myomectomy in HCM?

A

refractory symptosm with LVOT >50mmHg, SAM

362
Q

if you have a patient with HCM, what do you do with their family members?

A

screen all first degree. in genotype+phenotype negative patients, follow Q3H until 30 years and Q1Y during puberty

363
Q

what do mass and volume got to do with hypertrophy?

A

in HCM: inc LV mass to volume ratio

Athlete’s heart: normal LV mass/volume ratio

364
Q

MC inherited ataxia?

A

freidrich’s ataxia

365
Q

dx of hurler disease?

A

urinary GAG -> confirm with blood test
glycosaminoglycans are unable to break down due to alpha-L-iduronidase deficiency (mucopolysaccharide

hunters is milder with no corneal clouding (hunter vision through x-link in the scope)

366
Q

inborn error metabolism, type of cardiomyopathy typically?

A

HCM

367
Q

left and right heart failure with prominent S2

A

suspect RCM

368
Q

define PAH

A 
PH = mean PAP > 25
PAH = 
    mean PAP >25
    wedge<15
    PVRi > 3
369
Q

3 MC genes of PAH

A

BMPR2 (75%)
ACVRL1 ( also associated with HHT)
TBX4 (associated with abnl lung development)

370
Q

histo feature of adanced PAH?

A

plexiform lesions

371
Q

prevalence of PAH in CHD?

A

repaired 15%, unrepaired 30%

372
Q

how to decide operability in PAHD with CHD?

A

1) cath for PVR (if PVR>6, PVR/SVR>0.3)
2) next, vasodilator test
3) PH therapy with repeat cath

373
Q

single ventricle, what is abnormal pulm HTN?

A

PVRi>3, TPG>6

374
Q

first line PAH treatment in mild disease?

A

calcium channel blocker

375
Q

2 endothelin receptor subtypes an affects

A

A is on smooth muscle, B is on Both smooth and endothelial

376
Q

difference between ambrisentan and bosentan?

A

bosentan affects Both ET_A and ET_B and causes liver damage

ambrisentan specific for A (smooth muscle), no liver damage

377
Q

first line therapy for severe PAH and RV failure

A

prostacyclin

378
Q

WHEN TO DO SURGERY FOR PAH?

A

ONLY with suprasytemic RV pressure, to unload the right ventricle

379
Q

what are the ACC/AHA stages?

A

A: at risk, no dysfn, no symptoms
B: asymptomatic with dysfunction
C: symptomatic LV dysfunction
D: refractory requring VAD/Tx

380
Q

what are the NYHA stages?

A

1: asymptomatic
2: symptomatic with mod exertion
3: symptomatic with mild exertion
4: symptomatic at rest

381
Q

treatments associated with each ACC/AHA stage:

A

A: at risk, no dysfn, no symptoms -> monitor, treat risk factors
B: asymptomatic with dysfunction -> ACE + BB (carvedilol)
C: symptomatic LV dysfunction -> add lasix/aldactone, fluid/salt restrict
D: refractory requring VAD/Tx -> LVAD, HTx

382
Q

HF symptoms, what springs to mind?

A

Stage 3, fluid causes symptoms of congestion (start lasix/aldacton, restrict) -> digoxin for symptoms

383
Q

PV loop in heart failure, what does lasix do, what does afterload reduction do?

A

lasix dec preload, decrease right side

afterload: decrease top

384
Q

what is sacubitril?

A

part of entreso witch is scubitril/valsartan. neprilysin inhibitor, inhibits breakdown of BNP for diuresis

385
Q

aldosterone association

A

fibrosis

386
Q

laplace law equation

A

Wall stress = Pxr/(2x wall thickness) = pressure x volume/LV mass

387
Q

what does B1 do in heart failure?

A

B1 receptors are downregulated, BB help upregulate them

388
Q

cause of heart failure in kids?

A

non-adherance (esp adulescents with salt/fluid restriction)

389
Q

2 minute assessment of HF

A

cold/warm, dry/wet
wet -> diuretics
cold -> vasoactives

390
Q

HF with Low CO, treatment

A

vasodiltors, nipride/nitroglycerine, milrinone

391
Q

evidence for lasix

A

improves symptoms, no survival benefit

392
Q

evidence for ACE-inhibitor

A

reduces mortality, reverse remodeling (all patients with reduced EF

393
Q

when to use a -sartan?

A

when ACE causes cogh/angioedema

394
Q

evidence for carvedilol, notable side effect?

A

reduces mortality, reverse remodeling, use with all reduced EF, causes wheezing, would switch to metoprolol

395
Q

side effect spironolactone

A

hyperkalemiam if gynecomastic, switch to eplerinone

396
Q

evidence for spironolactone

A

reduce mortality, reverse remodeling

397
Q

PANORAMA trial

A

use of valsartan-sacubitril in kids reduces mortality, reverse remodeling (a wide panorama)

398
Q

HF drugs shown to improve survival and reverse remodel?

A

ACE/ARB
Beta blockers (carv, meto, biso)
Aldo receptor blockers
(NOT lasix, NOT digoxin)

399
Q

what evidence for digoxin?

A

reduces hospitalizations, improves symptoms but no survival

side effects: arrythmia, heart block, peaked T, prolonged QTc, med interactions with amiodarone

400
Q

hyperK on AC/ARB, what to do?

A

dose reduce spironolactone

401
Q

cough develops with ACE, what to do?

A

r/o heart failure(CXR), then switch to ARB if cough persists

402
Q

angioedem on ACE< what to do?

A

STOP immediately, switch to ARB

403
Q

when you admit a patient in heart failure, do you stop the beta blocker?

A

continue BB unless dec CO, when you would start inotropes

404
Q

wheezing on bet blocker

A

switch from carvedilol to metoprolol

405
Q

asymtomatic LV dysfn, what 1 drug to start?

A

ACE-I, (beta blocker second choice)

406
Q

what drug for HFpEF?

A

lasix only

407
Q

indication ICD in kids

A

DON”T use adult guideline of EF<35%

ONLY aborted Sudden Death, unexplained syncope, recurrent VT

408
Q

when to consider cardioc resyncrhonization therapy?

A

EF<35%, LBBB, QRS>150

single V with BBB (dominant-side) and EF <35%

409
Q

PLE; Dx? Tx? Px?

A

stool alpah 1 antitryypsin
Tx: aldactone, steroids, heparin, octreotide, fenestration, transplant
Px: 5yr 50%

410
Q

Class I indication for Heart transplant

A

Stage D
Stage C with limitation in physical activity, growth failure, sudden death, restrictive w/ PH
Retransplant for graft vasculopathy with dysfn

411
Q

Contraindications to Heart transplantation to know:

A

irreversible PVR>8WU (no NO response)

retransplant within 6 months of transplant

412
Q

1yr and 5 yr survivla of Tx in infants and adolescents

A

Infants: 1yr = 75%; 5yr = 68%
Adoles: 1yr = 88%; 5yr = 68%

413
Q

median survival of Heart Transplant

A

15 yr

414
Q

what does HLA lead to?

A

antibody mediated rejection

solution: avoid, desensitize, or treat AMR aggresively

415
Q

who can get ABO incompatible, what are the outcomes?

A

infants with isohemaglutinin titers <1:8 (outcomes same as blood group matched)

416
Q

goal ischemic time in HTx?

A

<4-6hours

417
Q

purpose of ATG?

A

blasts T cells, allows delay in calcineurin start (less kidney problems)

418
Q

ATG mechanism of action, treatment goal? alternative?

A

ATG is anti-CD3, CD3 count <30 (3, 30), alternative is basiliximab (IL-2)

419
Q

Most important immunosuppresant in HTx? MOA? side effects?

A

calcineurin inhibitors (tacro and cyclosporine)
block T cell activation, stop IL-2 production (looks like arrows)
nephrotoxic, diabetes (toxic personality and probably had DM)

420
Q

adjunct HTx meds to calcineurin inhibitors?

A

MMF (or imuran)
antimetabolite, prevents both T and B cell proliferation
GI side effects, neutropenia (studies neutrophils, GI upset often at that job)

421
Q

when do you use sirolimus?

A

kidney dysfunction and CAV
MOA mTOR inhibitor, but inhibits more than this (TORO inhibitor)
side effect: wound healing, aphthous ulcers

422
Q

side effects tacro

A

nephro, diabetes (ERIC C has toxic kidneys and DM)

423
Q

side effects cyclosporine?

A

HTN, gingival Hplasia

424
Q

side effects sirolimus?

A

delayed wound healing, proteinuria, hyperlipid

425
Q

side effects MMF

A

mycophenolate

anemia, neutropenia, GI side effects

426
Q

MC presentation of Acute cellular rejection

A

asymptomatic, picked up on biopsy

427
Q

grading of acute cellular rejection?

A 
mild = 1R = local inflammation
moder = 2R = loc inflam + focal necrosis
severe = 3R = diffuse inflammation + necrosis
428
Q

biopsy of AMR vs. ACR?

A 
antibody = edema and c4d positive (no cells)
cellulat = inflammation (cells) +/- necrosis
429
Q

causes of AMR? how to treat? risk for?

A

antibodies: high PRA, DSA, or positive cross match
Treat with IVIG + plasmapheresis
risk of CAV (very tight association)

430
Q

history of antibody mediated rejection puts transplant patient at risk for?

A

coronary artery vasculopathy, sirolimus helps by being active against B in addition to T

431
Q

HTx with Fever + diarrhea: treatment

A

CMV: risky when off valcyte, risk for CAV
Tx: gancyclovir

432
Q

HTx with Fever + pneumonia: treatment

A

CMV: risky when off valcyte, risk for CAV
Tx: gancyclovir

433
Q

HTx with abd pain and nodules. Dx? Tx?

A

likely PTLD< must Bx for diagnosis (EBER positive), EBV driven
Tx: decrease immunosuppression

434
Q

HTx, rising Creatintine? Dx? Tx?

A

Calcineurin inhibitor nephropathy

switch to sirolimus

435
Q

leading cause of late graft loss in HTx? Tx?

A

CAV
risks include antibody mediated rejection and CMV infection
Tx: switch to sirolimus, ReTx if dysfunction

436
Q

new 2018 ACHD guidelines, what worts of classifications

A

anatomic (simpled, moderate, great complexity), AND physiologic (NYHA class), classifcy according to highest complexity

437
Q

predictors of successful transition to ACHD?

A

1) older age at last visit 2) greater number of surgeries 3) documented recommendation to follow with ACHD center

438
Q

when do you start transition counseling? how should you do it?

A

12 years of age; written transition plan including need for lifelong care

439
Q

increased risk of center in ACHD?

A

adutls w/ CHD, 1.5-2x greater lifelong risk

440
Q

how many CHD patients develop a form of SVT?

A

45% with an atriotomy!

441
Q

causes of chronic breathing problems in ACHD?

A

30% restrictive spirometry, 20% OSA

442
Q

when do you do phlebotomy in ACHD?

A

used to be done routingly in cyanotic, but RARELY indicated now. Only if HGB>20 and hypovolemia has been excluded (Probably not the right answer here)

443
Q

a cynotic pt asks you about pregnancy, what do you counsel?

A

pregnancy contraindicated, also get on some non-estrogen birth control

444
Q

how to treat hyperviscocity in cyanotic patient?

A

adequate hydration?
iron deficiency anemia?
phlebotomy RARELY indicated if HGB >20

445
Q

EKG risk factor in TOF?

A

QRS >180, independent risk factor sudden death

446
Q

arrythmia in TOF?

A

double risk of mortality, V tach is more like 50%, atrial 10%

447
Q

TOF: diastolic dysfunction

A

relates to sustained VT

448
Q

ACHD TOF with dysfun?

A

need to treat aggresively, associated with poor outcomes

449
Q

Classes of indications for PVR in TOF?

A

Class 1: symptoms + moderate PI
Class IIa: ventricular enlargement w/ mod PR
mild-mod RV/LV dysfn
RVEDV>160, ESV>80, RVEDV>2xLV
RVSP >2/3 systemic
Class IIb: Another lesion or an arrythmia

450
Q

what does PVR do for outcomes in TOF?

A

no difference in outcomes! just good for symptoms

451
Q

higest incidence of aneurysm formation in coarct repair?

A

patch, but specifically dacron patch!

452
Q

causes of hypertension after coarct?

A

abnormal endothelial function, increased arterial stiffness (often have exercise induced hyeprtension

453
Q

boards answers for fontan adult with arterial desaturations? (8 of them)

A

systemic venous collaterals to the pulmonary vein**
then fenestration, atrial communication
pulm AV malformations
pre-fontan connection of hepatics to CS/LA
LSCV
Levoatrial cardinal vein
R-> L shunting through thebesian veins

454
Q

fontan anticoagulation outcomes?

A

ASA best, warfarin a little less, none is BAD

455
Q

worse type of valvular lesions in pregnancy?

A

Stenosis much worse than regurgitation (low SVR makes regurg less problematic)
Mitral stenosis: inc HR decreased filling time and pulm edema (Tx beta blockers)
Ao stenosis: inc CO leads to inc end-diastolic pressure
Treat beta blockers

456
Q

antigcoagulation in pregnancy scheme?

A

If Warf <=5mg: contineu warfarin
IF warf >5mg: switch to LMWH
2ns/3rd: all get warfarin and ASA
prior to deliver: planned vag delivery with UFH

457
Q

mechanical valve pregnancy, warfaring 3mg, what do you do?

A

keep warfarin, add ASA in 2nd trimester, planned V delivery with UFH

458
Q

mechanical valve pregnancy, warfaring 6mg, what do you do?

A

switch to LMWH, 2nd trimester, switch to warf+ASA, planned V delivery with UFH

459
Q

who can’t get combined contraceptives?

A

cyanosis, Fontan, mechanical heart valve: risk of thrombosis

460
Q

why is IUD preferred for most women w/ CHD?

A

<1% failure rate, no thrombosis, Fontan has a tiny risk of vagal reactions

461
Q

which at risk CHD women should not use progestrone-only birth control?

A

good because less thrombotic potential but higher failure rate (10%)
OK for itnracardiac shunt, OK for for cyanosis,
contraindicated in heart failure due to FLUID retention

462
Q

what are the 9 cardiac risk factors for mommas with CHD?

A 
fontan
prior cardiac event
poor functional capacity
cyanosis
mechanical heart valve
decreased Ventricular function
Pulmonary hypertension
aortopathies
symptomatic left-sided obstructive lesions
463
Q

3 mechaisms of arrythmia

A

automatic, re-entrant, triggered automatic

464
Q

strucutural heart disease associated with accesory pathway

A

ebsteins (20%), L-TGA (5%), HCM

465
Q

SVT shows preexcitation pattern, what does this mean?

A

antidromic AVRT (orthodromic goes through AV node, does not show preexcitation)

466
Q

which is more common, orthodromic or antidromic AVRT?

A

orthodromic in 95%

467
Q

what are the 2 main variants of accesory pathways?

A

PJRT: slow retrograde only, no preexcitation, long VA resulting in slower HR (slower in one’s PJs, almost like you are moving BACKWARDS)
Mahaim: antegrade only, wide complex SVT since orthodromic (M in Mahaim is wide, 2 A’s in mAhAim for antegrade)

468
Q

natural history of newborn SVT

A

93% no clinical SVT by 8 months.
subclinical goes away in 30% by 12 months.
Recurrs around age 8

469
Q

cascade of treatment for acute AP SVT

A 
vagal, adenosine, 
Beta blocker (if normal EF)
CCB (if normal EF, > 1yr and no preexcitation at baseline)
Digoxin (if no preexcitation)
Amiodarone (if reduced EF)
470
Q

who do you avoid CCB in SVT?

A

example is verapamil

dysfunction (depresses EF), <1yr (hypotension), or preexcitation (blocks AV node)

471
Q

chronic treatment for SVT with preexcitation?

A

beta blockers, avoid digoxin and verapamil (AVN blockers)

472
Q

chronic treatment for SVT without preexcitation?

A

beta blockers, digoxin, class Ia, Ic, III, IV

473
Q

what RR interval is dangerous in WPW?

A

<250 msec, risk of sudden death

474
Q

direction of typical AVNRT?

A

slow fast (short RP), P wave will be a barely visable inverted p at the end of QRS from fast Upstroke

475
Q

direction of atypical AVNRT

A

fast slow (long RP), P wave will be inverted from SLOW Upstroke

476
Q

4 types of long RP tachycardias

A 
atypical AV nodal reentry tachycardia (fast slow, slow upstroke)
PJRT (slow retrograde AP)
EAT (automatic atrial goes through AVN)
sinus tach (goes through AVN)
477
Q

natural history fo post-op a tach

A

resolves within 2-3 months

478
Q

natural history of a tach in patient < 3 years

A

resolves within 2-3 months

479
Q

5 CHD at risk fo A tach in long term

A

Fontan, dTGA s/p atrial switch, TOF, Ebstein, ASD

480
Q

irregular tachycardia, what do you look for?

A

distinct p waves, then it is atrial tachycardia (if not A fib)

481
Q

2 ypes of atrial flutter

A

typical (TV annulus)

atypical (incisional), will usually not have the sawtooth waveform)

482
Q

acute treatment for atrial flutter, how do you rate control?

A

depends on heart failure status:
if no heart failure: beta-blocker or calcium channel blocker, or combination BB/dig , CCB/dig
if heart failure: digoxin or amiodarone

483
Q

acute treatment for atrial flutter, how do you cardiovert?

A

If < 48 hours:
first try: pharmacologic (ibutilide, amio, procainamide)
then try: DC cardioversion
If > 48 hours:
option 1: rate control, anticoag x 3 weeks, then electie DCCV, the oral anticoagulation x 4 weeks
option 2: TEE, bolus hepatin, acute DCCV, oral anticoagulation x 4 weeks

484
Q

when do thromboembolic events happen after DCCV for afib/aflutter?

A

first 3 days due to atrial stunning, ALWAYS treat for 4 weeks with anticoagulation afterawrds

485
Q

4 types of a fib?

A

paroxysmal: terminates spontaneously, last < 1wk
persistent: lasts > 1 week
long standing: lasts > 1 yr
permanent: sustained, no further attempts to restore sinus rhtym

486
Q

rate control tretment for a fib, what considerations and what drugs?

A

heart failure and accesory pathway:
If no HF, no AP: BB, CCB, amio
if HF: dig, BB, amio (avoid CCB)
If AP: ibutilide, procainamide

487
Q

acute treatment for A fib with WPW?

A

rate control: procainamide, avoid AVN blocker as single agent
If < 48 hours: ibutilide, procainamide, DCCV

488
Q

definition of sustained VT?

A

> 30seconds

489
Q

risk of using ibutilide?

A

4% risk of torsade, avoid with low EF

490
Q

DDX for wide QRS complex tachycardio?

A

VT, SVT with BBB, SVT with aberration

491
Q

PVCs: 4 associated conditions

A

1) structurally normal,
2) cardiomyopathy/myocarditis
3: AVRC (>500 PVCs in 24h)
4: channelopathy (often multiform)

492
Q

when do you worry abouut PVC related cardiomyopathy?

A

> 10%

493
Q

how do you manage PVCs in kids?

A

aymptomatic with normal EF: observe
symptomatic infant: BB
symptoamtic child: BB, CCB
LV dysfunction: BB, CCB

494
Q

newborn with ventricular rhythm, what diagnosis?

A

accelerated idioventricular rythm if;

1) rates just faster than sinus
2) structurally normal heart
3) baseline EKG is normal
- -> benign, persists 2-3 months

495
Q

VT with LBBB pattern?

A

RVOT tachycardia

LBBB, Inferior axis

496
Q

12 lead with VT, where do you look?

A

look for BBB and inferior leads
LBBB + upright in II, III aVF —> RVOT
RBBB + downward in II, III aVF —> Idiopathic/fascicular VT

497
Q

RVOT tachycardia, what DDX?

A

usually structurally normal, also AVRC, myocarditis, tumor

498
Q

treatment of RVOT tachycardia?

A 
verapamil, adenosine sensitive
BB, class I, III agents
499
Q

treatment of idiopathic/fascicular VT?

A

verapamil sensitive

BB, CLass I, III agents

500
Q

what is AH? HV?

A

AV node conduction

purkinje conduction

501
Q

what is an effective refractory period?

A

longest RR where stimulus fails to propogate

502
Q

differentiate between mobitz 1 and 2 on invasive EP study?

A

mobitz 1: AH interval lengthens, loss of H

mobitz 2: AH stays the same, then there is a loss of V

503
Q

risks relating to location of ablation

1) coronary injury
2) recurrence rate
3) AV block
4) stroke

A

1) coronary injury - left posterior
2) recurrence rate - right free wall
3) AV block - septal
4) stroke - left side

504
Q

EP study, first question

A

stim or no stim

505
Q

EP study, stim, next question

A

atrium or ventricle

506
Q

EP study, stim atrium, next question

A

conduction to V (look for H and V). If His present at time of block, His/purkinje disease. If His drops, probably wenkebach

507
Q

EP study, stim ventricle, next question

A

conduction to A: eccentric or concentric

508
Q

EP study, no stim, next question

A

fast or slow

509
Q

EP study, no stim, fast, next question

A

reg/irreg, narrow/wide, AV relationship

510
Q

EP study, no stim fast, narrow, reg, A>V, DDX?

A

Fib and flutter are obvious, others can be EAT or 2:1 AVNRT

511
Q

EP study, no stim fast, narrow, reg, V>A, DDX?

A

junctional or VT

512
Q

EP study, stim ventricle, eccentric

A

left lateral pathway

513
Q

EP study, no stim fast, narrow, reg, V=A, DDX?

A

see if midline or eccentric

514
Q

define dual AV node physiology

A

AH interval increases at least 40 msec after 10 msec decrease in coupling interval

515
Q

short HV

A

accesory pathway!

516
Q

risky WPW number

A

250msec!

517
Q

what weight to get to prior to EP study

A

15 kg

518
Q

EP study, no stim, slow, earliest A in HRA, next question

A

HV interval (if small < 35, then its an accesory pathway)

519
Q

5 phases of channels for myocardium, what ions?

A 
0 = Na
1 = K 
2 = K and Ca+
3 = K (Long QT syndrome 1 and 2)
4 = K
520
Q

2 phases of channels for SA and AV node, what ions?

A 
0 = Ca++ (Ca is the clock)
2,3,4 = K+
521
Q

Class 1a agents, effect on AP curve?

A

quinidine (C), procainamide (desk), diopyramide (N)

delays depol and repol (late to bed, late awake)

522
Q

quinidine, type, channels, treatment, contraindications

A 
1a (Na with tiny K)
indication (Brugada)
don't use in long QT
risk of torsade and cinchonism
LOC: C bedside
523
Q

procainamide

A

1a (Na with modeate K)
some peripheral ganglionic blockade leading to ypotension
prolongs QT
used in svt, vt, jet
QT prolongation, particularly from hepatic conversion to NAPA
LOC: desk

524
Q

disopyramide

A

NA+ and K+ (similar to quinidine)

LOC: Nick bedside (anticholinergic and running clothes leading to VV syncope)

525
Q

cinchonism

A

quinidine

526
Q

class 1B EP stuff

A 
lidocaine (upper bunk), mexilitine (lower bunk), phenytoin (Fun-etoin)
Na+ selective
shortens QT (early awake)
No effect on atrium or accesory pathway, only for ventricular stuff
527
Q

lidocaine

A

NA + selective
no effect above His
LOC: C bed

528
Q

mexilitine

A

Na+ selective (oral lidocaine)
no effect above His
used as treatment for LQT3 (cutey 3)

529
Q

phenytoin

A

anti-seizure medication

used to be used for digoxin toxicity (dig in attic)

530
Q

class 1c stuff

A

flecainide (change table), propafenone (bed by the window since some BB activity)
prolongs QRS and QT (QRS widening is the big risk!)

531
Q

QRS widening

A

flecainide tox

flex slows conduction and slows dissociation

532
Q

flecaininide type, activity, watch out for, interactions

A

class 1c, widens QRS, slows dissociation, indicated for SVT, VT, watch out for wide QRS (VERY strong Na channel blocker)
increases digoxin and propranolol levels
amio and propranolol increase flec levels
LOC: (changing table, must be very careful)

533
Q

proprapenone type, activity, watch out for, interactions

A

increases action potential duration widens QRS, some BB activity
LOC: by the window
ineraction: may icnrease dig levels

534
Q

which Bet blockers are selective, which nonselective?

A

selective A:M, nonselective N=Z

selective: atenolol, esmolol, metoprolol
nonselective: nadolo, propranolol

535
Q

5 beta blocker metabolism?

A

nadolol and atenolol kidney (pool and sprinkler)
metoprolol and propranolol liver (mountain and prop play)
esmolol is blood

536
Q

drug drug interaction with beta blockers?

A

propranolol interacts with flecainide (both level higher)

NSAIDS potentiate hypotension in nonselective

537
Q

B1 and B2 effects?

A

B1: cardioselective, receptors on the heart, slow HR and conduction, decrease contractility
B2: lungs and smooeth muscle (bronchospasm, vascoconstriction)

538
Q

hypoglecmia and meds?

A

beta blockers

539
Q

strongest Na channel blocker?

A

flecainide, class 1C

540
Q

anticholinergic side effects med

A

disopyramide

541
Q

K channel EP effects

A 
delay repolarization (late brunch), prolong QT (important!)
risk of torsade with all
542
Q

amiodarone stuff

A

blocks K+, Na+, Ca++, some BB activity, some alpha blocker
less LV depression than other antiarrythmics
LOC: by the bananas
drug-drug: increases digoxin levels, interacts with coumadin
lots of adverse effects

543
Q

why is amio preferred over other antiarrythmics

A

less LV depression

544
Q

Sotalol stuff

A

K+, little Na+
nonselective BB
prolongs AP duration
risk of torsade

545
Q

ibutilide stuff

A 
K+ channel blockers, QT prolongation
only used in acute fib/flutter conversion 
(Ibutislide = getting out of a chair)
pretreat with K or Mg
LOC: tough chair
546
Q

Dofetilide

A

atrium only K channel, used in a flutter and fib
(LOC: big chair where you DOF your worries)
2-4% risk torsade

547
Q

Dronedarone

A

less effective amiodarone

LOC: fake kid kitchen, less effect, fake foods)

548
Q

where do Ca blockers act?

A

SA and AV nodes, Ca++ upstroke, slows it down

don’t use in AVRT due to AV block

549
Q

verapamil stuff

A 
Ca channel blocker
alpha adrenergic effect (hypotension)
used for fascicular / idiopathic VT
LOC: upsairs bathroom (use in VT)
contraindicated <1 year
don't use in WPW
550
Q

diltiazem

A

Ca channel blocker
used in SVT as fib and flutter control
LOC: downstairs bathroom , close to A fib and flutter K blocker meds)

551
Q

Adenosine stuff

A

K channel opener via A1 receptor (close to K stuff)
shortens action potential duration by openign K channels, hyperpolarizes membrane, so effects the duration of SA and AV node
LOC: basement

552
Q

Ivabradine

A

Inward K current blockers, inhibits SA node activity -> dec HR
used in POTS, inappropriate sinus tachycardia

553
Q

what meds increase digoxin levels?

A 
Frank - Flecainide
Ate - Amiodarone
Pizza - propafenone
Very - verapamil
Quickly - quinidine
554
Q

how many peopl have syncope? recurrence? population? % VV synceop, % cardiac syncope?

A

up to 50%, only 10% recurrence rate, MC adolescents and females
80% VV
2% cardiac

555
Q

mechanism of VV synceop

A

standing -> dec preload -> HYPERcontraction -> mechanoreceptors activated -> dec sympathetic and inc PS output:

1) bradycardia
2) peripheral vasodilation
3) failure of reflex cerebral dialtion

556
Q

4 signs of siezure over syncope

A

1) smell prodrome
2) rythmical movements before the fall
3) tongue bite,
4) confusion

557
Q

Treatments for VV synceop

A

salt and water
florinef (evidence in adults)
midodrine (most evidence in peds - 70% risk reduction)
beta blockers (better for > 40)

558
Q

Dx definition of POTS

A

Lasts . 6 months, > 40 bpm, absense of orthostatic hypotension (BP drop < 20)

559
Q

4 big risk factors for sudden death in HCM

A

1) personal hsitory of sustained VT, VF, or SCD event
2) family history SCD
3) unexplained syncope
4) thickness > 30mm

minor: NSVT, abnl BP exercise response

560
Q

Dx of LCA off right, next step:

A

restrict, surgery -> after 3 months exercise, if negative can participate in sports

561
Q

Dx RCA off left, next step

A

exercise test, can play sports if asympomatic and negative

562
Q

EKG findings of ARVC

A

V1 and V2 epsilon wave (post-excitation of islands of RV muscle)
upright T wave in V2, V3

563
Q

etiology of epsilon wave

A

(post-excitation of islands of RV muscle)

564
Q 
LQT1: 
gene, 
type of defect
trigger
ECG pattern
A

gene: KCNQ1
type of defect: loss of function of K channel
trigger: swimming, diving sprinting (diver looks like a number 1, we’re number 1!)
ECG pattern: broad based t waves (athetic broad based stance)

565
Q 
LQT2: 
gene, 
type of defect
trigger
ECG pattern
A

gene: KCNH2
type of defect: loss of function of K channel
trigger: auditory ( 2 ears)
ECG pattern: low amplitude T waves (keep the volume down!)

566
Q 
LQT3: 
gene, 
type of defect
trigger
ECG pattern
A

gene: SCN5a
type of defect: gain of function
trigger: at rest, during sleep (3 pillow) most dangerous
ECG pattern: flat ST and late peaking T waves (shark fin)

567
Q

IN general, what disk factors for LQT syndrome?

A

prior syncope, QTc >500 (except 3, where you use 470 as the cutoff)

568
Q

CPVT: what defect? what MOA

A

ryanodine receptor-> leasd to excessive Ca leak out of the SR, delayed after depolarization

569
Q

bidirectional VT

A

CPVT!

570
Q

CPVT< what type of VT on EKG

A

bidirectional VT!

571
Q

mortality of CPVT?

A

30-50% by 30 years

572
Q

treatment of CPVT

A

betablocker (Nadolol) +/- flecainide

ICD if recurrent VT or cardiac arrest

573
Q

brugada,
gene
EKG abnormalities

A

gene: SCN5A: reduced Na curent
EKG: coved ST elevation in V1 V2 that loks like a sledding hill, unmasked with Na channel blockers (procainamide, flecainide)

574
Q

9 risk factors for SCD in TOF?

A

2 history: more time from surgery to now, h/o syncope or symptomatic arrythmia
3 surgical: RVOT patch/ventriculotomy, prior shunt, older age at surgery
4 workup:
Cath: increased RV/LV pressure ratio (but LVEDP>12 is most important)
ECHO: reduced LV fn
EKG: QRS>180
EP: inducible VT, or NSVT

575
Q

Risk of SCD in aortic stenosis

A

2+ episodes of syncope, outflwo gradient > 50 mmHg, AI, High LVESP, LV dysfunction

576
Q

what is bachman’s bundle?

A

conductive tissue connects RA to LA

577
Q

how does the autonomic NS modulate SA and AV nodes?

A

CA++ permeability (cagus decreases which decreases upslope and slows HR)

578
Q

how to differentiate vagal bradycardia from sinus node dysfunction?

A

vagal is related to situation, position, carotid sinus, and AV node may be associated

579
Q

indications for pacemaker for sinus node dysfunction?

A

if symptomatic

580
Q

what % of maternal lupus end up for complete heart block?

A

5%

581
Q

class I indications for pacing with CHD?

A

symptoms, 7 days post-op,

congenital heart block with 1) wide QRS or dysfunction or 2) V rate < 50 (<70 in CHD)

582
Q

what is PVARP and why is it used

A

prevents atrial sensing of far field V (QRS), farfield T, or retrograde P. set time where atrial sensing DOES NOT occur.

583
Q

atrial refreactory period in pacemakers, what two components and what should it be?

A

TARP = PVARP + AV interval. needs to be less than atrial rate (cycle length)

584
Q

what does putting a magnet on a ICD do?

A

stops ICD shocks, but no pacemaker capability

585
Q

evidence for resynchronization therapy?

A

2005, multicenter with 103 patients, EF increasd by 12%, some taken off transplant list.

586
Q

fetal brady with occasional tachy

A

LQT

587
Q

structural heart disease assocated with fetalb CHB

A

heterotaxy, AVCD, ccTGA

remember: high mortality with CHD and hydrops + CCHB

588
Q

risk of CHB mom with lupus and recurrence risk

A

5%

recurrents 15%

589
Q

timeline of CCHB conversion to 3rd degree?

A

16-28 weeks

590
Q

evidence for steoids in 1st, 2nd, 3rd degree CCHB?

A

3rd: no evidence:
2nd,1st, some benefit
1st: 50% spont resolution

591
Q

fetal bradycardia for CHB with HR 52:

A

treat terbutiline for < 55

592
Q

causes of 2irregular heart rhythm in fetus?

A

PAC, PVC, 2nd degree AV block is the tricky one

how to differentiate: is the PAC premature or not!

593
Q

risk for death with fetal tachycardia?

A

% of time in tachy: < 20%, you nare okay, > 50%, hydrops

594
Q

5 risks for death in CCHB?

A

structural, hydrops, HR < 55, decline in HR by >5, dysfunction

595
Q

fetal flutter, treatment?

A

sotolol

596
Q

fetal avrt, treatment?

A

digoxin, then flecainide

597
Q

dose for fetal digoxin

A

1-2 mg/kg LOAD

0.5 maintenance

598
Q

when should you skip digoxin when treating fetal AVRT?

A

hydrops -> go straight to flecainide

599
Q

side effects with tacro levels are high

A

irritability and tremulousness -> can even lead to seizures

600
Q

which meds are notorious for increasing tacro levels?

A

antifungal meds -> also amiodarone and Ca++ channel blockers

601
Q

MLL2

A

kabuki syndrome (MuLan-Like)

602
Q

kabuki syndrome gene to know

A

MLL (MuLan-Like)

603
Q

echo best resolution

A

axial > lateral > elevational

604
Q

angle maximum on doppler to know?

A

20 degrees (cos theta = 0.94

605
Q

when does simplified bernouli not work and what are examples?

A

V1 not small: (stenoses in series like sub and supra valvar AS)
not measuring mean or peak differences (inertial component)
viscous energy losses not negligible (long narrow vessels (hypoplastic aorta, BT shunt) -> delta P underestimated

606
Q

which is bigger, peak instantaneous gradient or peak instantaneous gradient?

A

PIG > PTP

607
Q

4 components to load dependency of measuring systolic function

A

1) preload: increases shortenting
2) afterload: decreases shortending
3) contractility: changes in circumstance
4) heart rate: inc shortening via force frequency relationship

608
Q

what is vcf?

A

velocity of circumferencial fiber shortening
VCF = shortening fraction / ejection time
corrected VSD = (SF/ET)/sqrt(RR)
insensitive to preload (ejection time decreases with dec preload)

609
Q

what is dyskinesis?

A

ventricular muscle moves in the wrong direction

610
Q

where do you measure deceleration time?

A

downslope time of the E wave

611
Q

diastolic dysfunction, why look at pulm veins?

A

PV a wave duration -> should be LESS THAN the mitral valve inflow A eave

612
Q

E’, what cuoff to look for

A

10: anything less than 10 is abnormal

613
Q

how can you tell pseudonormmalization from normal in diastolic dysfn?

A

pseudo has a E’ < 10, E: E’>10, PV a waive is > MV a wave

614
Q

agitated saline intracardiac or intrapulmonary?

A

<3-5 beats

>5 beats (or if you see the pulm vein)

615
Q

loc eustacean valve

A

ANTERIOR aspect of IVC/RA junction

616
Q

recurrence risk of CHD with parent or sibling with CHD?

A

2-5%, LV obstruction is 8%

617
Q

HLHS recurrence risk?

A

8%

618
Q

which is riskier for a fetus with CHD? maternal or paternal HCD?

A

Meternal 3-7, paternal 2-3

619
Q

when is maternal phenylketonuria risky for CHD?

A

If poorly controlled P level >15: 10-15 increased

P <6: no risk

620
Q

does warfarin cause CHD?

A

no

621
Q

maternal Rubella assocated with (2)

A

persistent PDA and branch PA stenosis/hypoplasia

622
Q

risk of CHD with assisted reproduction technology?

A

1-3% risk, mostly septal defects (confounded by maternal age, twinning, etiology of infertility)

623
Q

fetabl bradycardia, risk of CHD
fetabl tachycardia, risk of CHD
fetabl irregular rhythm, risk of CHD

A

brady: 50%

tachyc and irregular < 1

624
Q

GI abnormalitiesm on obstetrical ultrasound will they have CHD?

A

20-30%

625
Q

what is nuchal translucency assocated with?

A

aneuploidy, notmal < 3, > 6 high risk of CHD

626
Q

what does absent ductus venosus do?

A

loss of sphincter, causes high output through venous chamber and heart failure

627
Q

define hydrops?

A

fluid accumulation in >= 2 compartmentss: pleural, pericardial, skin, abd, placenta

628
Q

umbilical vein doppler, what whould it look lie, what is abnormal?

A

should be flat, single pulsation is bad, double pulsation is really bad.

629
Q

normal ductus venosus pattern, change in disease state?

A

Norma: a wave flow not reversed
abnormal: (with anomolies, growth restriction) reduced, absent, reversed flow in atrial systole

630
Q

whats does umbilical artery flow tell you?

A

placental vascular resistance: A goes below baseline with worse resistance

631
Q

fetal Left to Right PFO flow?

A

L heart disease

632
Q

fetal retrograde arch flow

A

left heart disease

633
Q

MRI technique for imaging of constrictive pericardium?

A

spin echo for black blood, will be dark

634
Q

regurgitant fraction cutoffs for pulmonary regurgitation in MR?

A 
RF = retrograde volume / antegrade volume 
<20 = mild
20-40 = moderate
>40 = severe
635
Q

what things aren’t safe in the CMR magnet? which are?

A

prosthetic valves and steral wires safe, nonferromagnetic devices safe
wekaly ferromagnetic devices - wait 6 weeks
standard Pacemaker/ICD = unsafe
MRI compatible, conditionally safe

636
Q

Nuclear imaging: how dose a lung scan work?

A

tecnetium label Albumin sphreses get stuck in capillary bed, gives you relative R/L flow
doesn’t work with streaming, fontans

637
Q

which nuclea scan is used for Left to right shunt? right to left shunts?

A

Left to right: DTPA passes through capillaries, measure first pass scintigraphy and look for early recirculation
right to left: Albumin technetium, gets stuck in capillaries, compare density of body to lungs

638
Q

limitations of thermodilution

A

shunts, TR, PI, ery low CO, unstable core temp

639
Q

Fixk Eq:

Fick eq under 100% oxygen

A

Q = VO2 / [hgb x 13.6 x (high - low)]

denominator becomes Hgb x 13.6 x sat x PO2 x 0.03 - other

640
Q

what is effective Qp?

A

flow of deoxigenated blood that goes through the lungs

641
Q

In adults, what adult ASCVD score is significant, what would you do?

A

ASVCD score > 7.5 % -> start a statin

642
Q

in which population is BNP a prognostic indicator?

A

heart failure

Eisenmenger: BNP >140 poor outcomes

643
Q 
pulm valvuloplasty
Class I indications
Class III indications
predictions of good result
balloon size
risks
A

Class 1: critical, peak > 40, peak < 40 with rv dysfn, symptoms, shunt cyanosis
Class 3: PA/IVS with RV dep cor sinusoids
predictions : discrete valvar, thing valve, post-stenotic MPA dilation
balloon size: 1.2-1.4
risks: PI, perforation, annular disruptions suicide RV if subpulmonary narrowing

644
Q

tretment for suicide RV

A

PGE, beta blocker, RVOT stent

645
Q

when to do pulm valve eprforation:

A

TV Z score -2 o 0, tripartite RV, shot segment Pulm Atresia, no RV dependent coronary snusoids

646
Q 
aortic valvuloplasty
Class I indications
Class III indications
predictions of good result
balloon size
risks
A

Class I: critical, peak grad > 50, Peak grad > 40 with symptoms (syncope, angina), ST segment
Class IIb: >40 and desires pregnancy or sports
predictions: age < 3 months, > 40 years, undersize balloon, high gradient pre-dilation, coarctation
risk: AI
balloon size 0.8 - 1 of annulus

647
Q

mitral valvuloplasty
class I indication
class III
best outcome in

A

Class I: trheumatic heart disease severe (peak > 20, mean > 15) with a) symptoms or b) Pulm HTN
III: supra-mitral ring, or hypoplastic LV, duh
best outcome in Rheumatic heart disease, commisural fusion, balanced chordal attachemnets

648
Q 
branch PA angioplasty:
class I
poorly responsive 
balloon size
goals:
A

class I: peripheral branch PA stenosis with peak> 20 mmHg, RVP > 1/2 systemic, flow discrpancy <35%, angiographic narrowing
poorly responsive in congenital rubella, williams, alagille
balloon size 1) 2.5-3.5 x stenosis 2) <2 x the proximal and distal diameter

649
Q

branch PA stent
class I indication
method
risk

A

Class I: significant branch PA stenosis that can be dialted to adult size (delta P > 20mmHg, RV>1/2 systemic, flow < 35%, angio)

metho: no ballooning befre, risk of vessel rupture
risk: reperfusion injury

650
Q

pulm vein stenting
class I indications
risk:
best outcome

A

class I: acquired PV stenosis after RF ablation, lung transplant, external compression
all congenital stuff is class 2
risk: re-stenosis common
best outcome: stent diameter 6-10 mm

651
Q

coarctation angioplasty
class I indications
tech, what size balloon?

A 
class I: RECOARCTATION gradient > 20 mmHg, gradinet < 20 mmHg with collateralization, univentricular, or ventricular dysfunction
balloon: 2-3 x stenosis, <=1 mm larger than proximal or distal vessel
652
Q 
coarctation stent: 
class I indication
A

recurrent coarctation > 20 mmHg and can be expanded to adult size.

653
Q

can you stent a coarctation in a teenager?

A

there is no class I indication to stent native coarctation. stenting antive coactation is class IIa or IIB and only if therit can be dialted to adult size

654
Q

risk of complication in re-coarctation intervenion?

A

Balloon (44%)&raquo_space;> surgery (25%) > stent (5%)

655
Q 
RV-PA conduit stenting
class I indications
A

Significant stenosis with

a) stent will prolong the lfie of the conduit
b) PI will be tolerated
c) stent will not impinge the PA bifurcation or coronaries

656
Q

percutaneous pulmonary valve

“Class I indications”

A 
use within conduits or bioprosthetic that are > 16 mm
not native or patch augmented RVOT
   moderate+ regurgitation 
   OR
   mean RVOT gradient > 35 mmHg
657
Q

atrial septostomy
class I indications
technical considerations

A 
class I: 1) atrial mixing or LA egress 2) ECMO decompression
tech: balloon in newborns with thin atrial septum
IF thick or intact septum, risk of PV avulsion, do stenting
658
Q 
ASD device closure
class I indications
A 
class I: 
hemodynamically significant: R volume overload, exercise, Rt heart failure, Qp:Qs>1.5
suitable: secundum, >15 kg, 5mm rims (OK if ant-sup deficient and post-inf 2-3 mm)
659
Q

device closure with absence of superior or posterior rim?

A

no concern for sinus venosus defect

anteroinferior = risk of primum

660
Q

device closure with absence of anteroinferior rim?

A

anteroinferior = risk of primum

absent superior or posterior: concern for sinus venosus defect

661
Q

which rims may be deficient in ASD device closure?

A

anterosuperior (aortic)
posteroinferior must be > 2-3 mm
all other must be > 5 mm

662
Q

risk of erosion in ASD device clousre?

A

deficient anteriosuperiro rim (overall risk 0.1%)

other factors: device over-sizing, splaying, movement

663
Q

s/p ASD closure, tamponade, what causes it?

A

erosion

664
Q

s/p ASD closure, at 1 week develops headache, rash, fever

A

nickel allergy
occurs 2d-1m
headache, rash, dyspnea, fever, effusion
medical management of allergy

665
Q

do you give aspirin after ASD closure?

A

yes ASpirin and SBE x 6m

666
Q

PDA closure class I indications?

A

moderate-large with heart failure, FTT, pulm overcitculation, LA/LV dilation

667
Q

subtypes of PDA A-E

A 
A = conical (an A is a cone)
B = window (a B looks like a window)
C = Tubulare ( a C looks like a tubular crashign wave)
D = saccular (D looks like a backpack or sack)
E = Elongated
668
Q

when to use coils to close a PDA?

A

< 2.5 mm diameter

669
Q

how long to use aspirain after PDA device?

A

trick, no aspirin becaue you want it to clot

670
Q

lenth of SBE prophylaxis following any device cath implantation?

A

just say 6 months

671
Q

length of Aspirin therapy post stent, device, or pulm valve?

A

device or stent: 6 months
PDA device: no aspirin
pulm vvalve: indefinitely

672
Q

treatment for suicide RV?

A

PGE
beta blocker
RVOT stent

673
Q

favorable anatomy for pulmonary perforation?

A

normal TV size (or mildly hypoplastic)

absence of RVDCC

674
Q

least common fusion in BAV?

A

NL = note likely (1%)
RL: really lkely (80%)
RN: not really (19%)

675
Q

aortic valvuloplasty makes moderate AI, what balloon size to try next?

A

TRICK! stop once moderate AI is created regardless of residual obstruction

676
Q

balloon size in Aortic valvuloplasty?

A

0.8-1 x annulus

677
Q

risk of suboptimal result in aortic valuoplasty?

A

< 3 months, > 40 years
undersized baloon, higher pre-dilation gradient, smaller annular Z score, valve calcifivation, unrepaired coarctation
prior procedure

678
Q

when is branch PA angioplasty less likely to be successfu?

A

congenital rubella
williams syndrome
Alagille syndrome

679
Q

long term success following branch PA dilation?

A

Rarely produces complete permanent resolution of obstruction

680
Q

patient s/p branch PA balloon and stenting, tachypnea and cough?

A

reperfusion injury
will find focal pulm edema on CXR
treat with diuretics, oxygen and time

681
Q

difference in heart failure in coarctation presentation: early vs. late what do you see on echo?

A

early: RV dilation
late: LV dysfunction, dilation

682
Q

neonate with sepsis

A

systemic blood flow obstruction

683
Q

acute increase in afterload, what does the PV loop do?

A

narrow and tall PV loop (dec stroke volue, increase pressure)
in chronic, hypertrophy develops, normalizes wall stress, loop widens slightly and moves to the left (end systolic volume decreases over time)

684
Q

chronic increase in afterload, what does the PV loop do?

A

in acute, PV loop becomes narrow and tall.

685
Q

box shaped heart on CXR

A

ebstein’s

686
Q

ebsteins displacement index, what is it?

A

> 8mm/m2

687
Q

what is celermajer index?

A 
[RA + aRV] / [RV+LA+ LV]
<0.5: no risk = normal
.5-1: 10%
1-1.5: 50-100%
>1.5: 100% risk of death
how much of the RA gets put in the "Cellar"
688
Q

which lesions is at risk for cirular shunt?

A

ebsteins, if you have PI:

PDA -> PI -> TR -> RA -> PFO -> LA, LV, Ao, PDA

689
Q

tet spell treatmen

A

sedation, preload, beta blockade, phenylephrine

690
Q

BAV, aortic stenosis and coarctation genes

A 
NOTCH 1 (notch in the posterior aorta)
SMAD6 (super mad 6 day old)
691
Q

NOTCH 1 - gene puts you at risk for

A

BAV, aortic stenosis and coarctation genes
NOTCH 1 (notch in the posterior aorta)
SMAD6 (super mad 6 day old)

692
Q

SMAD6 - gene puts you at risk for

A

BAV, aortic stenosis and coarctation genes
NOTCH 1 (notch in the posterior aorta)
SMAD6 (super mad 6 day old)

693
Q

myosin heavy chain 11 mutation, put you at risk for which CHD?

A

PDA
Aortic aneurysm
11 looks like a vessel wall | |

694
Q

ankyrin repeat domain mutation

A

TAPVR
ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)

695
Q

platelet-derived growth factor receptor alpha mutation

A

TAPVR
ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)

696
Q

TPAVR genes

A

ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)

697
Q

PA/IVS, most important determinant, what cutoffs?

A

TV > -2.5: 2 ventricle

TV

698
Q

DILV, most common aortic position?

A

left and anterior
Conduction system
- L looped: anterior to pulmonary outflow tract
- D looped RV: lateral to pulmonary outflow tract

699
Q

cutoff for a small bulboventricular foramen?

A

<2cm2/m2 -> risk for late obstruction to outflow tract arising from BVF

700
Q

<2cm2/m2

A

<2cm2/m2 -> risk for late obstruction to outflow tract arising from BVF

701
Q

0.8-1

A

pulm balloon size

702
Q 
SVR trial
# patients and time frame
1 year survival
3 year surival
6 year survival
A

patients and time frame: 549 (2005-2009)
1 year survival (better RVPA shunt 74 x 64)
3 year surival (no survival advantage (67 vs 61)
- RVPAS worse RV EF, more catheter interventions
6 year survival
- no survival advantage (64% vs. 59%)

703
Q

enalapril in Infants with SINGLE Ventricle
ISV trial -
outcome?

A

administratio of enalapril did not improve somatic growth, ventricular function or heart failure severity

704
Q

normal fontan by the numbers

A 
PA pressure < 15 mmHg
TPG < 10 (normal 5-8)
sat 90+/- 5
SVC = IVC = Fontan = RPA = LPA
no AVV regurg
no systemic in/outflow obstruction
normal function
Sinus rhythm
705
Q

FOntan IART, who is more likely to have it?

A

atriopulmonary&raquo_space; ECC

706
Q

5 year survival in PLE?

A

88%

707
Q

treatment of PLE

A 
If high Fontan pressure -> sildenafil, fene spirnolactone
If EF < 55%: digoxin, ACE/ARB, spironolactone
low fat diet
MCT
budesonide
octreotide
heparin
zinc
albumin
diuretics
anmiea
thyroid
708
Q

In a VSD, what are the neurohormonal changes?

A

chamber distention -> sympatheticoadrenal stimulation
splancnic, renal, peripheral vasoconsitrction -> INc SVR -> inc L to R shunt
myocardial beta receptor -> tachycardia
renain -> Na retention
vasopressin -> water retention

709
Q

NKX2.5

A

ASD and conduction defects

Nick’s disease

710
Q

GATA mutations

A

ASD association

Goetta is in the shape of a circle, so is an ASD

711
Q

left hand bubble study, fills LA

A

unroofed coronary sinus

712
Q

define partial AVSD

A

partial: primum +/- cleft (ASD physiology)
transitional: restrictive VSD (ASD physiology)
Intermediate: tongue of tissue across AVVs making 2 orifice (ASD+VSD physiology)
complete: 1 AVV (ASD + VSD physiology)

713
Q

define transitional AVSD

A

partial: primum +/- cleft (ASD physiology)
transitional: restrictive VSD (ASD physiology)
Intermediate: tongue of tissue across AVVs making 2 orifice (ASD+VSD physiology)
complete: 1 AVV (ASD + VSD physiology)

714
Q

define intermediate AVSD

A

partial: primum +/- cleft (ASD physiology)
transitional: restrictive VSD (ASD physiology)
Intermediate: tongue of tissue across AVVs making 2 orifice (ASD+VSD physiology)
complete: 1 AVV (ASD + VSD physiology)

715
Q

define complete AVSD

A

partial: primum +/- cleft (ASD physiology)
transitional: restrictive VSD (ASD physiology)
Intermediate: tongue of tissue across AVVs making 2 orifice (ASD+VSD physiology)
complete: 1 AVV (ASD + VSD physiology)

716
Q

when do you surgerize a partial AVSD?

A

elective > 1 year

717
Q

when does the PDA close
term
preterm
<1000g

A

term: 12 hrs (ligamentum by 2 weeks)
preterm: 1/3 have a PDA, lower BW inc risk
<1000g: sponteous closure by day7 in 1/3

718
Q

which is better for closing a PDA, ibuprofen or indomethacin?

A

same efficacy

indomethacin associated with renal disease

719
Q

3 typs of AP windows

A

type I: proximal
type II: distal
type III: both (includes proximal branch PAs as well)

720
Q

AP window associated with which IAA?

A

Type A = Berry Syndrome

721
Q

Berry syndrome

A

AP window and IAA type A

722
Q

which emb arches disappear?

A

1,2,5

723
Q

normal right 4th aortic arch gives risk to?

A

3rd: CARotid
4th Rt SCA , leftaortich arch
6th: Ductus

724
Q

double aortic arch, which is more likely dominant

A

Rt: 70%, lt 20%, codom 10%

descending aortia typicall opoosite to dominant arch

725
Q

what is a cervical arch?

A

left 3rd arch persists and both 4th arches regress
pulsatile neck mass
rt ductus results in ring
aortic obstruction due to redundancy

726
Q

pulsatile neck mass

A

concern for cervical arch
left 3rd arch persists and both 4th arches regress
rt ductus results in ring
aortic obstruction due to redundancy

727
Q

barium esophagram: ant on trach, post on eoph

A

Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
aber subclav: posterior on esophagus
PA sling: anterior compression of esophagus

728
Q

barium esophagram: ant on trach only

A

Innominate art: ant on trach
Double AoA: ant on trach, post on eoph
aber subclav: posterior on esophagus
PA sling: anterior compression of esophagus

729
Q

barium esophagram: posterior on esophagus

A

aber subclav: posterior on esophagus
Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
PA sling: anterior compression of esophagus

730
Q

barium esophagram: anterior compression of esophagus

A

PA sling: anterior compression of esophagus
Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
aber subclav: posterior on esophagus

731
Q

when do you replace an aneurysmal aorta in Marfans?

A

Marfans: >50mm, >45 mm if risk factors (>3mm/yr, preg, fam Hx dissection, severe AR), otherwise
BAV: >55, >50 if risk factors, >45 if mixed aortic valve disease
LDS: >42mm TEE > 44mm CT.MRI
Turner: 40-50 mm, CSA area/ht > 10cm2/m

732
Q

when do you replace an aneurysmal aorta in BAV?

A

Marfans: >50mm, >45 mm if risk factors (>3mm/yr, preg, fam Hx dissection, severe AR), otherwise
BAV: >55, >50 if risk factors, >45 if mixed aortic valve disease
LDS: >42mm TEE > 44mm CT.MRI
Turner: 40-50 mm, CSA area/ht > 10cm2/m

733
Q

when do you replace an aneurysmal aorta in LDS?

A

Marfans: >50mm, >45 mm if risk factors (>3mm/yr, preg, fam Hx dissection, severe AR), otherwise
BAV: >55, >50 if risk factors, >45 if mixed aortic valve disease
LDS: >42mm TEE > 44mm CT.MRI
Turner: 40-50 mm, CSA area/ht > 10cm2/m

734
Q

when do you replace an aneurysmal aorta in Turner syndrome?

A

Marfans: >50mm, >45 mm if risk factors (>3mm/yr, preg, fam Hx dissection, severe AR), otherwise
BAV: >55, >50 if risk factors, >45 if mixed aortic valve disease
LDS: >42mm TEE > 44mm CT.MRI
Turner: 40-50 mm, CSA area/ht > 10cm2/m

735
Q

CPR: goal coronary perfusion pressure

A

goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes

736
Q

CPR: goal arterial diastolic pressure

A

goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes

737
Q

CPR: goal ETCO2

A

goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes

738
Q

CPR: goal defibrillation time

A

goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes

739
Q

NEC risks (2)

A

< 30 days of age, ductal dependency

740
Q

post op - bleed with low fibrinogen: treatment

A

low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical

741
Q

post op - bleed with deficient clotting factors, treatment?

A

low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical

742
Q

post op - bleed with normal coags, treatment?

A

low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical

743
Q

post-pericardiotomy syndrome, when does it happen?
what lesions?
symptoms?
treatment?

A 
1 week after surgery (peak day 10)
ASD< VSD, TOF< OHT
chest pain, malaise, arthralgias
echo: effusion (tamponade rare)
Treament: diuretics, NSAID, ASA, no steroirds, no colchicine
744
Q

fontan, what PEEP to use?

A

3-5 mmHg

745
Q

if unable to extubate a fotna, post-op, what to consider?

A

VV collaterals

746
Q

intubated glenn, what strategy to increase sats?

A

slightly inc PA CO2 by hypoventilation to inc cerebral blood flow and inc Pulm blood flow

747
Q

definition of pulsus paradoxus?

A

> 10 mmHg drop in SBP with inspiration ina spontaneously breathing person

748
Q

risk of being on both digoxin and lasix?

A

hypokalemia from lasix makes digoxin tricky

749
Q

risk of lasix and NSAID?

A

NSAID makes lasix less effective

750
Q

long term risks of lasix?

A

ototox, nephrocalcinosis in neonates

751
Q

bioavailability of thiazide?

A

20%

752
Q

med to correct secondary metabolic alkalosis?

A

acetazolamide: prox convoluted tubule

753
Q

risks of carbonic anhydrase inhibitor?

A

sulfonamide reaction: SJS, TEN, hepatic necrosis, bone marrow suppresion

electrolyte abnormalities

754
Q

PRIMACORP study:
what endpoint was improved with milrinone
by how much?

A

prevention of LCOS or death in first 36 hours post-op
placebo 27%
low dose 18%
high dose 9%

755
Q

mechanisms of nitroprisside?

A

direct vasodilation and nitric oxide donor

756
Q

patient with metabolic acidosis and normal mixed venous O2?

A

nitroprusside -> cyanide poisoning caused by tissue hypoxia (cytochrome oxidase inhibition = mitochondria poison)

Can happen with liver disease

757
Q

risk of ARB and aldactone?

A

hyperkalemia

758
Q

calcium channel blocker for a fib a flutter?

A

diltiazem (rate control)

759
Q

drug of choice for HTN after coarctation repair?

A

propranolol: HTN from carotid baroreceptors, gets to the source

760
Q

slows and strengthens the heart

A

digoxin

761
Q

what drugs reduce clearance of digoxin?

A 
amiodarone 
Ca channel blockers (verapamil)
flecainide
propafenone
quinidine
762
Q

scooped ST segments

A

digoxin toxicity (When you DIG, you SCOOP out the ST wave)

Treatment:
stop digoxin, treat hypokalemia, atropine for brady, esmolol, lidocaine for ventricular arrythmia
DIGIBIND

763
Q

what is nesiritide?

A

peptide and augments guan cylase receptor, inc cGMP, smooth muslce relaxation, dec BP and muscle relax

764
Q

what is fenolopam?

A

potent DOPA-1 receptor agonist, augments urine output

765
Q

downstream signaling of beta-adrenergic

A

beta: Adenalate cyclase (ABC) -> increase cAMP 1) Ca channels for inotropy and chronotropy 2) protein kinase for vasodilation
alpha: G protein -> phospholipase makes DAG and IP3 –> increase Ca++ -> vasoConstriction

766
Q

downstream signaling of alpha-adrenergic

A

beta: Adenalate cyclase (ABC) -> increase cAMP [myocardial] Ca channels for inotropy and chronotropy [vascular] myosin light chain protein kinase for vasodilation
alpha: G protein -> phospholipase makes DAG and IP3 –> increase Ca++ -> vasoConstriction

767
Q

MOA Etomidate

caution with

A

potentiates gaba receptors
Caution: adrenal suppression -> blocks cortisol production pathway
may potentiate seizures in patients with known disorder

768
Q

hemodynamic effects of inhaled anesthetics:
airway effects?
danger?

A

vasodilation causes dec BP, reflex tachycardia

airway: bronchodilation
danger: malignant hyperthermia

769
Q

mechanism of malignant hyperthermia

treatment?

A

mech: block regulatory effect o Mg on sarc ret, Ca++ release -> inc VO2, inc Co2 (autodomal dominant on 19)
treat 1) discontinue 2) hyperventilate 3) dantrolene, cooling

770
Q

mech difference for roc/vec and succinylcholine?

A

succinylcholine is depolarizing and can cause rhaobdo, bradycardia, hyperthermia, hyperkalemia
roc/vec are nondepolarizing (bind choolinergic receptor)

771
Q

what is protamine reaction?

A

bradycardia, hypotension, anaphylaxis that occurs after protamine is given (see it after surgery with protamine reversal)

772
Q

LMWH PPx and Tx doses?

A

PPx 0.5 mg/kg/dose q12

Tx: start 1mg/kg/dose, then titrate

773
Q

warfarin inhibits

A

2,7,9,10, C, S

774
Q

how does TPA work?

A

binds fibrin, activates plasminogen which breaks down fibrin

775
Q

Mech clopidogrel?

A

irreversible blocks GPIIb/IIIa (ADP receptor), inhibits platelet aggregation

776
Q

what is RER, why does it increase suddenly at peak exercise?

A

RER = VCO2 / VO2

as lactate production increases, VCO2 is produces as buffering of HCO3

777
Q

noninvasive surrugate of lactate threshold?

A

Ventilatory anaerobic threshold

778
Q

VO2 =

A

VO2 = CO * oxygen content

= (HR * SV) * (a - v o2 diff)

779
Q

when does stroke volume plateau in exercise?

A

40% of peak, then HR keeps going up

780
Q

why does A-a O2 difference widen with increasing exercise?

A

Hgb dissociation: heat, acidosis, etc

781
Q

equation for breathing reserve

A

(1 - max VE / MVV) * 100%

MVV calculated by hyperventilating for 10 seconds, multiple by 6

782
Q

normal work rate during exercise?

A

3 watts/ kg for boys/girls

3.5 watts/kg for post-puberscent boys

783
Q

normal BP response to exercise

A

SBP increases up to 40%
abnormal if it is blunted (< 20 mmHg) or falls
DBP should stable or fall

784
Q

exercise recommendation for toddlers
for children
for adolescents

A

toddlers: > 3 hours/day

children/adolescent: > 1 hour

785
Q

indications to restrict from exercise
1A
1A+1B

A

1A only: Pulm HTN, severe AS, EF < 40%
1A or 1B: severe PS/PI, RV dilation
1A, 1B, 2A: moderate AS

numbers are static, letters are dynamic

786
Q

which wave corresponds to RVEDP in absence of TC stenosis?

A

a wave
(same with a wave of LA)
however pressure tracing in LA is v wave dominant (closed system)

787
Q

what causes RA a wave to be higher? v wave to be higher?

A

a wave: atrium contracting against closed TV (block), stenosis (partially closed), RV restriction (functionally closed)
v wave = Volume V stuff, TR, vein of galen, ASD, PAPVR, LV-RA shunt

788
Q

max contrast dose

A

6cc/kg

789
Q

what is the hepatoclavicular view on angiography? what is it good for? echo analogy?

A

45 LAO, 45 cranial
good for, crux of the heart, avv anatomy,
analogoes to apical 4 chamber view

790
Q

rate of culture negative IE in kids? which organism?

A

5%, bartonella

791
Q

how long does dental bacteremia last?

A

30-60 minutes

cardiology testing 123 (10 decks)

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