Board Review Mix Flashcards
MC 2 gene HCM
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
MYH7
MC gene HCM (myosin heavy chain) MY Heavy Sevey
MC cause SCD athletes adolescent
HCM (about half)
indications ICD placement in HCM (6)
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
septal thickness to Dx HCM
> 15mm = 1.6 cm (think 16mm)
OR >13 mm with appropriate family history
traditional indexed PVR cutoff for HTx?
PVRI<6
doxyrubicin max dose
300 mg/m2
thiazide diuretic side effects that separate it from loops
hyperglycemia, hyperlipidemia
digoxin drug-drug interaction
amiodarone (less common beta blocker)
amio drug drug interaction
digoxin
ACE cough cause?
blocks breakdown of bradykinin
types of calcineurin inhibitors
tacrolimus and cyclosporin
immunosuppresant antimetabolite
cellcept = mycophenolate (inhibits purine synthesis in lymphocytes) imuran = azothioprine (same, is precursor to 6-MP)
side effect ATG
thrombocytopenia (also fever chills)
side effects tacro
nephro, HKalemia, Sz, glucose intolerance, l’il more PTLD
side effects cyclosporine
gum overgrowth, hair growth, little more rejection
typical Immunesupprasant combo
calcineurin and cellcept (tacro and cellcept)
MOA sirolimus
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
side effect cellcept
also with imuran GI distress and leukopenia (which is the whole point)
investigator intentionally misleads participants
deception (acceptible under only specific instances)
investigator witholds information about some aspect of research
incomplete disclosure (acceptible under only specific instances)
SFI (significatn Financial Interest) (4)
Renumeration >$5000 from an entity, holds equity interest, IP rights, reimbursement
making up data
fabrication
manipulate research process to get
falsification
using someone elses ideas/words
plagerism
investigator makes mistake because he is human
honest error (not research misconduct)
dispute about elements of study, stats
difference of opinion (not research misconduct)
belmont report written why and why?
1974, tuskegee syphilis study
3 principles of belmont report
respect for persons, beneficence justice
what is clinical equipoise
needed for a randomized study to continue. may only continue if uncertain that one therapy is better than another
uncertainty whether one therapy is better than another
clinical equipoise
research subject does not understand purpose of research
therapeutic misconception
threat to get subject to enroll in a study
coersion
when does a child become an adult?
depends on the state, check local law
when can you do a study involving study with greater than minimal risk?
1)direct benefit or 2) generalizable knowledge
cardiac crescent is from what turns into what
mesoderm, turns in pprimary and 2nd hear tube
primary heart tube is from what, turns into what
from cardiac cresent, turns into atrium and embryonic ventricle
atrial appendage forms from
primative atrium, from primary heart field, from cardiac crescent, from mesoderm
LV forms from
embryonic ventricle, from primary heart field, from cardiac crescent (mesoderm)
bulbus comes from
secondary heart field, from cardiac crescent
bulus cordis turns into
RV, conus, outflow tract
RV comes from
proxmal bulbus cordis, from seconadry hear tfield, from cardiac crescent
conus come from
mid bulbus cordis, from seconadry hear tfield, from cardiac crescent
truncoartic sac comes from
distal bulbus, from seconadry heart field,
outflow tract derived from
bulbus cordis AND neural crest cells
cardiac crescent and heart tube derived from
creschent, mesoderm ; heart tube endo and meso
time of V looping
d22-27 (think of a milenial “figuring themselves out” in mid-20s
cardiac timeline in terms of growing up
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)
time of V septum closure and outflow tract closure
VSD 6 weeks (6 looks like a VSD), outflow 6-8 (8 looks like a PA and aorta divided)
straddlign of AVV through which VSD
TV through inlet VSD, MV through malaligned VSD
which arch becomes innominate artery
contralateral 4th
association RAA with ab left SC artery?
22q11
normal arch, what involutes
right dorsal aortic arch, right 6th
RAA arch w/ mirror, what involutes
left dorsal aortic arch, right 6th (left 6th usual stay around, but is not a ring)
findings of left atrium
CS bosy, pect muscles stay in appendage, thin primary septum on the left side
findings of the RA
pect muscles go to free wall, CS os, Limbus, thick septum secundum
RAA with ab L subclav, what involutes?
L 4th involutes, if left ducuts, ring
MC rings
doubale Ao arch, RAA with ab L subclavian
Type A IAA associated with?
AP window
normal ear sat, low arm and leg sat, Dx?
type B IAA with ab R subclavian A, both SC come from ductus, both carotids come from asc aorta
4 weak pulses, low RA and LA sat, normal ear sat
type B IAA with ab R subclavian A, both SC come from ductus, both carotids come from asc aorta
type B IAA with ab R subclavian A, sats?
carotids normal, subclavians low
anterior indentation on esophagram
LPA sling (only to go between trachea and esophagus)
vein embryology: R horn sinus venosus becomes
joins into RA
vein embryology: L horn sinus venosus becomes
coronary sinus
vein embryology: R cardinal vein becomes
SVC (male cardinal flies down)
vein embryology: L cardinal vein becomes
involutes, or LSVC to CS (female cardinal redness involvules)
venous structure from abnormal LA egress
levoatrial cardinal vein (not PAPVR, this is a decompressing vein from LUPV to innom vein)
Fetal % RV:LV
RV:LV 60:40
Fetal % RV flow
2/3 goes out ductus to the placenta (low resistance), < 1/3 goes to lungs = 20% total CO
% total fetal CO through isthmus
5-10% through isthmus, most LV output goes to the head and neck
left heart obstruction in fetal, what effect
low O2 delivery to brain, retrograde ductus
TGA in fetal, what effect
low O2 delivery to brain and coronary arteries
fetal myocardium differences
immature sarc ret, fewer contractile, dependent on HR, sensitive to change in afterload
metabolic demand change upon birth, cardiac output change
3fold inc in metabolism (temp regulation, breathing), CO increases by 50%
effect of cyanosis and increased pulmonary blood flow
very delayed drop in pulmonary vascular resistance (TGA/VSD)
highest risk for delayed drop in pulmonary vascular resistance
cyanosis with high pulmonary blood flow (TGA/VSD)
electron microscopy: sarcomere
z disc to z disc (Zarcomere)
troponin C vs. troponin I
C bind calcium, conformational change, I Inhibits by covering actin binding spot
thin vs. thick filament, which protein
thin = Actin, thick = myosin (M is thicker than A), myosin pulls the actin when ATP and Ca are available
how does calcium get into the cell
voltage gated L type Ca channel, Ca influx activates ryanodine receptor on the Sarc Ret
what pumps calcium away from myosin?
SERCA2 in sarcoplasmic reticulum (inhibited by phospholamban during systole LAMB = calm and docile), enhances diastolic function
how does milrinone work?
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
what does Titan do?
passive stiffness of sarcomere, binds thin filament to actinin ( Z line)
2 ways that sarcomere is connected to cell membrane?
1) dystrophin glycoprotein complex (binds thin filament to ECM 2) titin to actinin (Z line) to Integrin on cell membrane
define length tension relationship
passive force generated by stretching a muscle
4 ways to alter PV loop to change Stroke volume
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
sympathetic nervous system effect on heart muscle
most from circulating catecholamines, adults have some B1 innervation
thyroid effect on heart
increase adrenergic receptors, inc mitochondria, inc myocyte proteins
what does endothelin do to pulm vasculature
vasoconstricts, of course (endothelin receptor antagonists)
effect of sildenafil, MOA?
inhibits PDE-5, decreased NO breakdown, increased cGMP, vasodilation
MOA endothelin
ET A and B receptors, potent vasocontriction AND smooth muslce proliferation
MOA prostacyclin
prostaglandin, cAMP, vasodilation
mechanism of hypoxic vasoconsitrction
mitochondrial sensor: increased oxygen species in electron trsansport chain, K channel opens, decreases voltage gated Ca influx, vasodilation
what do baroreceptors respond to? what do they do?
respond to mechanical stretch (pressure), decrease sympathetic output and inc vagal tone, dec HR, vasodilation
4 subdivisions of fetal CO and percentages?
30% brain, 10% isthmus, 40% ductus, 20% lungs
which electron microscopy lines of a sarcomere shorten?
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
4 types of AVM causes
HHT, Abernathy syndrome, hepatopulmonary Glenn
HHT mutations
ACVRL1 (A to V, R to L in 1 sec), ENG (endoglin, ENGineering makes you bleed yours eyes out)
Portal disease, vs. Hepatic disease, what effects on the lungs?
portal causes PAH, hepatic disease causes AVMs
how does RA hypertension lead to pulmonary edema?
impaired pulm lymphatic drainage
how does pulmonary edema cause weezing?
perivascular cuffing of airways by engorged vessels, causes obstructive defecr
asociation of PA sling?
complete tracheal rings (50%)
what is FRC?
volume of air left in the lungs at the end of tidal volume
what are the 2 main effects of respiration on blood pressure?
preload of RV, afterload of LV
effect of normal negative pressure expiration on CO
inspiration -> dec pleural pressure -> dec RA pressure, -> inc RV stroke volume -> inc CO
effect of normal negative pressure expiration on CO
inspiration -> inc pleural pressure -> inc RA pressure, -> dec RV stroke volume -> dec CO
Equation LV systolic transmural pressure
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
poiseuille equation
R proportional to viscocity * length / radius^4
normal PA - Pa gradient?
5mmHg
Alveolar gas equation
PAO2 = (Ptm - Po2) *FiO2 - PACO2/RQ
equation for oxygen content
CaO2 = SaO2 * Hgb * 1.34 +PaO2 * 0.003
What is a Libman-Sacks lesion?
endocarditis lesion in lupus, found on left sided valves MV>Ao Valve, 10% NEW DX LUPUS, IRREGULAR VEGETATIONS
lupus endocarditis
Libman-Sacks
L type calcium channels located on?
cell membrane (voLtage gated), ryanodine (Ca dependent) on the sarc ret
where and why is renin made?
kidney juxtaglomerular apparatus, in response to lowe kidney perfusion pressure
board andswer for pulmonary artery embryonic arch?
6th arch
Most common additional cardiac finding in tetralogy of fallot
ASD/PFO in 80% (pentalogy of fallot), RAA in 25%
MC coronary abnormality in D-TGA
circ off of RCA (16%, circle and circle), followed by single RCA, then inverted RCA and Cx (RCA off LAD, Circ off posterior)
space seen near LCA on echo
transverse sinus
where is ANP made and what does it do?
both atria in response to stretch. increases GFR by arteriolar glomerular effect, distal tubule sodium reabsorption is blocked
most reliable differentiating factor between MV and TV
TV offset apically at hinge point
when do you see left vs. right juxtaposition of atrial apendages?
left more common in complex heart lesions with abnormal VA connections, right in simpler lesions
MC great artery position in DORV
side-by-side = aorta right, PA left
what is mitral arcade
absent/abnormal chordal insertions
why is the second heart sound loud in TGA?
anterior aorta
absent pulmonary arteries in TOF vs. truncus?
in TOF, the absent PA is opposite the aortic arch. In truncus, the absent PA is on the same side as the arch
blood supply to AL and PM paps
AL from LAD and Circ, PM from right. AL is a Cheery round LAD. PaM is always right.
what is the thebesian valve and the vieussens valve?
Thebesian valve is located at the mouth of the CS. Vieussens is located where the cardiac vein merges with coronary sinus.
MC coronary abnormality in normal hearts?
Circ from the RCA
where do T tubules associate?
with the Z disc, continuation of sarcolemma
what is an intercalated disc?
junctions between myocytes, link myofibrils to the edge to transmit force from the sarcomere. LINK everything together
when is ATP used during the power stroke?
during the power stroke when the myosin bends, release of ADP allows the extension of the myosin head
indications for coarct repair (5)
- BP gradient > 20, 2. BP gradient <20mmHg with >50% narrowing at coarct level 3. hypertension 4. other cardiac lesions 5. elevated LVEDP
coarct repair from side or front?
all about the size of proximal arch, front if proximal arch small: -2 z score, 60% of asc ao, < weight in kg + 1
risk of subclavian flap
aneurysm
long term risks after coarct repair?
HTN in 30-90%, recoarct in 5-10%
long term complications after IAA repair?
LVOTO (30%), recoarct (8-30%)
1st 2nd most common coronary arrangements of D-TGA?
first is normal, 2nd is circ off the RCA. (circle and circle in paralell D-TGA)
4 options for TGA/LVOTO
1) switch with LVOT resection
2) Rastelli
3) Nikaidoh
4) REV
TGA/LVOTO surgery of choice if pulm valve very small
Nikaidoh (remember, risk of kinking CA, homer simpsons is big and walking around saying DOH!
risk of death after ASO, which factors?
abnl coronary pattern, multiple VSDs, older age
late complications of arterial switch
supravalvar PS, neo-aortic dilation, insufficiency
stage 1 treatment of chois if 1.5 kg
hybrid, then sano , then BTTS
10 yr survival after norwood
65%
Tri atresia, what are the types?
I - normal (70%) II - D transpo (30%) III - L transpo (<5%) A = atrtic or small PV B = balanced C = over Circulated
what is trussler’s rule?
how big is the inner diameter of a PA band? 21mm + weight (in kg)
when do people doa Mee shunt?
central PA shunt to asc aorta in MAPCAS or small PA
for the boards, best long term survival with which type fo fontan?
ECC
location of obstruction in supracardiac TAPVR? infracardiac
supra (25-50%):between left PA and L bronchus (vice) > vertical vein insertion
infra (99%): hepatic sinusoids, insertion to IVC, location of diaphragm
MC type of truncal valve (# cusps)
tri in 60, then quad
mortality of truncus IAA?
up to 50%
indications for AS surgery?
critical, asymptomatic >50 mmHg, symptomatic or ischemic ECG changes
which VSD most likely to cause AI?
juxta-arterial
location of conduction system in VSDs: perimembranous
inlet
muscular
doubly committed
perimembranous: inferior rim
inlet: inferior rim
muscular: less at risk
doubly committed: less at risk, caudal limb septal Y band
how do you handle the AVV in a one-patch repair of the AVSD?
incise them
rate of PV replacement for repaired tet at 20 years?
30%
taussig bing
TGA (TausiG) type DORV, subpulmonary VSD, risk of coarctation
TGA type DORV, subpulmonary VSD, risk of coarctation
taussig bing (TausiG) = TGA
gene for williams syndrome
elastin (ELN)
surgery for supra-valvar AS
Doty Y patch repair (or three patch)
indications for subas repair
40mmHg, symptoms, 50% if tunnel-like, or Aortic involvement with AI
what is modified in the modified konno?
resect septum and place VSD patch to make more room
cath indication for ASD closure?
QpQs 1.5:1, otherwise R dilation, lung diseases, R to L shunt
what determines the type of surgery in PAPVR?
height of the RUPV, if closure, do a intracardiac baffle from RUPV orifice to ASD. If high, do a Warden Procedure
which ASD repair has a high risk of sinus node dysfunction?
PAPVER repair with 2 patch repair
associated features of scimitar syndrome?
basic disease is R PVs go to the IVC/RA junction, associated:
AP collaterals to right lower lobe
right lung hypoplasia
+/- right diaphragmatic hernia
what is dysphagia lusoria?
LAA with ab right subclavian
what is innominate artery syndorme and how to you fix it?
innominate artery compresses the trachea anteriorly, fix by aortopexy or innominate artery reimplantation
what is the Takeuchi repair?
repair of ALCAPA, baffle from coronary OS through the PA to a window between the aorta and the PA
definition of RV-dependent coronary circulation?
myo perfusion from RV fistulae from obstruction of 2+ coronary branches OR ostial atresia
MC types of IE organisms
staph 43%, strep 40%, then beta hemolitic strep, enterococcus hacek
strep IE associations vs. staph
staph is acute, CHD< prosthetic, catheters, newborns (also candida). strep is subacute, non-chd, >1
candida IE
newborns
newborn IE orrganisms
staph, then candida
which IE organism only needs 1 blood culture?
Coxiella (Cox for blood Cx)
Duke criteria
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)
what are the duke minor vascular findings?
arterial emboli, septic emboli, mycotic aneurysm, janeway lesion, conjunctival hemorrhage
what are the duke minor immunologic findings?
Roth spots, osler node, glomerulonephritis
HACEK organisms
Haemophilus, Aggregatibacter, Cardiomacterium, Eukenella, Kingella
empiric drugs to treat IE
beta lactam, aminoglycoside +/- vanc, rifamin if prosthetic valve
adjuvant antibiotic if the patient has a prosthetic valve?
rifampin
surgical indicatsion for IE (3 buckets)
vegetation reasons, valve reasons, perivalvar things
what are the vegetation reasons for surgical intervention for IE
persistent vegetation after embolism, anterior mitral vegetation, 2 emboli in 2 weeks, 3 eboli total
what are the valve reasons for surgical intervention for IE
valve regurgitation, flail, or heart failure
what are the perivalvar reasons for surgical intervention in IE
valvular dehiscence, heart block, large abscess
which gets IE prophylaxis?
prior IE, prosthetic valve, heart transplant with regurgitation, CHD that is cyanotic, 6 months after surgery, residual defect
when do you give IE prophylaxis
before dental procedures, before pulm procedures, when working with infected tissue, not before GI/GU surgeries, not for orthodontics
KD predominantly affects kids < __ years old
5
risk of KD aneurysm decreases from __ to __ with IVIG
25 to 4 %
recurrence rate of KD
3%
msot KD happens in which season in the US?
spring
pathogenesis of KD and difference between moderate and large aneurysms?
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)
3 stages of KF pathogenesis
acute 2 weeks, neutrophil necrotic, subacute lyumphs months - years, chronic is luminal myofibroblastic proliferation
MC vessels affects in KD
LAD, RCA, LMCA (LMCA will almost always have other vessels affected)
Z score cutoffs in KD for vessel dilation
nml <2, dilation 2-2.5, mild 2.5-5 moderate 5-10, large >10
myocarditis findings in KD
subclinical dysfunction 50%, KD shock (7%)
valvitis in KD
MR (25%)
echocardiographic findings to support atypical KD
one of 3: LAD or RCA 2.5 or greater, coronary aneurysm, or dysfunction AND MR AND effusion AND LAD/RCA 2-2.5
indication for plavix in KD
in subacute disease, moderate dilation or chronic moderate/large dilation
indication for lovenox in KD
large/giant aneurysms or rapidly progressing aneurysms
adjucntive anticoagulants in subacte KD?
plavix if moderate, lovenox is large+
LMP
luminal myoproliferation
cardiac tumor associated with V arrythmia
fibroma
PKU in mother at risk for which CHD?
left sided obstructive lesions
chromosome in williams syndrome
7q11
7q11
williams syndrome
chromosome in jacobsen syndrome
11q23 (123)
11q23
chromosome in jacobsen syndrome (123)
what is LEOPARD syndrome
Lentigenes ECG conduction defects Ocular hypertelorism Pulmonary stenosis Abnormal Genitals Retarded growth Deafness
gene in kabuki syndrome
MLL2
MLL2
kabuki syndrome
how to differentiate between Marfan and Loez Dietz on the boards?
Loez Dietz will have dilation of the extra-aortic vessels
WPW associated with which 2 forms of CHD?
1) Ebsteins 20-30%, 2) HCM (10%)
age of rheumatic fevere and rheumatic heart diseas
fever 5-15 years, RHD 25-45
what % of acute rheumatic fever gets severe valvulitis with 1 episode?
10%
MC clinical manifestation of ARF?
carditis in 5-070%, arthritis second, carditis defines as MR or AI
why is ARF a disease of the margins?
erythema marginatum, socioeconomic factors affect people at the MARGINS of society, margins of valves are affected by inflammation in acute phase
ARF More common in males/females?
females 2:1
what forms on valves in ARF?
verrucae of fibrin and cells on the margins of valves
jones criteria for diagnosis
Initial: 2 major or 1M 2min
Recurrent: 2 major or 1M 2min, or 3min
Jones criteria major and minor in low risk
Major: carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules
Minor: polyarthRALGIA, Fever >38.5, ESR60CRP3, prolonged PR
Jones criteria major and minor differences in high risk vs. low risk
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
echocardiographic diagnosis of carditis in ARF?
MR: 2 views, 2cm jet, 3 m/sec pansystole
AI: 1cm jet, 2 views, 3m/sec, pandiastole
main difference between carditis in ARF and chronic RHD?
carditis = insufficiency, chronic is mitral stenosis and aortic insufficiency
mild MR murmur
high pitched, holosystolic at apex
high pitched, holosystolic at apex
mild MR murmur
harsh, holosystolic, loud S2, S3, mid-diastolic rumble?
severe MR (diastolic from relative MS)
murmur severe MR
harsh, holosystolic, loud S2, S3, mid-diastolic rumble?
classic MS murmur
loud S1, low pitched rumble, possible loud S2 (from P2 PH)
loud S1, low pitched rumble, possible loud S2 (from P2 PH)
classic MS murmur
CXR with curly B lines
LA hypertension
Tx ARF
high dose ASA, NSAIDs, PCN, treat family members
ARF prophylaxis meds and duration?
PCN G monthly, V BID, erythromycin,
no carditis 5 years or until 18
carditis 5 years or until 21, or longer if residual lesions
retinoic acid is associated with which CHD?
conotruncal defects (lets pretend it has to do with the movement of neural crest cells)
prevalence of elevated BP and HTN among youth?
elevated BP 10%, HTN 3%
rates of HTN with each of these co-morbidities: obesity, diabetes, OSA, CKD, left sided heart disease
obesity: 25% diabetes 6% OSA 3-14% CKD 50% left sided >50% in coarct
cuff size:
get arm circumference: lenght is 80%, width is 40%
easy to remember BP screening cutoffs for 3 y/o, 11 y/o 13 y/o
this is the 90th percentile:
3 y/o: 100 mmHg
11 y/o: 110 mmHg
13 y/o: 120 mmHg
criteria to diagnose with HTN?
ausculatory BP reading >95%ile at 3 different visits
BP categories
1-12years: <90% = normal, 90-95% elevated BP, 95% - 95% +12 = stage I, >95% + 12 = stage II
>13 years: adult cutoffs: 120,130,140
MCC of HTN in age groups?
>6 = essential HTN <6 = renovascular disease
initial lab work up for HTN?
UA, renal, lipids. RENAL ultrasound if <6 or abnormal UA
when do you get an ECHO or EKG for elevated BP?
echo when startign drugs to assess for end-organ damage or for etiology. EKG not needed
when do you get a renal ultrasound in HTN?
<6 years old, abnromal UA, stage II, diastolic HTN, abnormal renal, or h/o kidney disease
treatment goals for HTN?
<13 years: <90 %ile
>13 years: 130/80
first line treatment for HTN:
ACE inhibitor, send is thiazide, then calcium channel blockers
walk thru the lipid metabolism
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
which is the atherogenic apoprotein?
ApoB
causal genes for familial Hcholesterolemia?
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
range for common Hchol, hetero FH, homog FH?
common 130-200, hetero 160-400, homoz >400
clinical findings in hoozygous FH?
xanthomas, supra-AS, coronary abnl
what does lipoprotein A do?
makes LDL bond in a stickier fashion to vessel walls
range of hypertriglyceridemia in famililal and severe?
familil 200-100,severe > 1000, manage with very low fat diet
what is Tangier’s disease?
very low HDL with orange tonsils (Think of Tracy Jordan)
when to screen for FH?
9-11, or 17-19, age 2 with strong fmaily history or obesity other risk factors may screen earlier
treatment threshold for common Hcholestereol?
190 for everyone, 160 if risk factors, 130, if high risk. preceded by 6 months counceling and only if > 10 years
2 tests for screening for familial Hchol?
non-HLD = total cholesterol - HDL cholesterol, should be < 145
or fasting lipids
treatment of high trigluycerides
- lifestyle
- fenofibrate
- fish oil
treatment for hypercholesterolemia meds?
- lifestyle
- statins
- Ezetimibe
expected LDL drop with lifestlye and statins
lifestly 10-15%, statins up to 50%
contraindicated medication with statin?
gemfibrozil (common hypertriglyceride medication) think of a sharp GEM causing rhabdomyolisis of a static muscle
acute pancreatitis
hypertriglyceridemia (Lipoprotein lipase deficiency)
IF all else fails and you can’t remember inheritance, what general rule can you apply?
most CHD is autosomal dominant, most cardiomyopathy is autosomal recessive
orofacial cleft, micropthalmia, prominent nasal bridge, Dx?
Trisomy 13, associated with post-axial polydactyly, holoprosencephay
Card: VSD, TOF, complex, polyvalvar, Pulmn HTN
(LOC mid-BDRM)
prominent occiput, narrow bifrontal, short palpebral fissues
trisomy 18 - chromosomal
Card: polyvalvar disease, VSD< TOF, complex (LOC Mast Bath)
female, short stature, webbed neck
Turner 45 XO - chromosomal
left sided lesions, asc ao dilation, PAPVR, prolonged QT
LOC library
INT aortic arch B
22q11 microdeletion
Tubular nose, hypoplastic nasal aleae, bulbous nose tip, cleft palate velopharyngeal insufficiency,
Card: IAA-B, truncus, TOF, RAA
LOC downstair workshop
stellate irises
Williams-Beuron 7q11.23 microdeletion
Card: SVAS, PVAS, PPS, CoA, areteriopathy
Other: UNique cognitive profile hoarse voice
LOC (donstair gues bed)
coloboma, choanal atresia
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
triangular face, Hypertelorism, pointed chin
Aligille - singel gene JAG1, Notch 2 branch PAS, TOF< VSD live bile duct stuff, butterfly vert LOC living room
pre-axial abnormality
Holt Oram - single gene TBX5 non-dysmprphic, arm abnormalities progressive conduction disease LOC entryway lovated by stairs, conduction disease
downslanting palpebral fissurees, epicanthal folds, ptosis, webbed neck
Noonan - single gene (RASopathy)
PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship
short stature, pectus, PS with dysplastic valve, HCM
Noonan’s like with deafness
LEOPARD = Noonans with multiple lentigenes (RASopathy)
PTPN11 (90%), dominant negative
lentigenes after puberty
LOC loft
cafe-au-lait, iris lisch nodules
NF1 - single gene (RASopathy)
rare cardiac Coarct, PS (2%)
learning diability, cerebral artery aneurysm, hypertension
arched eyebrows, broad thumbs
Rubenstein Taybi - single gene CREBBP or microdeletion 16
Cardiac: PDA, septal defects, CoA, PS
LOC stairs going down (creeping down the stairs)
bell shaped ribcage, short, polydactyly
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!)
dextrocardia, situs inversus totalis
Kartagener = single gene but autosomal recessive DNAI1, DNAH5 - strands of DNA nondysmorphic frequent lung infections LOC: small bathroom
malar hypoplasia, narrow face, micrognathia
Marfan = fibrillin single gene - autosomal dominant
FBN1
Card: aortic dilation, risk of dissection, MV prolapse
has eye findings (ectopia lentis)
Marfans with no eye stuff
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)
marfan with contractures
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
bruising and scarring with thin skin
vascular ehlers danlos - single gene autodom
COL3A
spontaneour arterial rupture
no mitral valve prolapse or aortic root dilation
LOC bottom of driveway
7q11.23 microdeletion
Williams
11q23
Jacobsen
16p deletion
Rubenstein taybi (or CREBBS single gene) creep down stairs
CHD7
CHARGE CHD7 = Charred (LOC side of house by grill)
JAG1
Aligille - singel gene
JAG1, Notch 2
Notch 2
Aligille - singel gene
JAG1, Notch 2 Jagged 1 alligator with 2 Notched teeth
TBX5
Holt Oram - single gene
PTPN11
Noonan - single gene (RASopathy)
PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship
OR
LEOPARD (90%) dominant negative
SOS1 (10%)
Noonan - single gene (RASopathy)
PTPN11 (50%), SOS1 (10%) Pretty pretty, save our ship
NF1
Nerufibromatosis (RASopathy)
CREBBP
Rubenstein Taybi - single gene CREBBP or microdeletion 16
EVC, EVC2
Ellis Van Crevald - single gene autosomal recessive
gene EVC, EVC2 (Evacuate!)
DNAI1, DNAH5
Kartagener = single gene but autosomal recessive
DNAI1, DNAH5 - strands of DNA
TGFB1,2
Loez Dietz = single gene - auto dominant TGFB
TGFB1,2 (the greenest finest beans)
FBN2
Beals disease = congenital contractural arachnodactyly
single gene FBN2 (fibrillin 2)
COL3A
vascular ehlers danlos - single gene autodom
COL3A
gene for VACTERL
trick! its an association
cardiac rhabdo
tuberous sclerosis (auto dominant)
TSC1, TSC2
facial angiofibroma, ungual fibroma, retinal hamartoma,
TSC1, TSC2
tuberous sclerosis (auto dominant) TSC1, TSC2
atrial myxoma
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
PRKAR1A
atrial myxoma (PARKOUR on the back PORCH)
5p
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
4p
wolf hirschorn
Shield face
4p deletion
LOC: pool room with chewbacca
MYBPC 3
MYBPC 3: My Blood Pressure Contractility! 3
gene mutation differences between Duchenne and Becker
Duchenne = frameshift mutation causing absent protein Becker = in-frame causing a shorter protein
when do DMD get cardiomyopathy by?
Dilated 100% by age 18, BMD in the teens as well, but live into their mid-40s
genetic causes of Emery Drifuss MD?
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
elbow and achilles contractures
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
mutation of proteins that associate the cell membrane with dytrophin
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
mutation at risk for SCD in muscular dystrophy?
EMD (soap), LMNA (washcloth), DES (DESmin)
ataxia with HCM
Friedrich's ataxia Frataxin FXN (FRAT, FRAT, FRAT!) HCM -> DCM LOC: my room
elevated lactate, ptosis, seizures, encephalopathy
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
decompensation with fastinging, hypoglycemia, metabolic acidosis
fatty acid oxidation defect
HCM and arrythmia
LOC: downstairs desk
weakness, failure to thrive, hepatomegaly, giant ECG voltages
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
coarse facial features, skeletal and joint problems
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)
lipidoses with heart problems
Gaucher (Toaster) and Fabry (Stove)
progressive neurological deterioration with HSmegaly, anemia, thrombocytopenia, bone pain
Gaucher disease GBA (Bad GPA) Tea and Toast diet leads to anemia glucocerebtosidase (grain brain) LOC: Toaster
GBA
Gaucher, bad GPA = GBA
male with periodic crisis of severe extremity pain, angiokeratomas, corneal clouding
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
round face, tall forehead, deep set eyes, prominent ears
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
4 big categories of cardiomyopathies
Sarcomeric, Dystrophinopathy, Muscular dystrophies, Inborn Errors of Metabolism
ACHD TOF, risk of PCOD?
Potts or Waterston shunt, direct connection causes enregulated pulm blood flow, risk of PHTN and then PCOD
what is Nakata Index, how is it used?
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
which rim is most important to allow for device closure of an ASD?
posteroinferior
which coarct repair has the highest risk of speudoaneurysm?
synthetic patch repair
treatment f post-pericardiotomy syndrome
high dose aspirin for 4-6 weeks
onc kid with sepsis
early form of anthracyclin-induced CMP
LVNC, which syndrome to associate?
Barth - Tefazzin mutation (think about steak being cut up by tazmanian devil), X linked
LGE location: epicardial
myocarditis (closest to the artery)
LGE location: midwall
DCM
LGE location: subendocardial
ischemia (farthest from the artery)
LGE location: LV-RV junction
HCM (pulled the most in these regions)
LGE location: none, instead aneurysm+clot
Chagas
LV anuerysm disease
Chagas
Prognosis of fulminant myocarditis
fast on and fast off. hemodynamic compromise with 2 weeks of presentation, Px better than acute which has a less distinct onset
myocarditis that doesn’t get better after 2 weeks
suspect giant cell myocarditis, treat with steroids. poor prognosis, due to autoimmune most likely, 10% recurrence following transplant
histo slide with multinucleated giant cell
giant cell myocarditis
Tx lyme carditis w/ dysfunction
IV CTX 3 weeks
lyme carditis, po or IV antibiotics?
If complete heart block -> IV
If PR>300 -> IV
If PR <300 -> po
how to Dx lyme carditis?
lyme titers
germ for lyme carditis?
borrelia burgdorferi (sounds like you are eating peanut butter)
right sided myocarditis? disease? germ? Dx? Tx? associate?
Chagas disease, trypanasoma cruzi
Dx with ELISA test
Tx: bendazole
Associate LV aneurysm -> clot -> stroke
incidence of DCM? 5 yr survivival?
0.57 / 1k
5 year survival = 50%
nutritional deficience that causes DCM?
thiamine deficiency
sarcomeric causes of DCM?
Titan TTN, MyH6,7
Lamin defect causes?
DCM with conduction issues
Tx asymptomatic DCM? then if symptomatic
betablock and ace
syptoms -> lasix, aldactone, digoxin
tefazzin gene?
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)
neutropenia
think of Barth syndrome
desmosome mutations
ARVC, autosomal dominant
Define HCM
LV hypertrophy without an identifiable cause (no increased afterload)
Define DCM
dilation and systolic dysfunction in 1 or more ventricles
MCC SCD in athletes
HCM
incidence of HCM?
0.47 / 100k
disarray in a biopsy micro?
HCM
normal BP response to exercise?
> 20
in HCM, < 20 is an indication for ICD
on HCM therapies that are proven to improve survival?
1) ICD
2) exercise restriction
beta blockers don’t!
medical treatment of HF in HCM?
1) beta blocker
2) CCB (verapamil)
3) exercise restriction
indications myomectomy in HCM?
refractory symptosm with LVOT >50mmHg, SAM
if you have a patient with HCM, what do you do with their family members?
screen all first degree. in genotype+phenotype negative patients, follow Q3H until 30 years and Q1Y during puberty
what do mass and volume got to do with hypertrophy?
in HCM: inc LV mass to volume ratio
Athlete’s heart: normal LV mass/volume ratio
MC inherited ataxia?
freidrich’s ataxia
dx of hurler disease?
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)
inborn error metabolism, type of cardiomyopathy typically?
HCM
left and right heart failure with prominent S2
suspect RCM
define PAH
PH = mean PAP > 25
PAH =
mean PAP >25
wedge<15
PVRi > 33 MC genes of PAH
BMPR2 (75%)
ACVRL1 ( also associated with HHT)
TBX4 (associated with abnl lung development)
histo feature of adanced PAH?
plexiform lesions
prevalence of PAH in CHD?
repaired 15%, unrepaired 30%
how to decide operability in PAHD with CHD?
1) cath for PVR (if PVR>6, PVR/SVR>0.3)
2) next, vasodilator test
3) PH therapy with repeat cath
single ventricle, what is abnormal pulm HTN?
PVRi>3, TPG>6
first line PAH treatment in mild disease?
calcium channel blocker
2 endothelin receptor subtypes an affects
A is on smooth muscle, B is on Both smooth and endothelial
difference between ambrisentan and bosentan?
bosentan affects Both ET_A and ET_B and causes liver damage
ambrisentan specific for A (smooth muscle), no liver damage
first line therapy for severe PAH and RV failure
prostacyclin
WHEN TO DO SURGERY FOR PAH?
ONLY with suprasytemic RV pressure, to unload the right ventricle
what are the ACC/AHA stages?
A: at risk, no dysfn, no symptoms
B: asymptomatic with dysfunction
C: symptomatic LV dysfunction
D: refractory requring VAD/Tx
what are the NYHA stages?
1: asymptomatic
2: symptomatic with mod exertion
3: symptomatic with mild exertion
4: symptomatic at rest
treatments associated with each ACC/AHA stage:
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
HF symptoms, what springs to mind?
Stage 3, fluid causes symptoms of congestion (start lasix/aldacton, restrict) -> digoxin for symptoms
PV loop in heart failure, what does lasix do, what does afterload reduction do?
lasix dec preload, decrease right side
afterload: decrease top
what is sacubitril?
part of entreso witch is scubitril/valsartan. neprilysin inhibitor, inhibits breakdown of BNP for diuresis
aldosterone association
fibrosis
laplace law equation
Wall stress = Pxr/(2x wall thickness) = pressure x volume/LV mass
what does B1 do in heart failure?
B1 receptors are downregulated, BB help upregulate them
cause of heart failure in kids?
non-adherance (esp adulescents with salt/fluid restriction)
2 minute assessment of HF
cold/warm, dry/wet
wet -> diuretics
cold -> vasoactives
HF with Low CO, treatment
vasodiltors, nipride/nitroglycerine, milrinone
evidence for lasix
improves symptoms, no survival benefit
evidence for ACE-inhibitor
reduces mortality, reverse remodeling (all patients with reduced EF
when to use a -sartan?
when ACE causes cogh/angioedema
evidence for carvedilol, notable side effect?
reduces mortality, reverse remodeling, use with all reduced EF, causes wheezing, would switch to metoprolol
side effect spironolactone
hyperkalemiam if gynecomastic, switch to eplerinone
evidence for spironolactone
reduce mortality, reverse remodeling
PANORAMA trial
use of valsartan-sacubitril in kids reduces mortality, reverse remodeling (a wide panorama)
HF drugs shown to improve survival and reverse remodel?
ACE/ARB
Beta blockers (carv, meto, biso)
Aldo receptor blockers
(NOT lasix, NOT digoxin)
what evidence for digoxin?
reduces hospitalizations, improves symptoms but no survival
side effects: arrythmia, heart block, peaked T, prolonged QTc, med interactions with amiodarone
hyperK on AC/ARB, what to do?
dose reduce spironolactone
cough develops with ACE, what to do?
r/o heart failure(CXR), then switch to ARB if cough persists
angioedem on ACE< what to do?
STOP immediately, switch to ARB
when you admit a patient in heart failure, do you stop the beta blocker?
continue BB unless dec CO, when you would start inotropes
wheezing on bet blocker
switch from carvedilol to metoprolol
asymtomatic LV dysfn, what 1 drug to start?
ACE-I, (beta blocker second choice)
what drug for HFpEF?
lasix only
indication ICD in kids
DON”T use adult guideline of EF<35%
ONLY aborted Sudden Death, unexplained syncope, recurrent VT
when to consider cardioc resyncrhonization therapy?
EF<35%, LBBB, QRS>150
single V with BBB (dominant-side) and EF <35%
PLE; Dx? Tx? Px?
stool alpah 1 antitryypsin
Tx: aldactone, steroids, heparin, octreotide, fenestration, transplant
Px: 5yr 50%
Class I indication for Heart transplant
Stage D
Stage C with limitation in physical activity, growth failure, sudden death, restrictive w/ PH
Retransplant for graft vasculopathy with dysfn
Contraindications to Heart transplantation to know:
irreversible PVR>8WU (no NO response)
retransplant within 6 months of transplant
1yr and 5 yr survivla of Tx in infants and adolescents
Infants: 1yr = 75%; 5yr = 68%
Adoles: 1yr = 88%; 5yr = 68%
median survival of Heart Transplant
15 yr
what does HLA lead to?
antibody mediated rejection
solution: avoid, desensitize, or treat AMR aggresively
who can get ABO incompatible, what are the outcomes?
infants with isohemaglutinin titers <1:8 (outcomes same as blood group matched)
goal ischemic time in HTx?
<4-6hours
purpose of ATG?
blasts T cells, allows delay in calcineurin start (less kidney problems)
ATG mechanism of action, treatment goal? alternative?
ATG is anti-CD3, CD3 count <30 (3, 30), alternative is basiliximab (IL-2)
Most important immunosuppresant in HTx? MOA? side effects?
calcineurin inhibitors (tacro and cyclosporine)
block T cell activation, stop IL-2 production (looks like arrows)
nephrotoxic, diabetes (toxic personality and probably had DM)
adjunct HTx meds to calcineurin inhibitors?
MMF (or imuran)
antimetabolite, prevents both T and B cell proliferation
GI side effects, neutropenia (studies neutrophils, GI upset often at that job)
when do you use sirolimus?
kidney dysfunction and CAV
MOA mTOR inhibitor, but inhibits more than this (TORO inhibitor)
side effect: wound healing, aphthous ulcers
side effects tacro
nephro, diabetes (ERIC C has toxic kidneys and DM)
side effects cyclosporine?
HTN, gingival Hplasia
side effects sirolimus?
delayed wound healing, proteinuria, hyperlipid
side effects MMF
mycophenolate
anemia, neutropenia, GI side effects
MC presentation of Acute cellular rejection
asymptomatic, picked up on biopsy
grading of acute cellular rejection?
mild = 1R = local inflammation moder = 2R = loc inflam + focal necrosis severe = 3R = diffuse inflammation + necrosis
biopsy of AMR vs. ACR?
antibody = edema and c4d positive (no cells) cellulat = inflammation (cells) +/- necrosis
causes of AMR? how to treat? risk for?
antibodies: high PRA, DSA, or positive cross match
Treat with IVIG + plasmapheresis
risk of CAV (very tight association)
history of antibody mediated rejection puts transplant patient at risk for?
coronary artery vasculopathy, sirolimus helps by being active against B in addition to T
HTx with Fever + diarrhea: treatment
CMV: risky when off valcyte, risk for CAV
Tx: gancyclovir
HTx with Fever + pneumonia: treatment
CMV: risky when off valcyte, risk for CAV
Tx: gancyclovir
HTx with abd pain and nodules. Dx? Tx?
likely PTLD< must Bx for diagnosis (EBER positive), EBV driven
Tx: decrease immunosuppression
HTx, rising Creatintine? Dx? Tx?
Calcineurin inhibitor nephropathy
switch to sirolimus
leading cause of late graft loss in HTx? Tx?
CAV
risks include antibody mediated rejection and CMV infection
Tx: switch to sirolimus, ReTx if dysfunction
new 2018 ACHD guidelines, what worts of classifications
anatomic (simpled, moderate, great complexity), AND physiologic (NYHA class), classifcy according to highest complexity
predictors of successful transition to ACHD?
1) older age at last visit 2) greater number of surgeries 3) documented recommendation to follow with ACHD center
when do you start transition counseling? how should you do it?
12 years of age; written transition plan including need for lifelong care
increased risk of center in ACHD?
adutls w/ CHD, 1.5-2x greater lifelong risk
how many CHD patients develop a form of SVT?
45% with an atriotomy!
causes of chronic breathing problems in ACHD?
30% restrictive spirometry, 20% OSA
when do you do phlebotomy in ACHD?
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)
a cynotic pt asks you about pregnancy, what do you counsel?
pregnancy contraindicated, also get on some non-estrogen birth control
how to treat hyperviscocity in cyanotic patient?
adequate hydration?
iron deficiency anemia?
phlebotomy RARELY indicated if HGB >20
EKG risk factor in TOF?
QRS >180, independent risk factor sudden death
arrythmia in TOF?
double risk of mortality, V tach is more like 50%, atrial 10%
TOF: diastolic dysfunction
relates to sustained VT
ACHD TOF with dysfun?
need to treat aggresively, associated with poor outcomes
Classes of indications for PVR in TOF?
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
what does PVR do for outcomes in TOF?
no difference in outcomes! just good for symptoms
higest incidence of aneurysm formation in coarct repair?
patch, but specifically dacron patch!
causes of hypertension after coarct?
abnormal endothelial function, increased arterial stiffness (often have exercise induced hyeprtension
boards answers for fontan adult with arterial desaturations? (8 of them)
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
fontan anticoagulation outcomes?
ASA best, warfarin a little less, none is BAD
worse type of valvular lesions in pregnancy?
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
antigcoagulation in pregnancy scheme?
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
mechanical valve pregnancy, warfaring 3mg, what do you do?
keep warfarin, add ASA in 2nd trimester, planned V delivery with UFH
mechanical valve pregnancy, warfaring 6mg, what do you do?
switch to LMWH, 2nd trimester, switch to warf+ASA, planned V delivery with UFH
who can’t get combined contraceptives?
cyanosis, Fontan, mechanical heart valve: risk of thrombosis
why is IUD preferred for most women w/ CHD?
<1% failure rate, no thrombosis, Fontan has a tiny risk of vagal reactions
which at risk CHD women should not use progestrone-only birth control?
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
what are the 9 cardiac risk factors for mommas with CHD?
fontan prior cardiac event poor functional capacity cyanosis mechanical heart valve decreased Ventricular function Pulmonary hypertension aortopathies symptomatic left-sided obstructive lesions
3 mechaisms of arrythmia
automatic, re-entrant, triggered automatic
strucutural heart disease associated with accesory pathway
ebsteins (20%), L-TGA (5%), HCM
SVT shows preexcitation pattern, what does this mean?
antidromic AVRT (orthodromic goes through AV node, does not show preexcitation)
which is more common, orthodromic or antidromic AVRT?
orthodromic in 95%
what are the 2 main variants of accesory pathways?
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)
natural history of newborn SVT
93% no clinical SVT by 8 months.
subclinical goes away in 30% by 12 months.
Recurrs around age 8
cascade of treatment for acute AP SVT
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)
who do you avoid CCB in SVT?
example is verapamil
dysfunction (depresses EF), <1yr (hypotension), or preexcitation (blocks AV node)
chronic treatment for SVT with preexcitation?
beta blockers, avoid digoxin and verapamil (AVN blockers)
chronic treatment for SVT without preexcitation?
beta blockers, digoxin, class Ia, Ic, III, IV
what RR interval is dangerous in WPW?
<250 msec, risk of sudden death
direction of typical AVNRT?
slow fast (short RP), P wave will be a barely visable inverted p at the end of QRS from fast Upstroke
direction of atypical AVNRT
fast slow (long RP), P wave will be inverted from SLOW Upstroke
4 types of long RP tachycardias
atypical AV nodal reentry tachycardia (fast slow, slow upstroke) PJRT (slow retrograde AP) EAT (automatic atrial goes through AVN) sinus tach (goes through AVN)
natural history fo post-op a tach
resolves within 2-3 months
natural history of a tach in patient < 3 years
resolves within 2-3 months
5 CHD at risk fo A tach in long term
Fontan, dTGA s/p atrial switch, TOF, Ebstein, ASD
irregular tachycardia, what do you look for?
distinct p waves, then it is atrial tachycardia (if not A fib)
2 ypes of atrial flutter
typical (TV annulus)
atypical (incisional), will usually not have the sawtooth waveform)
acute treatment for atrial flutter, how do you rate control?
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
acute treatment for atrial flutter, how do you cardiovert?
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
when do thromboembolic events happen after DCCV for afib/aflutter?
first 3 days due to atrial stunning, ALWAYS treat for 4 weeks with anticoagulation afterawrds
4 types of a fib?
paroxysmal: terminates spontaneously, last < 1wk
persistent: lasts > 1 week
long standing: lasts > 1 yr
permanent: sustained, no further attempts to restore sinus rhtym
rate control tretment for a fib, what considerations and what drugs?
heart failure and accesory pathway:
If no HF, no AP: BB, CCB, amio
if HF: dig, BB, amio (avoid CCB)
If AP: ibutilide, procainamide
acute treatment for A fib with WPW?
rate control: procainamide, avoid AVN blocker as single agent
If < 48 hours: ibutilide, procainamide, DCCV
definition of sustained VT?
> 30seconds
risk of using ibutilide?
4% risk of torsade, avoid with low EF
DDX for wide QRS complex tachycardio?
VT, SVT with BBB, SVT with aberration
PVCs: 4 associated conditions
1) structurally normal,
2) cardiomyopathy/myocarditis
3: AVRC (>500 PVCs in 24h)
4: channelopathy (often multiform)
when do you worry abouut PVC related cardiomyopathy?
> 10%
how do you manage PVCs in kids?
aymptomatic with normal EF: observe
symptomatic infant: BB
symptoamtic child: BB, CCB
LV dysfunction: BB, CCB
newborn with ventricular rhythm, what diagnosis?
accelerated idioventricular rythm if;
1) rates just faster than sinus
2) structurally normal heart
3) baseline EKG is normal
- -> benign, persists 2-3 months
VT with LBBB pattern?
RVOT tachycardia
LBBB, Inferior axis
12 lead with VT, where do you look?
look for BBB and inferior leads
LBBB + upright in II, III aVF —> RVOT
RBBB + downward in II, III aVF —> Idiopathic/fascicular VT
RVOT tachycardia, what DDX?
usually structurally normal, also AVRC, myocarditis, tumor
treatment of RVOT tachycardia?
verapamil, adenosine sensitive BB, class I, III agents
treatment of idiopathic/fascicular VT?
verapamil sensitive
BB, CLass I, III agents
what is AH? HV?
AV node conduction
purkinje conduction
what is an effective refractory period?
longest RR where stimulus fails to propogate
differentiate between mobitz 1 and 2 on invasive EP study?
mobitz 1: AH interval lengthens, loss of H
mobitz 2: AH stays the same, then there is a loss of V
risks relating to location of ablation
1) coronary injury
2) recurrence rate
3) AV block
4) stroke
1) coronary injury - left posterior
2) recurrence rate - right free wall
3) AV block - septal
4) stroke - left side
EP study, first question
stim or no stim
EP study, stim, next question
atrium or ventricle
EP study, stim atrium, next question
conduction to V (look for H and V). If His present at time of block, His/purkinje disease. If His drops, probably wenkebach
EP study, stim ventricle, next question
conduction to A: eccentric or concentric
EP study, no stim, next question
fast or slow
EP study, no stim, fast, next question
reg/irreg, narrow/wide, AV relationship
EP study, no stim fast, narrow, reg, A>V, DDX?
Fib and flutter are obvious, others can be EAT or 2:1 AVNRT
EP study, no stim fast, narrow, reg, V>A, DDX?
junctional or VT
EP study, stim ventricle, eccentric
left lateral pathway
EP study, no stim fast, narrow, reg, V=A, DDX?
see if midline or eccentric
define dual AV node physiology
AH interval increases at least 40 msec after 10 msec decrease in coupling interval
short HV
accesory pathway!
risky WPW number
250msec!
what weight to get to prior to EP study
15 kg
EP study, no stim, slow, earliest A in HRA, next question
HV interval (if small < 35, then its an accesory pathway)
5 phases of channels for myocardium, what ions?
0 = Na 1 = K 2 = K and Ca+ 3 = K (Long QT syndrome 1 and 2) 4 = K
2 phases of channels for SA and AV node, what ions?
0 = Ca++ (Ca is the clock) 2,3,4 = K+
Class 1a agents, effect on AP curve?
quinidine (C), procainamide (desk), diopyramide (N)
delays depol and repol (late to bed, late awake)
quinidine, type, channels, treatment, contraindications
1a (Na with tiny K) indication (Brugada) don't use in long QT risk of torsade and cinchonism LOC: C bedside
procainamide
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
disopyramide
NA+ and K+ (similar to quinidine)
LOC: Nick bedside (anticholinergic and running clothes leading to VV syncope)
cinchonism
quinidine
class 1B EP stuff
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
lidocaine
NA + selective
no effect above His
LOC: C bed
mexilitine
Na+ selective (oral lidocaine)
no effect above His
used as treatment for LQT3 (cutey 3)
phenytoin
anti-seizure medication
used to be used for digoxin toxicity (dig in attic)
class 1c stuff
flecainide (change table), propafenone (bed by the window since some BB activity)
prolongs QRS and QT (QRS widening is the big risk!)
QRS widening
flecainide tox
flex slows conduction and slows dissociation
flecaininide type, activity, watch out for, interactions
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)
proprapenone type, activity, watch out for, interactions
increases action potential duration widens QRS, some BB activity
LOC: by the window
ineraction: may icnrease dig levels
which Bet blockers are selective, which nonselective?
selective A:M, nonselective N=Z
selective: atenolol, esmolol, metoprolol
nonselective: nadolo, propranolol
5 beta blocker metabolism?
nadolol and atenolol kidney (pool and sprinkler)
metoprolol and propranolol liver (mountain and prop play)
esmolol is blood
drug drug interaction with beta blockers?
propranolol interacts with flecainide (both level higher)
NSAIDS potentiate hypotension in nonselective
B1 and B2 effects?
B1: cardioselective, receptors on the heart, slow HR and conduction, decrease contractility
B2: lungs and smooeth muscle (bronchospasm, vascoconstriction)
hypoglecmia and meds?
beta blockers
strongest Na channel blocker?
flecainide, class 1C
anticholinergic side effects med
disopyramide
K channel EP effects
delay repolarization (late brunch), prolong QT (important!) risk of torsade with all
amiodarone stuff
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
why is amio preferred over other antiarrythmics
less LV depression
Sotalol stuff
K+, little Na+
nonselective BB
prolongs AP duration
risk of torsade
ibutilide stuff
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
Dofetilide
atrium only K channel, used in a flutter and fib
(LOC: big chair where you DOF your worries)
2-4% risk torsade
Dronedarone
less effective amiodarone
LOC: fake kid kitchen, less effect, fake foods)
where do Ca blockers act?
SA and AV nodes, Ca++ upstroke, slows it down
don’t use in AVRT due to AV block
verapamil stuff
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
diltiazem
Ca channel blocker
used in SVT as fib and flutter control
LOC: downstairs bathroom , close to A fib and flutter K blocker meds)
Adenosine stuff
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
Ivabradine
Inward K current blockers, inhibits SA node activity -> dec HR
used in POTS, inappropriate sinus tachycardia
what meds increase digoxin levels?
Frank - Flecainide Ate - Amiodarone Pizza - propafenone Very - verapamil Quickly - quinidine
how many peopl have syncope? recurrence? population? % VV synceop, % cardiac syncope?
up to 50%, only 10% recurrence rate, MC adolescents and females
80% VV
2% cardiac
mechanism of VV synceop
standing -> dec preload -> HYPERcontraction -> mechanoreceptors activated -> dec sympathetic and inc PS output:
1) bradycardia
2) peripheral vasodilation
3) failure of reflex cerebral dialtion
4 signs of siezure over syncope
1) smell prodrome
2) rythmical movements before the fall
3) tongue bite,
4) confusion
Treatments for VV synceop
salt and water
florinef (evidence in adults)
midodrine (most evidence in peds - 70% risk reduction)
beta blockers (better for > 40)
Dx definition of POTS
Lasts . 6 months, > 40 bpm, absense of orthostatic hypotension (BP drop < 20)
4 big risk factors for sudden death in HCM
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
Dx of LCA off right, next step:
restrict, surgery -> after 3 months exercise, if negative can participate in sports
Dx RCA off left, next step
exercise test, can play sports if asympomatic and negative
EKG findings of ARVC
V1 and V2 epsilon wave (post-excitation of islands of RV muscle)
upright T wave in V2, V3
etiology of epsilon wave
(post-excitation of islands of RV muscle)
LQT1: gene, type of defect trigger ECG pattern
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)
LQT2: gene, type of defect trigger ECG pattern
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!)
LQT3: gene, type of defect trigger ECG pattern
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)
IN general, what disk factors for LQT syndrome?
prior syncope, QTc >500 (except 3, where you use 470 as the cutoff)
CPVT: what defect? what MOA
ryanodine receptor-> leasd to excessive Ca leak out of the SR, delayed after depolarization
bidirectional VT
CPVT!
CPVT< what type of VT on EKG
bidirectional VT!
mortality of CPVT?
30-50% by 30 years
treatment of CPVT
betablocker (Nadolol) +/- flecainide
ICD if recurrent VT or cardiac arrest
brugada,
gene
EKG abnormalities
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)
9 risk factors for SCD in TOF?
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
Risk of SCD in aortic stenosis
2+ episodes of syncope, outflwo gradient > 50 mmHg, AI, High LVESP, LV dysfunction
what is bachman’s bundle?
conductive tissue connects RA to LA
how does the autonomic NS modulate SA and AV nodes?
CA++ permeability (cagus decreases which decreases upslope and slows HR)
how to differentiate vagal bradycardia from sinus node dysfunction?
vagal is related to situation, position, carotid sinus, and AV node may be associated
indications for pacemaker for sinus node dysfunction?
if symptomatic
what % of maternal lupus end up for complete heart block?
5%
class I indications for pacing with CHD?
symptoms, 7 days post-op,
congenital heart block with 1) wide QRS or dysfunction or 2) V rate < 50 (<70 in CHD)
what is PVARP and why is it used
prevents atrial sensing of far field V (QRS), farfield T, or retrograde P. set time where atrial sensing DOES NOT occur.
atrial refreactory period in pacemakers, what two components and what should it be?
TARP = PVARP + AV interval. needs to be less than atrial rate (cycle length)
what does putting a magnet on a ICD do?
stops ICD shocks, but no pacemaker capability
evidence for resynchronization therapy?
2005, multicenter with 103 patients, EF increasd by 12%, some taken off transplant list.
fetal brady with occasional tachy
LQT
structural heart disease assocated with fetalb CHB
heterotaxy, AVCD, ccTGA
remember: high mortality with CHD and hydrops + CCHB
risk of CHB mom with lupus and recurrence risk
5%
recurrents 15%
timeline of CCHB conversion to 3rd degree?
16-28 weeks
evidence for steoids in 1st, 2nd, 3rd degree CCHB?
3rd: no evidence:
2nd,1st, some benefit
1st: 50% spont resolution
fetal bradycardia for CHB with HR 52:
treat terbutiline for < 55
causes of 2irregular heart rhythm in fetus?
PAC, PVC, 2nd degree AV block is the tricky one
how to differentiate: is the PAC premature or not!
risk for death with fetal tachycardia?
% of time in tachy: < 20%, you nare okay, > 50%, hydrops
5 risks for death in CCHB?
structural, hydrops, HR < 55, decline in HR by >5, dysfunction
fetal flutter, treatment?
sotolol
fetal avrt, treatment?
digoxin, then flecainide
dose for fetal digoxin
1-2 mg/kg LOAD
0.5 maintenance
when should you skip digoxin when treating fetal AVRT?
hydrops -> go straight to flecainide
side effects with tacro levels are high
irritability and tremulousness -> can even lead to seizures
which meds are notorious for increasing tacro levels?
antifungal meds -> also amiodarone and Ca++ channel blockers
MLL2
kabuki syndrome (MuLan-Like)
kabuki syndrome gene to know
MLL (MuLan-Like)
echo best resolution
axial > lateral > elevational
angle maximum on doppler to know?
20 degrees (cos theta = 0.94
when does simplified bernouli not work and what are examples?
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
which is bigger, peak instantaneous gradient or peak instantaneous gradient?
PIG > PTP
4 components to load dependency of measuring systolic function
1) preload: increases shortenting
2) afterload: decreases shortending
3) contractility: changes in circumstance
4) heart rate: inc shortening via force frequency relationship
what is vcf?
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)
what is dyskinesis?
ventricular muscle moves in the wrong direction
where do you measure deceleration time?
downslope time of the E wave
diastolic dysfunction, why look at pulm veins?
PV a wave duration -> should be LESS THAN the mitral valve inflow A eave
E’, what cuoff to look for
10: anything less than 10 is abnormal
how can you tell pseudonormmalization from normal in diastolic dysfn?
pseudo has a E’ < 10, E: E’>10, PV a waive is > MV a wave
agitated saline intracardiac or intrapulmonary?
<3-5 beats
>5 beats (or if you see the pulm vein)
loc eustacean valve
ANTERIOR aspect of IVC/RA junction
recurrence risk of CHD with parent or sibling with CHD?
2-5%, LV obstruction is 8%
HLHS recurrence risk?
8%
which is riskier for a fetus with CHD? maternal or paternal HCD?
Meternal 3-7, paternal 2-3
when is maternal phenylketonuria risky for CHD?
If poorly controlled P level >15: 10-15 increased
P <6: no risk
does warfarin cause CHD?
no
maternal Rubella assocated with (2)
persistent PDA and branch PA stenosis/hypoplasia
risk of CHD with assisted reproduction technology?
1-3% risk, mostly septal defects (confounded by maternal age, twinning, etiology of infertility)
fetabl bradycardia, risk of CHD
fetabl tachycardia, risk of CHD
fetabl irregular rhythm, risk of CHD
brady: 50%
tachyc and irregular < 1
GI abnormalitiesm on obstetrical ultrasound will they have CHD?
20-30%
what is nuchal translucency assocated with?
aneuploidy, notmal < 3, > 6 high risk of CHD
what does absent ductus venosus do?
loss of sphincter, causes high output through venous chamber and heart failure
define hydrops?
fluid accumulation in >= 2 compartmentss: pleural, pericardial, skin, abd, placenta
umbilical vein doppler, what whould it look lie, what is abnormal?
should be flat, single pulsation is bad, double pulsation is really bad.
normal ductus venosus pattern, change in disease state?
Norma: a wave flow not reversed
abnormal: (with anomolies, growth restriction) reduced, absent, reversed flow in atrial systole
whats does umbilical artery flow tell you?
placental vascular resistance: A goes below baseline with worse resistance
fetal Left to Right PFO flow?
L heart disease
fetal retrograde arch flow
left heart disease
MRI technique for imaging of constrictive pericardium?
spin echo for black blood, will be dark
regurgitant fraction cutoffs for pulmonary regurgitation in MR?
RF = retrograde volume / antegrade volume <20 = mild 20-40 = moderate >40 = severe
what things aren’t safe in the CMR magnet? which are?
prosthetic valves and steral wires safe, nonferromagnetic devices safe
wekaly ferromagnetic devices - wait 6 weeks
standard Pacemaker/ICD = unsafe
MRI compatible, conditionally safe
Nuclear imaging: how dose a lung scan work?
tecnetium label Albumin sphreses get stuck in capillary bed, gives you relative R/L flow
doesn’t work with streaming, fontans
which nuclea scan is used for Left to right shunt? right to left shunts?
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
limitations of thermodilution
shunts, TR, PI, ery low CO, unstable core temp
Fixk Eq:
Fick eq under 100% oxygen
Q = VO2 / [hgb x 13.6 x (high - low)]
denominator becomes Hgb x 13.6 x sat x PO2 x 0.03 - other
what is effective Qp?
flow of deoxigenated blood that goes through the lungs
In adults, what adult ASCVD score is significant, what would you do?
ASVCD score > 7.5 % -> start a statin
in which population is BNP a prognostic indicator?
heart failure
Eisenmenger: BNP >140 poor outcomes
pulm valvuloplasty Class I indications Class III indications predictions of good result balloon size risks
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
tretment for suicide RV
PGE, beta blocker, RVOT stent
when to do pulm valve eprforation:
TV Z score -2 o 0, tripartite RV, shot segment Pulm Atresia, no RV dependent coronary snusoids
aortic valvuloplasty Class I indications Class III indications predictions of good result balloon size risks
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
mitral valvuloplasty
class I indication
class III
best outcome in
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
branch PA angioplasty: class I poorly responsive balloon size goals:
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
branch PA stent
class I indication
method
risk
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
pulm vein stenting
class I indications
risk:
best outcome
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
coarctation angioplasty
class I indications
tech, what size balloon?
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
coarctation stent: class I indication
recurrent coarctation > 20 mmHg and can be expanded to adult size.
can you stent a coarctation in a teenager?
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
risk of complication in re-coarctation intervenion?
Balloon (44%)»_space;> surgery (25%) > stent (5%)
RV-PA conduit stenting class I indications
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
percutaneous pulmonary valve
“Class I indications”
use within conduits or bioprosthetic that are > 16 mm not native or patch augmented RVOT moderate+ regurgitation OR mean RVOT gradient > 35 mmHg
atrial septostomy
class I indications
technical considerations
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
ASD device closure class I indications
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)
device closure with absence of superior or posterior rim?
no concern for sinus venosus defect
anteroinferior = risk of primum
device closure with absence of anteroinferior rim?
anteroinferior = risk of primum
absent superior or posterior: concern for sinus venosus defect
which rims may be deficient in ASD device closure?
anterosuperior (aortic)
posteroinferior must be > 2-3 mm
all other must be > 5 mm
risk of erosion in ASD device clousre?
deficient anteriosuperiro rim (overall risk 0.1%)
other factors: device over-sizing, splaying, movement
s/p ASD closure, tamponade, what causes it?
erosion
s/p ASD closure, at 1 week develops headache, rash, fever
nickel allergy
occurs 2d-1m
headache, rash, dyspnea, fever, effusion
medical management of allergy
do you give aspirin after ASD closure?
yes ASpirin and SBE x 6m
PDA closure class I indications?
moderate-large with heart failure, FTT, pulm overcitculation, LA/LV dilation
subtypes of PDA A-E
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
when to use coils to close a PDA?
< 2.5 mm diameter
how long to use aspirain after PDA device?
trick, no aspirin becaue you want it to clot
lenth of SBE prophylaxis following any device cath implantation?
just say 6 months
length of Aspirin therapy post stent, device, or pulm valve?
device or stent: 6 months
PDA device: no aspirin
pulm vvalve: indefinitely
treatment for suicide RV?
PGE
beta blocker
RVOT stent
favorable anatomy for pulmonary perforation?
normal TV size (or mildly hypoplastic)
absence of RVDCC
least common fusion in BAV?
NL = note likely (1%)
RL: really lkely (80%)
RN: not really (19%)
aortic valvuloplasty makes moderate AI, what balloon size to try next?
TRICK! stop once moderate AI is created regardless of residual obstruction
balloon size in Aortic valvuloplasty?
0.8-1 x annulus
risk of suboptimal result in aortic valuoplasty?
< 3 months, > 40 years
undersized baloon, higher pre-dilation gradient, smaller annular Z score, valve calcifivation, unrepaired coarctation
prior procedure
when is branch PA angioplasty less likely to be successfu?
congenital rubella
williams syndrome
Alagille syndrome
long term success following branch PA dilation?
Rarely produces complete permanent resolution of obstruction
patient s/p branch PA balloon and stenting, tachypnea and cough?
reperfusion injury
will find focal pulm edema on CXR
treat with diuretics, oxygen and time
difference in heart failure in coarctation presentation: early vs. late what do you see on echo?
early: RV dilation
late: LV dysfunction, dilation
neonate with sepsis
systemic blood flow obstruction
acute increase in afterload, what does the PV loop do?
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)
chronic increase in afterload, what does the PV loop do?
in acute, PV loop becomes narrow and tall.
box shaped heart on CXR
ebstein’s
ebsteins displacement index, what is it?
> 8mm/m2
what is celermajer index?
[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"
which lesions is at risk for cirular shunt?
ebsteins, if you have PI:
PDA -> PI -> TR -> RA -> PFO -> LA, LV, Ao, PDA
tet spell treatmen
sedation, preload, beta blockade, phenylephrine
BAV, aortic stenosis and coarctation genes
NOTCH 1 (notch in the posterior aorta) SMAD6 (super mad 6 day old)
NOTCH 1 - gene puts you at risk for
BAV, aortic stenosis and coarctation genes
NOTCH 1 (notch in the posterior aorta)
SMAD6 (super mad 6 day old)
SMAD6 - gene puts you at risk for
BAV, aortic stenosis and coarctation genes
NOTCH 1 (notch in the posterior aorta)
SMAD6 (super mad 6 day old)
myosin heavy chain 11 mutation, put you at risk for which CHD?
PDA
Aortic aneurysm
11 looks like a vessel wall | |
ankyrin repeat domain mutation
TAPVR
ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)
platelet-derived growth factor receptor alpha mutation
TAPVR
ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)
TPAVR genes
ankyrin repeat domain mutation (failure to ANKYLOSE the LA and PVs)
platelet-derived growth factor receptor alpha mutation (platelets faile to combine)
PA/IVS, most important determinant, what cutoffs?
TV > -2.5: 2 ventricle
TV
DILV, most common aortic position?
left and anterior
Conduction system
- L looped: anterior to pulmonary outflow tract
- D looped RV: lateral to pulmonary outflow tract
cutoff for a small bulboventricular foramen?
<2cm2/m2 -> risk for late obstruction to outflow tract arising from BVF
<2cm2/m2
<2cm2/m2 -> risk for late obstruction to outflow tract arising from BVF
0.8-1
pulm balloon size
SVR trial # patients and time frame 1 year survival 3 year surival 6 year survival
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%)
enalapril in Infants with SINGLE Ventricle
ISV trial -
outcome?
administratio of enalapril did not improve somatic growth, ventricular function or heart failure severity
normal fontan by the numbers
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
FOntan IART, who is more likely to have it?
atriopulmonary»_space; ECC
5 year survival in PLE?
88%
treatment of PLE
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
In a VSD, what are the neurohormonal changes?
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
NKX2.5
ASD and conduction defects
Nick’s disease
GATA mutations
ASD association
Goetta is in the shape of a circle, so is an ASD
left hand bubble study, fills LA
unroofed coronary sinus
define partial AVSD
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)
define transitional AVSD
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)
define intermediate AVSD
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)
define complete AVSD
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)
when do you surgerize a partial AVSD?
elective > 1 year
when does the PDA close
term
preterm
<1000g
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
which is better for closing a PDA, ibuprofen or indomethacin?
same efficacy
indomethacin associated with renal disease
3 typs of AP windows
type I: proximal
type II: distal
type III: both (includes proximal branch PAs as well)
AP window associated with which IAA?
Type A = Berry Syndrome
Berry syndrome
AP window and IAA type A
which emb arches disappear?
1,2,5
normal right 4th aortic arch gives risk to?
3rd: CARotid
4th Rt SCA , leftaortich arch
6th: Ductus
double aortic arch, which is more likely dominant
Rt: 70%, lt 20%, codom 10%
descending aortia typicall opoosite to dominant arch
what is a cervical arch?
left 3rd arch persists and both 4th arches regress
pulsatile neck mass
rt ductus results in ring
aortic obstruction due to redundancy
pulsatile neck mass
concern for cervical arch
left 3rd arch persists and both 4th arches regress
rt ductus results in ring
aortic obstruction due to redundancy
barium esophagram: ant on trach, post on eoph
Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
aber subclav: posterior on esophagus
PA sling: anterior compression of esophagus
barium esophagram: ant on trach only
Innominate art: ant on trach
Double AoA: ant on trach, post on eoph
aber subclav: posterior on esophagus
PA sling: anterior compression of esophagus
barium esophagram: posterior on esophagus
aber subclav: posterior on esophagus
Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
PA sling: anterior compression of esophagus
barium esophagram: anterior compression of esophagus
PA sling: anterior compression of esophagus
Double AoA: ant on trach, post on eoph
Innominate art: ant on trach
aber subclav: posterior on esophagus
when do you replace an aneurysmal aorta in Marfans?
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
when do you replace an aneurysmal aorta in BAV?
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
when do you replace an aneurysmal aorta in LDS?
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
when do you replace an aneurysmal aorta in Turner syndrome?
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
CPR: goal coronary perfusion pressure
goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes
CPR: goal arterial diastolic pressure
goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes
CPR: goal ETCO2
goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes
CPR: goal defibrillation time
goal coronary perfusion pressure >20mmHg
arterial diastolic pressure > 30 mmHg
ETCO2 > 15 mmHg
Debrillation < 3 minutes
NEC risks (2)
< 30 days of age, ductal dependency
post op - bleed with low fibrinogen: treatment
low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical
post op - bleed with deficient clotting factors, treatment?
low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical
post op - bleed with normal coags, treatment?
low fibrinogen: cryoprecipitate
deficient clotting factors: FFP
normal coagulation: CT output > 10 cc/kg/hr x 2 hours = Surgical
post-pericardiotomy syndrome, when does it happen?
what lesions?
symptoms?
treatment?
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
fontan, what PEEP to use?
3-5 mmHg
if unable to extubate a fotna, post-op, what to consider?
VV collaterals
intubated glenn, what strategy to increase sats?
slightly inc PA CO2 by hypoventilation to inc cerebral blood flow and inc Pulm blood flow
definition of pulsus paradoxus?
> 10 mmHg drop in SBP with inspiration ina spontaneously breathing person
risk of being on both digoxin and lasix?
hypokalemia from lasix makes digoxin tricky
risk of lasix and NSAID?
NSAID makes lasix less effective
long term risks of lasix?
ototox, nephrocalcinosis in neonates
bioavailability of thiazide?
20%
med to correct secondary metabolic alkalosis?
acetazolamide: prox convoluted tubule
risks of carbonic anhydrase inhibitor?
sulfonamide reaction: SJS, TEN, hepatic necrosis, bone marrow suppresion
electrolyte abnormalities
PRIMACORP study:
what endpoint was improved with milrinone
by how much?
prevention of LCOS or death in first 36 hours post-op
placebo 27%
low dose 18%
high dose 9%
mechanisms of nitroprisside?
direct vasodilation and nitric oxide donor
patient with metabolic acidosis and normal mixed venous O2?
nitroprusside -> cyanide poisoning caused by tissue hypoxia (cytochrome oxidase inhibition = mitochondria poison)
Can happen with liver disease
risk of ARB and aldactone?
hyperkalemia
calcium channel blocker for a fib a flutter?
diltiazem (rate control)
drug of choice for HTN after coarctation repair?
propranolol: HTN from carotid baroreceptors, gets to the source
slows and strengthens the heart
digoxin
what drugs reduce clearance of digoxin?
amiodarone Ca channel blockers (verapamil) flecainide propafenone quinidine
scooped ST segments
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
what is nesiritide?
peptide and augments guan cylase receptor, inc cGMP, smooth muslce relaxation, dec BP and muscle relax
what is fenolopam?
potent DOPA-1 receptor agonist, augments urine output
downstream signaling of beta-adrenergic
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
downstream signaling of alpha-adrenergic
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
MOA Etomidate
caution with
potentiates gaba receptors
Caution: adrenal suppression -> blocks cortisol production pathway
may potentiate seizures in patients with known disorder
hemodynamic effects of inhaled anesthetics:
airway effects?
danger?
vasodilation causes dec BP, reflex tachycardia
airway: bronchodilation
danger: malignant hyperthermia
mechanism of malignant hyperthermia
treatment?
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
mech difference for roc/vec and succinylcholine?
succinylcholine is depolarizing and can cause rhaobdo, bradycardia, hyperthermia, hyperkalemia
roc/vec are nondepolarizing (bind choolinergic receptor)
what is protamine reaction?
bradycardia, hypotension, anaphylaxis that occurs after protamine is given (see it after surgery with protamine reversal)
LMWH PPx and Tx doses?
PPx 0.5 mg/kg/dose q12
Tx: start 1mg/kg/dose, then titrate
warfarin inhibits
2,7,9,10, C, S
how does TPA work?
binds fibrin, activates plasminogen which breaks down fibrin
Mech clopidogrel?
irreversible blocks GPIIb/IIIa (ADP receptor), inhibits platelet aggregation
what is RER, why does it increase suddenly at peak exercise?
RER = VCO2 / VO2
as lactate production increases, VCO2 is produces as buffering of HCO3
noninvasive surrugate of lactate threshold?
Ventilatory anaerobic threshold
VO2 =
VO2 = CO * oxygen content
= (HR * SV) * (a - v o2 diff)
when does stroke volume plateau in exercise?
40% of peak, then HR keeps going up
why does A-a O2 difference widen with increasing exercise?
Hgb dissociation: heat, acidosis, etc
equation for breathing reserve
(1 - max VE / MVV) * 100%
MVV calculated by hyperventilating for 10 seconds, multiple by 6
normal work rate during exercise?
3 watts/ kg for boys/girls
3.5 watts/kg for post-puberscent boys
normal BP response to exercise
SBP increases up to 40%
abnormal if it is blunted (< 20 mmHg) or falls
DBP should stable or fall
exercise recommendation for toddlers
for children
for adolescents
toddlers: > 3 hours/day
children/adolescent: > 1 hour
indications to restrict from exercise
1A
1A+1B
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
which wave corresponds to RVEDP in absence of TC stenosis?
a wave
(same with a wave of LA)
however pressure tracing in LA is v wave dominant (closed system)
what causes RA a wave to be higher? v wave to be higher?
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
max contrast dose
6cc/kg
what is the hepatoclavicular view on angiography? what is it good for? echo analogy?
45 LAO, 45 cranial
good for, crux of the heart, avv anatomy,
analogoes to apical 4 chamber view
rate of culture negative IE in kids? which organism?
5%, bartonella
how long does dental bacteremia last?
30-60 minutes
