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Congential Heart Disease Flashcards

1
Q

Aortic Coarctation repair

A

Sx of systemic upper extremity HTN, drop in BP to LE, DOE

Peak gradient >20mm Hg
OR
<20mm Hg if presence of large collaterals on MRI/CT (echo may underestimate gradient with small passage)

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2
Q

Shunt repair

A

No repair if PA pressure/systemic pressure >2/3 (vasodilators can be used to bring down PAP to meet goal)

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3
Q

PDA with eisenmengers

A

reversal of shunt in descending Ao (pink fingers, blue toes) ie differential cyanosis
(pt with unrepair TOF have cyanosis of upper and LE)
(Pt’s with coarcation and anomaloous return of PV do not have cyanosis)

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4
Q

Pulmonary HTN from shunt

A

most from L->R shunts DISTAL to TV (ie VSD, PDA) morning

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5
Q

Cyanosis/eisenmengers

A

only with lesions below TV (not ASD, PFO, anamolous return of PV)

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6
Q

Ebstein’s anamaly

A

Severe TR (holosystolic murmur)
Apical displacement of septal TV leaflet
redundant, elongated sail like anterior leaflet with sail sign (mid systolic click)
TV displacement of 8mm/m^2
ASD/PFO in 50% +- bidirectional shunting, paradoxical emboli, cyanosis on exertion
WPW in 20% (or some sort of SVT)
atrialized RV (tissue properties of RV but is part of atrium)
Regurgitant TV

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7
Q

TOF

A

Have deg of PS or even PA atresia
(two variations - with pulmonary stenosis or with pulmonary atresia (rare form) - with pulm atria must have VSD and and aorto-pulmonary shunt)
-need to replace the RV->PA conduit

s/p blalock shunt, s/p difinitive repair p/w severe PR, dilated RV
-pulmonary valve replacement

Resting QRS >180 a/w risk of sudden death
RBBB/iRBBB common

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8
Q

Initial palliative TOF repair

A

1) Waterson shunt - Asc Ao->RPA
2) Potts shunt - Dsc Ao-> LPA
3) Classic Blalock-Taussig shunt - L SC->LPA
4) Modified Blalock-Taussig - R SC-> RPA

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9
Q

Definitive repair

A

VSD patch repair
Resection of RVOT obstruction, annular patch
No patch if LAD arises from RCA or RCC - need PA->MPA shunt conduit instead
QRS>180ms bad prognosis

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10
Q

Ebstein’s anamaly tx

A

NYHA III-IV Sx with severe TR -> Class I TV repair/replacement

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11
Q

Sinus Venosus ASD

A

a/w Ananamalous pulmonary venous return (RUPV) - superior sinus venosus ASD has RUPV->SVC
Needs surgical closure

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12
Q

Primum ASD

A 
Cleft mitral valve
VSD
Down syndrome (Trisomy 21)
PE
Fixed split S2
flow murmur (pulmonic flow)
MR murmur - systolic best heard near nipple radiating to axilla
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13
Q

Secundum ASD

A

only ASD that can be device closed
Ebstein’s anomaly
HOLT ORAM (heart hand) sydrome - upper limb abn, missing digits - arm shorter than other
family hx +

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14
Q

Partial Anamolous Pulmon Vein return

A 
unexplained right side vol o/l
neg bubble study if no ASD (if no ASD can still cause RH enlargement and pulm HTN)
RH enlargement
RUPV->SVC/RA
RLPV->IVC (scimitar syndrome)
LUPV->inominate vein (vertical vein)
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15
Q

Turners (45XO)

A

Bicupid AV

Ao Coarct

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16
Q

Dilated CS

A

confirm by saline injection into left antecubital vein or imaging of L SVC

17
Q

Pericardial cyst

A

anterior to RA/RV

18
Q

Partial AtrioVentricular Septal Defect

A 
s/p repair
usually MR with holosytolic murmur
ASD+VSD (primum ASD)
Cleft MV (causes MR and holosystolic murmur - VSD/ASD repaired but cleft MV remains)
a/w Downs (Trisomy 21)
19
Q

ASD Closure

A

QP:QS>1.5-2 with vol o/l signs (right heart enlargement)

If pulmonary HTN - carefully consider whether to close or not - do not close if PVR>7 W.U.

20
Q

ASD Sx

A 
Fixed split S2
Pulm outflow murmur
RBBB
RAD
RAE
21
Q

Noonan’s syndrome

A 
Pulmonary stenosis 2/2 dysplastic valve
high pitched systloic click
cresendo decrescendo murmur 
S2 widely split (wider = worse)
delayed S2 2/2 prlonged ejection time from PS
lengthens and peaks later with worse obstruction
developmental delay
short stature
predilection for leukemia
22
Q

PFO

A

~25% population

23
Q

PFO etiology

A

1) First 2 months dev crescent wall (septum primum) divides LA/RA with hole called ostium primum
2) Eventually crescent wall closes off ostium primum -> 2nd hold forms in septum primum - ostium secundum
3) 2nd septum (septum secundum) grows and partially covers ostium primum - hole called fossa ovalis
4) IVC blood directed across fossa ovalis via eustacian valve in RA
5) Birth - LAP rises as lungs perfuse -path through foramen ovales forced shut by LAP>RAP (was held open as RAP>LAP - kept flap of septum secundum open.
6) Septal aneurysm makes potential path more likely to open

24
Q

Shone complex

A

Left heart obstructive dz

supravalvular MS, Parachute MV, coarctation of Ao, subvavlular AS, AS

25
TOF
Coronary anamoly - LAD from RCA - relevant if surgical procedure at RVOT -> can ligate LAD -> instead of RVOT patch can use RV->MPA conduit
26
Benign coronary anamoalies
1) Seperate origen of right conus artery 2) Seperate ostia of LAD and Cx (no LM) 3) Origen of LCx from RCA or right sinus of valsalva (coursing posterior to aorta)
27
D-TGA
origin of RCA from posterior CC | origin of both RCA/LCx posterior CC
28
QP:QS Calculation
MVO2=(3*SVC)+IVC/4 big artery-big veins lung artery-lung vein FA-MVO2 -------------- PV-PA
29
L-TGA
RCA from ANTERIOR CC systemic RV dysfxn heart block 2/2 unusual location of AV node and HIS bundle -> high grade AVB possible VSD
30
Eisenmengers
cyanosis, R->L shunt usually from defect distal to TV ie VSD, PDA, ASVD full unlikely with TOV even with VSD because subpulmonic stenosis protects pulm circ from inc'd pressure
31
L-TGA - congentially corrected TGA
can present in adulthood if no other associated lesions | anteriorly placed conduction system - incidence of CHB high (2%/year)
32
Tricuspid atresia s/p fontan
Protein losing enteropathy edmema, pleural effusion, low albumin, diarrhea Dx: Check stool alpha 1 antitypsin level
33
Tricuspid/pulmonary atresia repair
Glenn - SVC->disconnected RPA (this didn't work as well beacuse encouraged right lung pulm AVM and cyanosis) OR Bidirectional Glenn -> main PA trunk disconnected -> SVC-> RPA (now SVC blood to both lungs) NEXT: Fontan - IVC blood to lungs: 1) Classic - RAA->main PA (no glenn performed here -> all blood (SVC&IVC) go into RA->RAA->main PA) 2) Tunnel from IVC to RPA - offset to prevent competitive flow with glenn - lung not used to all this flow and can cause initial pulm HTN/resisteance - back pressure on fontan tunnel may be high -> lateral tunnels are often fenestrated into RA along way to allow for pop-off valve to relieve some of back pressure - later once lungs get used to pressure and pulm resistance lowers fenestrations are closed with closure device (fontan hemodyn can fail with any cause of pulmn HTN because relies on systemic venous pressure exceeding PAP higher prssure in hepatic vein from backflow can cause cirrhosis and HCC Poor toleration of atrial arrythmias coagulaopahty PLE
34
VSD Repair
``` PVR=(mPAP-PCWP)/CO normal ~1.5 (still L->R shunting) Check QP:QS - pulm blood flow : systemic blood flow vol o/l on echo (enlarged RV chambers) PVR:SVR >2/3 high risk for closure VSD ``` When PVR inc's with time with VSD -> R->L shunting/eisengengers, pulm/syst pressure ratio inc's cyanosis -> higher risk for closure
35
Classic fontan
Direct connection RA-> PA (Modern operations ie lateral tunnel or extracardiac conduit exclude RA) Significant risk of atrial arrythmias - interatrial re-entrant tachycardia - fibrosis from surgery DCCV for tx - TEE r/o atrial thrombus Work up for FONTAN obstruction if recurrent - antiarrythmics or ablation can do surgical revision of fontan
36
ASD
PE TTE bubble study - need valsalva to make RAP>LAP Fixed split S2 Pulmonic outflow murmur (soft systolic ejection murmru) ``` If large RH enlargement iRBBB inc'd pulmonary vascularity pulm HTN ``` ``` Repair considerations RH enargement without other explanation if pulm HTN need to carefully consider early repair better less atrial arrythmias less eisenmengers ```
37
Aoritc coarct
``` brachiofemoral pulse delay Rib notching (enlargement of collaterals) ```
38
Coronary artery fistula
Closure (surgical or perc) Class I - large fistulae (asx) -small fistula if documented ischemia, arrythmias, syst/diast dysfxn, chamber enlargement, endartitis
39
ASD repair
QP:QS>1.5 and RH chamber enlargement If QP:QS<1.0 then right to left shunting (Eisengengers) and should not be closed

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