Congential Heart Disease Flashcards
Aortic Coarctation repair
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)
Shunt repair
No repair if PA pressure/systemic pressure >2/3 (vasodilators can be used to bring down PAP to meet goal)
PDA with eisenmengers
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)
Pulmonary HTN from shunt
most from L->R shunts DISTAL to TV (ie VSD, PDA) morning
Cyanosis/eisenmengers
only with lesions below TV (not ASD, PFO, anamolous return of PV)
Ebstein’s anamaly
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
TOF
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
Initial palliative TOF repair
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
Definitive repair
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
Ebstein’s anamaly tx
NYHA III-IV Sx with severe TR -> Class I TV repair/replacement
Sinus Venosus ASD
a/w Ananamalous pulmonary venous return (RUPV) - superior sinus venosus ASD has RUPV->SVC
Needs surgical closure
Primum ASD
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
Secundum ASD
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 +
Partial Anamolous Pulmon Vein return
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)
Turners (45XO)
Bicupid AV
Ao Coarct
Dilated CS
confirm by saline injection into left antecubital vein or imaging of L SVC
Pericardial cyst
anterior to RA/RV
Partial AtrioVentricular Septal Defect
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)
ASD Closure
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.
ASD Sx
Fixed split S2 Pulm outflow murmur RBBB RAD RAE
Noonan’s syndrome
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
PFO
~25% population
PFO etiology
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
Shone complex
Left heart obstructive dz
supravalvular MS, Parachute MV, coarctation of Ao, subvavlular AS, AS
TOF
Coronary anamoly - LAD from RCA - relevant if surgical procedure at RVOT -> can ligate LAD -> instead of RVOT patch can use RV->MPA conduit
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)
D-TGA
origin of RCA from posterior CC
origin of both RCA/LCx posterior CC
QP:QS Calculation
MVO2=(3*SVC)+IVC/4
big artery-big veins
lung artery-lung vein
PV-PA
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
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
L-TGA - congentially corrected TGA
can present in adulthood if no other associated lesions
anteriorly placed conduction system - incidence of CHB high (2%/year)
Tricuspid atresia s/p fontan
Protein losing enteropathy
edmema, pleural effusion, low albumin, diarrhea
Dx: Check stool alpha 1 antitypsin level
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
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
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
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
Aoritc coarct
brachiofemoral pulse delay Rib notching (enlargement of collaterals)
Coronary artery fistula
Closure (surgical or perc)
Class I - large fistulae (asx)
-small fistula if documented ischemia, arrythmias, syst/diast dysfxn, chamber enlargement, endartitis
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
