Peds Test 6 Flashcards
Anomalous Pulmonary Venous Return
-pulm veins return to systemic venous return (due to abnormal development in first 8 weeks of embryo)
Total Anomalous Pulmonary Venous Return (TAPVR)
- all 4 pulm veins return to right heart= circles= O2 blood never goes to body, increasing RV vol
- symptoms= cyanosis, clammy, trouble breathing, tacky, thin, weak, irritable
- MUST have ASD
1) Supracardiac (52%)- has “vertical vein” which connects to SVC====DILATED SVC and vertical vein= SNOWMAN
2) Intracardiac (30%)- PVs drain into coronary sinus or RA
3) Infracardiac (12%)- PVs drain into IVC/portal vein, they can cross the diaphragm and become obstructive = CHF
4) Mixed (6%)- PVs enter at different locations
Obstructive TAPVR
WORST- true emergency
- PV runs thru diaphragm- squeezing vein and causing lung back up (increase RA,RV pressures)
- enlarged RA, more cyanosis, respirator distress
Partial Anomalous Pulmonary Venous Return (PAPVR)
- PVs return to both LA and RA
- with ASD (sinus venous or secundum)
- not as symptomatic
- Scimaitar Syndrome- right PV returns to IVC (passes thru diaphragm
Treatment Goals
1) connect PV to LA
2) stop connection of PV to systemic venous
3) close ASD
- -sometimes will do balloon atrial septostomy to open ASD more
TAPVR Repairs
- mostly repaired by attaching PV confluence to LA
1) supra cardiac- sew PV to LA and close ASD, 18 degrees
2) intracardiac- redirect flow, 28 degrees
3) Infracardiac- life heart to face, cut PV at diaphragm and attach to LA, 18 degrees
Recurrent Venous Obstruction
develops in 5-15% of patients
-repair with balloon angioplasty, patch, OR sutureless repair (for best results)= grows and won’t distort
CPB with APVR
cannulate- aortic and bicaval (PAPVR) or single atrial (TAPVR)
- may need ECMO pre and post
- the change in pulmonary flow can shock the body
Tetralogy of Fallot
1) Large VSD 2) Pulm Stenosis (RVOT obstruction)= R-L shunt
3) Overidding aorta (over the VSD)
4) acquired RV hypertrophy
- -truncus arteriosus divided by spiral septum (which isolates aorta/PA and ventricles
- -but with TOF, if it shifts right= aortic enlarged
- -shifts left= pulm artery enlarged
1) TOF with Pulmonary atresia (psedotruncus arterous)- no pulm outlfow, all blood goes thru aorta= lungs make collaterals
2) Pentaology of Fallot- TOF with ASD
- Symptoms= HIGH HCT cuz low saturation, cyanosis, clubbing, fatigue, passing out, TET spells (crying= increase CO2, decrease SVR= R-L shunt)
- squatting helps- increase aortic wave reflection= increase LV pressure= decrease R-L shunting
TOF Treatment
- palliative- BT or Central shunts (mostly for TOF with PA)
- full repair
1) receive RVOT stenosis
2) repair VSD - -For TOF with absent RV (dilated) or PA (hypoplasic)= Rastelli shunt, pacing, close VSD
TOF CPB
cannulation- aorta and bicaval
- mid hypothermia
- CPG antegrade, multiple doses cuz collaterals
- deal with high HCT
Double Outlet Right Ventricle (DORV)
PA and aorta both come from RV (with VSD L-R)
- symptoms- blue, clubbing, edema, dsynea= CHF or Pulm HTN
1) sub-aortic VSD= most common depends on Pulm Stenosis (increase cyanosis) , but no PS increase pulm flow= CHF, bad
2) sub- pulmonary VSD (Taussig- Bing)= transposed great arteries (PA receives LV oxygenated blood and aorta gets desaturated from RV)
3) doubly committed VSD= huge VSD and neither PA or aorta is favored
4) non-committed VSD= VSD so low in ventricle= LV hypoplasy= uni-ventricular treatment
DORV Treatment
1) anatomic- restores circulation with 2 ventricles
2) uni-ventricular= only 1 ventricle functional
- –
1) sub-aortic VSD- Intra-ventricular tunnel (LV- VSD- Aorta) and Rastelli for PS
2) sub-pulmonary VSD- intra-ventricular tunnel to Pa or aorta, close VSD to PA and atrial switch
3) Doubly committed VSD= intra-ventricular tunnel (LV to aorta), Rastelli for PS
4) Non-committed VSD= uni-ventricular repair: complex intra-vent tunnel (eventually to Fontan)
DORV CPB
cannulation- aorta and bicaval
- mid hypothermia
- CPG antegrade, multiple doses cuz collaterals
- deal with high HCT
- ECMO depending on lungs
Double Inlet Left Ventricle (DILV)
Both AV valves enter LV (which is connected to hypo plastic RV by VSD)
- symptoms= cyanosis, edema, CHF, tacy, dsypena,
- VENTRICLES are switched!!, TGA is common
- want PS= better circulation (No PS- pulm over circulation)
