Congenital Flashcards
JET
Hypothermia and atrial overdrive pacing can help. Avoid catecholamines,
If above fails, amiodarone is drug of choice
William’s Syndrome
Elivn faces/developmental delay
Supravalv AS (most common abnormality) - may progress or stay same. Balloon dilation not effective.
PS (second most common), often self resolves, but AS does not.
Also associated with coronary abnormalities (ostial stenosis), Aortic valve abn, peripheral arterial stenosis
CCTGA (L-TGA)
Just the ventricles are switched. AV valves stay with the ventricle
LA->TV->RV->Aortic/systemic -> RA->LV-> Pulmonic/PA
Often has VSD which allows for popoff of elevated pressures LV->RV
Often has complete heart block (situs solitus more common to have CHB)
If flow is balanced, no intervention and person may survive to adulthood.
Sinus of valsalva aneurysm
Rupture can occur most commonly to RV (60%) RA (30%)
Heart failure symptoms develop
Surgical Patch closure
TOF-APV
Absent pulm valve rare (5% TOF)
Often goes with PA aneurysms that can cause airway compression. this is most likely cause for emergent intervention after delivery.
PA plication and valve insertion
Warden procedure
Tx for anomalous PV draining in to SVC
Divide SVC above entry of PVs. Internal baffle connecting the PV drainage to existing ASD (to LA)
SVC reanastamosed to right atrial appendage
Drainage must be >2cm above RA and above azygous
DORV
Most common is sub aortic type - VSD is below aorta. LV blood streams across to aorta, RV blood goes to PA. Presentation depends on PA obstruction which may cause TOF presentation w cyanosis. Repair in first 6 months with intracardiac baffle/enlarge VSD to make passage.
Subpulmonary VSD variant - functions as TGA and pateitnns would get switch operation. If PS, do Rastelli/REV/Nikaidoh.
Non committed VSD - VSD is far from great vessels. very difficult to treat.
Early complications are due to problems with baffles. Later due to somatic growth/outflow obstructions
Rastelli Procedure
Used for transposition with VSD (Switch not doable in this case)
Patch baffle from LV through VSD to aorta. RV to PA valved conduit.
Most will need conduit replacement at some point
Long term sequelae - CHB, conduit failure, restenosis of LVOT
Norwood
First stage of Tx HLHS/TA/DORV
Atrial septectomy
Reconstruct atretic aorta/arch (patch).
Divide Main PA and Ligate
BT Shunt (Inominate to PA shunt) OR Sano (RV to PA)
Glenn
Second stage of Tx for HLHS/TA/DORV
SVC to PA (oversew RA)
Takedown of BT shunt
Fontan
IVC to PA with conduit
Can fenestrate the conduit to RA which will pop off if PA pressures high
HLHS
Due to any let sided lesion that prohibits blood flow/growth (Aortic/mitral stenosis/atresia).
Ductal dependent (for systemic circulation) and need ASD for PV return to mix. Start prostaglandin to keep ductus open
Prior to intervention, need to maintain balance from systemic/pulmonary. As PVR drops, can get overcirculation. Don’t want to drop PVR or create a large ASD which would cause overcirculation and hypoxemia
Norwood (3-5 days)
Glen (3-6 months)
Fontan (years)
Double Chamber RV
Septation in RV - low and hiigh pressure chamber
90% have VSD
If VSD connects to high pressure chamber, mimics TOF (Tet spells)
Surgery to remove septation
Anterior malalignment VSD
Associated with RVOT obstruction, underdeveloped RV, aortic arch hypoplasia or coarct
Pediatric cardiac tumors
Rhabdomyoma most common
Usually spontaneously regress but surgery to remove if causing hemodynamic effects (debulk)
Serial echos
Tuberous sclerosis complex causes multiple rhabdomyomas in different organs
HOCM
In kids, symptoms should prompt medical therapy (BB first line)
Refractory symptoms or Lvot>60 warrant surgery
Pda
Most close spontaneously by 2 months in full term babies so no intervention until then unless hemodynamic instability or symptoms
Premature babies often won’t close but still wait 2 months
Medical closure with indomethican (high tox) or ibuprofen. Fails in 10%
Coil occlusion if <3mm or device closure for larger than 3
Premature and symptomatic infants <6kg should get surgical closure
Role for Perc ASD closure
Secubdum defects up to 3.8 mm
Primum, venosus and CS have to be closed surgically
Cor triatriatrum
LA divided by fivromuscular septum that blocks off PV return from LAA/mitral.
Symptoms of pulmonary venous congestion
Treatment is surgical to remove septum
VSD
Spontaneous closure by 12-18 months is likely
After 2 years unlikely to close so repair then
Symptomatic patients should get closure after failed medical mgmt
For outlet VSD - approach through pulmonary arteriotomy
Typical approach to VSD is through RA via tricuspid
Prrimembranous or inlet VSD repair has risk to bundle of his
Coarctation
50-80% have bicuspid valve, 30-60% have VSD
55% present in first month of life as ductus starts to close.
Young children/adults may present with milder cases
Iv Pge to keep ductus open
Balloon angioplasty for critically ill neonates but has high recurrence
Stent placement comparable to surgery in older kids and adults so this is best treatment
For neonates or older children/adults that aren’t stent candidates, left posterooateral thoraco with end to end anastamosis is preferred tc
TOF
Usually has anterior malalignment VSD below overriding aorta
Associated with right aortic arch (25%), additional VSD (15%), aberrant coronaries (5%) - LAD from RCA coursing over RVOT is surgical risk
RVOT obstruction determines age of presentation
Severe RVOT obstruction presents early w closure of Ducati’s (first months of life)
Ideally delay tx until 4-6 months (can do balloon pulmonary valvuloplasty, BT shunt, stent ductus to get ti window)
If pulmonary Z score is less than -2, transannular patch +/- PVR
Treatment for TGA with PS
Can’t do normal ASO because there will be LVOT obstruction
Options instead -
Rastelli (if VSD is large enough, baffle flow from LV to aorta and Place RV to PA conduit)
REV - like rastelli but no conduit. PA bifurcation directly anastamosed to RV
Nikaidoh- transfer aortic root to LV
Ebsteins anomaly
Leaflets fail to delaminate
Presentation in neonate - RV failure and cyanosis
Do TV repair (monocusp repair)
If pulmonary atresia, will require single ventricle pathway (patch close tricuspid with a fenestration), enlarge ASD.
Kids and adults may present with heart failure and cyanosis, tachyarrythmia - indications for repair. Try to repair with Cone technique but replace with bio prosthetic if unable. If significant RV dysfunction, can do Glenn to create 1.5 ventricle repair
Ebstiens anomaly
If cyanotic newborn, with PS - have to do single ventricle repair.
Patch close TV (w fenestration) and do BT shunt
Right to left shunt ASD in newborn
Tapvr
