Congenital

Question 1

JET

Answer 1

Hypothermia and atrial overdrive pacing can help. Avoid catecholamines,

If above fails, amiodarone is drug of choice

Question 2

William’s Syndrome

Answer 2

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

Question 3

CCTGA (L-TGA)

Answer 3

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.

Question 4

Sinus of valsalva aneurysm

Answer 4

Rupture can occur most commonly to RV (60%) RA (30%)

Heart failure symptoms develop

Surgical Patch closure

Question 5

TOF-APV

Answer 5

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

Question 6

Warden procedure

Answer 6

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

Question 7

DORV

Answer 7

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

Question 8

Rastelli Procedure

Answer 8

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

Question 9

Norwood

Answer 9

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)

Question 10

Glenn

Answer 10

Second stage of Tx for HLHS/TA/DORV

SVC to PA (oversew RA)
Takedown of BT shunt

Question 11

Fontan

Answer 11

IVC to PA with conduit

Can fenestrate the conduit to RA which will pop off if PA pressures high

Question 12

HLHS

Answer 12

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)

Question 13

Double Chamber RV

Answer 13

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

Question 14

Anterior malalignment VSD

Answer 14

Associated with RVOT obstruction, underdeveloped RV, aortic arch hypoplasia or coarct

Question 15

Pediatric cardiac tumors

Answer 15

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

Question 16

HOCM

Answer 16

In kids, symptoms should prompt medical therapy (BB first line)
Refractory symptoms or Lvot>60 warrant surgery

Question 17

Pda

Answer 17

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

Question 18

Role for Perc ASD closure

Answer 18

Secubdum defects up to 3.8 mm
Primum, venosus and CS have to be closed surgically

Question 19

Cor triatriatrum

Answer 19

LA divided by fivromuscular septum that blocks off PV return from LAA/mitral.

Symptoms of pulmonary venous congestion

Treatment is surgical to remove septum

Question 20

VSD

Answer 20

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

Question 21

Coarctation

Answer 21

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

Question 22

TOF

Answer 22

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

Question 23

Treatment for TGA with PS

Answer 23

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

Question 24

Ebsteins anomaly

Answer 24

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

Question 25

Ebstiens anomaly

Answer 25

If cyanotic newborn, with PS - have to do single ventricle repair.

Patch close TV (w fenestration) and do BT shunt

Question 26

Right to left shunt ASD in newborn

Answer 26

Tapvr