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Understand how to calculate the estimated left to right shunt for an ASD

Aortic Saturation - SVC saturation/ Pulmonary venous saturation - Pulmonary artery saturation

A 2:1 shunt should be repair with elevated PA pressure


What are two most appropriate or preferred repair techniques for coarctation in the neonatal age group

Extended end to end anastomosis

left subclavian flap


What are the 3 most appropriate or preferred repair techniques for coarctation in the adult age group

Interposition graft
resection and end to end anastomosis
balloon dilation with intralumenal stent


List 5 complications of coarctation surgery

phrenic nerve injury/palsy
Horner's syndrome
post operative/residual hypertension
recurrent coarctation
Postcoartecomy syndrome/abdominal pain/mesenteric arteritis


Describe 3 features that preclude percutaneous ASD device closure

Location of ASD
primum defect
sinus venosus defect
deficient spetal rim
Size of ASD
a defect over 2.5 cm
Associated anomalies
azygous continuation
partial anomalous venous return
pulmonary hypertenion


List 5 complications of ASD closure

Residual shunt
migration of shunt
obstruction of coronary sinus
obstruction of pulmonary veins
thromboembolic event
cardiac chamber performation


List 6 potential causes of hypoxemia folllowing bidirectional cavopulonary anastomosis

pulmonary artery stenosis
stenotic anastomosis
elevated pulmonary vascular resistance
cardiac tamponade
left ventricular dysfunction, pulmonary edema
atrioventricular valve dysfunction/mitral regurgitation
runoff through venous collaterals- azygos vein
excessive PEEP
Vq.Q mismatch, atelectasis


Describe the anatomical features/locations of the following types of ventricular septal defects:
Perimembranous subaortic ASD
Inlet VSD
Muscular VSD
Doubly comitted subarterial VSD

Perimembranous VSD: located in perimembranous septum under the commissure of the anterior and septal leaflet of the tricuspid valve.
Inlet VSD: located in the inlet of the tricuspid valve, under the commissure of the septal and posterior leaflets.
Muscular VSD: defect is completely surrounded by muscular rim and can be located anywhere in the interventricular septum
Doubly comitted subarterial VSD: there is fibrous continuity between the leaflets of the pulmonary and aortic valves because of the absence of the infundibular septum


Describe the pathophysiology of subvalvular subaortic stensosis

Fixed fibromuscular discrete membrane.
Tunnel diffuse subarotic stenosis
Hypertrophic cardiomyopathy (IHSS)
Abnormal insertion of mitral valve chordae
Hypoplastc aortic valve ring


List 4 complications of subaortic stenosis repair

Residual obstruction
heart block
ventricular septal defect
aortic valve tearing


What is natural history of perimembranous VSD if left unoperated

Progressive RV enlargement
Congestive heart failure
prolapse aortic valve and aortic valve insufficiency
risk of endocarditis
adult pulmonary hypertension


Patient wishes to have device closure of VSD . What anatomtomicl structures are importance to assess before closure

Tricuspid valve
aortic valve
size of defect
distance from aortic valve to superior edge of defect


What are complications following closure of perimembranous VSD device closure

Left ventricular outflow tract obstruction
residual shunt
device embolization/migration
tricuspid/mitral/aortic valve regurgitation
Arrhythmia/heart block


List the 5 complications or pitfalls specifically associated with surgery for Ebstein's anomaly

Injury to the AV node or heart block
Injury to the right coronary or posterior descending coronary artery
residual right ventricular outflow tract obstruction
residual atrial septal defect
tricuspid stenosis
residual tricuspid regurgitation


3 year old presents with a right sided effusion 4 weeks post Fontan procedure

List 4 criteria that would strongly suggest the effusion represents a chylothorax

presence of chylomicrons
WBC > 1000
Lymphocyte > 80%
Triglyceride > 1mM


List 4 treatment options in the sequence that normally would be employed for chylothorax

Pleural drainage
NPO or low fat or medium change triglyceride diet
total parenteral nutrition
surgical ligation of thoracic duct
octreotide treatment


List 5 complications of unrepaired congenital ventricular septal defect

Congestive heart failure
Aortic insufficiency
Pulmonary hypertension (Eisenmenger's syndrome)
right ventricular outflow tract (RVOT) obstruction
severe failure to thrive


Start CPB and the PA pressure remain high? What is this? what can be done?

Likely a patent ductus arteriosus, so quickly gain control of the ductus and ligate it.

Alternatively, clamp the distal left and right pulmonary arteries and then ligate ductus


Classify interrupted aortic arch

Type A: Interruption distal to left subclavian
Type B: interruption between left subclavian and left common carotid (most common)
Type C: interruption between innominate and left common carotid artery


What are associated lesions with interrupted arch

Isolated VSD (most common)
Truncus arteriosus
transposition of great arteries with VSD including taussig-bing anomaly
aortopulmonary window
single ventricle
subaortic obstruction


What is DiGeorge Syndrome? What 2 things must be done postop?

No thymus. Watch of this when doing sternotomy.

Monitor Calcium level
Blood products must be irrigated


In a 30 year adult should you close an ASD?

Yes, reduces the risk of paradoxical embolism but this controversial.
Yes, because of mortality benefit if closed before age of 40?


New born presents with extreme cyanosis (arterial saturation 45%) that occurs 2 hours after birth. ET and positive pressure ventilation with an FiO3 of 1.0 results in minimal improvement in oxygenation. What are 4 likley diagnosis?

Transposition of Great Arteries
Pulmonary artery atresia with or without VSD
Obstructed total anomalous pulmonary venous drainage
tricuspid atresia


What is immediate pharmacological treatment for a new born in extreme cyanosis

PGE1 infusion should be started immediately to restore ductal patency. Most important thing regardless of diagnosis!


What is surgical treatment for TGA

Definitive operation Arterial switch is performed early (some in first week but most 2 to 4 weeks of life).

If delayed presentation then primary arterial switch is not likely to succeed as the drop in pulmonary pressure causes involution of the left ventricle making it unlikley to be able to function immediately. The right ventricle cannot sustain the required systemic pressure.

The two options for this patient would be pulmoanry artery banding to prepare the RV for systemic pressure or perform an atrial switch. (Mustard or Senning).


What are classical infow and outflow "from" that constitutes the collateral circulation around a classical coarctation of the aorta in an adult partnre]

Internal mammary artery
vertrbral artery
costocervival artery
scapular vessels
thryocervival trunk
superior epigastric/inferior epigastric
first two pair of intercostal arteries distal to coarctation (3rd and 4th intercosteal arteries)
Descending aorta


What is most common aortic arch congenital anomaly

anomalous origin of the right subclavian artery


List 10 complications of PDA closure

recurrent laryngeal nerve injury
ductal recanalization or persistent ductal patency
left pulmonary artery occlusion
accidental aortic ligation
esphageal injury
pulmonary injury
vagus nerve injury
phrenic nerve injury


4 week old baby girl presents with cyanosis with oxygen saturation at 68% on room air. Investigations show tricuspid atresia , transposition of great arteries, and severe pulmonary stenosis. List the 3 operations that would be used for her staged repair and the age at which each should occur.

Blalock-Taussig shunt (or central shunt) to control cyanosis in newborn period

Bidirectional Glenn anastomisis at 6 to 12 months of age (SVC onto the Pulmonary artery)

Fontan operation at 3 years of age after the child is allowed to grow (Lateral tunnel or extraanatomic bypass from IVC to PA.


what are the 3 most common cardiac causes of cyanosis in a newborn

Tetralogy of Fallot-particulary with pulmonary stenosis
Tricuspid atresia and pulmonary stenosis


list anatomical information needed for repair of TOF

The number, size,and location of VSD
The size and morphology of the pulmonary arteries (confluent, presence of MAPCAS)
presence of anomolous coronary artery (typically LAD from RCA)
size of pulmonary annulus
presence of a PDA/ASD


5 week female with TGA presents post balloon septosotomy. What is main concern for proceeding with arterial switch?

What are two other surgical options?

The ability of an unprepared left ventricle to maintain flow to the high pressure systemic circulation

Atrial repair (mustard or senning)

pulmonary artery banding with or without a BT shunt


List 4 anatomic type of vascular rings

double aortic arch
right aortic arch with left ligamentum artiosum
anomalous innominate artery and compression syndrome
anomalous right subclavian artery (dysphagia lusoria)
pulmonary artery sling


Which type of vascular ring is characterized by an anterior indentation in the esphagus on barium swallow

pulmonary artery sling: the left pulmonary artery passes anterior to the esophagus while all other vascular rings run posterior


what are 3 main components of Rastelli repair for TGA with left ventricular outflow tract obstruction

Tunneling a VSD to aortic root
RV-PA conduit
Closure of main PA
Closure of ASD


15 year old who had TOF with transannular patch. List 4 tests you require to assess cardiac function

Cardiac MRI
exercise stress test
CT angiogram


List 5 criteria for pulmonary valve implantation

Free or severe Pulmonary insufficiency
symptoms of decreased exercise tolerance
progressive RV dilation
decreased exercise tolerance on exercise test
pulmonary artery bifurcation residual stenosis
new onset of TR in the presence of severe pulmonary insufficiency
QRS > 180 msec


7 day old comes to ER respiratory distress, Systolic ejection murmur, abstent femoral pulses, pulmonary edma, severe metabolic acidosis.

List 4 cardiac manifestations

Critical coarctation with VSD
Critical Aortic stenosis
Interrupted aortic arch
hypoplastic left heart syndrome


List treatment plan for 7 day old presenting with severe metabolic acidosis

PGE1 infusion (0.05 mcg/kg/min to .1 mcg/kg/min)
intubation and ventilation
inotropic support
correction of metabolic acidosis


10 month old with TOF, showing hypoplastic pulmonary valve annulus measuring 6 mm with a main pulmonary artery at 6mm. Describe the main components of the operative procedure

Closure of VSD
resection of infundibular pulmonary stenosis
placement of transannular pericardial patch


Briefly describe the different types of total anomalous pulmonary venous connections

Supracardiac-- connected by a vertical vein usually to innominate vein

Cardiac--directly into the coronary sinus

infracardiac- to the hepatic vein or the IVC

mixed type: with some veins draining by a vertical vein to the innonminate vein and the rest into the coronary sinus


How to calculate Pulmonary vascular resistance

PVR = mean PA pressure - Wedge (pressure in left atrium)/ Cardiac output. It then equals woods units

do not close thus shunt as PVR is too high resulting in R to L shunting. ASD closure likely to result in right heart failure.


List 4 characteristics of scimitar syndrome

partial anomalous pulmonary venous connection
systemic pulmonary arterial blood supply
right pulmonary artery hypoplasia
right lung hypoplasia or bronchopulmonary sequestraction


List associated lesions that adversely affect the natural history of atrioventricular canal defects

coarctation of the aorta
patent ductus arteriosus
tetralogy of fallot
ventricular hypoplasia


4 month old undergoes uneventful repair of complete AV canal. 6 hours post there is hypotension and elevated left atrial pressure, oilguria, metabolic acidosis.

List 4 reasons for the patients poor status

cardiac tamponade
acute mitral regurgitation
residual ventricular septal defect
undiagnosed coarctation


List 3 morphological factors that determine the feasibility of biventricular repair in an infant with pulmonary atresia and intact ventricular septum

size and competence of tricuspid valve
size and type of right ventricular cavity (Z score of Tricuspid annulus)
presence (or absence) of right ventricle to coronary artery fistula
size and confluence of pulmonary arteries


What are the types of vascular rings

Complete ring
a Double arch: right or left dominant, balanced
b. right arch/left ligamentum
c. right or left arch dominant
Incomplete ring
d.left arch aberrant RSCA
e. innominant artery compression syndrome
f. pulmonary artery sling


Review fetal circulation

Well oxygenated blood returns from placenta to umbilical vein
IVC receives all the blood from the placenta--50% goes to hepatic sinusoids and 50% bypasses the liver through the ductus venosus to the IVC
Then from the IVC---RA---through the PFO and on to the LA-LV--and to systemic flow.


What are types of coarctation

Preductal coarctation (5% of infants with Turner Syndrome)

Ductal (usually occurs when the ductus arteriosus closes)

Post ductal coarctation: The narrowing is distal to the insertion of the ductus. Most common in adults


List the classification for congenital ventricular septal defects

Conoventricular (membranous)
Conal (outlet)
Inlet (AV canal)
Muscular (VSD)


Describe conoventricular defects

Most common, 80% of all VSD
located between conal septum and ventricular septum
exclusively in the membranous septum at the anterior-septal commissure of the tricuspid valve


How do you measure the magnitude and direction of the shunt across a VSD

Large VSD offer little resistance to blood flow and labelled "nonrestrictive".
Right ventricular pressure equals the left ventricular pressure

Small VSD do offer resistance to flow and labelled "restrictive"

Moderate are Qp:QS of 2.5:1 and 3:1


Natural history and indications for VSD closure

approximately 30% develop severe symptoms of CHF and need surgery in first year

Aggressive medical management is indicated in remained membranous and muscular VSD tend to close spontaneously. Asymptomatic with small restrictive VSD can be followed

Malaliignment and or inlet VSD are unlikely to close and closure at time of diagnosis is recommended.


What are other indications for VSD closure

Development of pulmonary vascular resistance
PA pressure greater than one half the systemic pressure in a child older than 1 year
A small proportion of patients with membranous or outlet VSD develop a prolapse of an aortic cusp into the VSD and need surgery


What are surgical approaches for VSD

For conoventricular VSD---approach through the right atriotomy
retract the anteriorseptal commissure
(can detach the septal or anterior leaflet by making incision that paralles the tricuspid annulus)
Class with patch.
Can do infundibular incision
Transventricular approach for conal septal


What are lesions of Ebstein's anomaly

1. adherence of the triscupid leaflets to the underlying myocardium (failure of delamination
2. downward/apical displacement of the functional tricuspid annulus
3. dilation of the atrialized portion of the right ventricle
4. redundancy, fenstrations, and tethering of anterior leaflet
5. dilation of the atrioventricular junction


What is Carpentier classification of Ebsteins

a reproducible classification is not possible because of variability.

There is a Carpentier classifcation in the literature.


What are selection criteria for the Fontan procedure

Initially for tricuspid atresia
1. minimal age 4
2. sinus rhythm
3. normal caval drainage
4. right atrium normal pressure
5. mean pulmonary pressure < 15 mmhg
6. pulmonary artery resistance < 4
7. no impairing effects of previous shunts
8. competent atroventricular valve
9. normal ventricles
10. pulmonary-artery-to aorta-diameter ratio of > 0.75


What is Fontan circulation

pulmonary and systemic blood flow are in series with the single ventricle connected to the systemic circulation. Venous drainage bypasses the right heart and enters directly into the pulmonary artery


What are components of complete AV canal defect

Primum ASD
Unrestrictive inlet VSD
Common atrioventricular valve


What cardiac lesions is Down Syndrome associated with

Complete AV canal defect--50% of DS

75% of complete AV Canal defects have DS

Rare for a partial AV canal defect to be associated with DS


What are the 3 types of complete AV canal Defect

Rastelli A (75)
Rastelli B --very rare
Rastelli C -associated with TOF

Appears to be based on the superior common leaflet.


What lesions are associated with Coarc

Hypoplasia of arch


List congenital lesions of mitral valve

Anomalous pulmonary venous drainage (total or partial)
Mitral stenosis--supravalvular and parachute
Mitral atresia


What is mustard procedure

1. pericardial patch is sutured to direct drainage of the pulmonary veins in the pulmonary venous atrium and outflow through the tricuspid valve into the RV and out into the aorta for systemic flow.
2. The SVC, IVC, and CS are redirected to drain into the systemic venous atrium and outflow through the mitral valve into the LV which then connects to the pulmonary artery and lungs.


What is congenitally corrected Transposition L-TGA

1.The morphologic RV is the systemic ventricle and gives rise to the aorta.
2.The morphologic LV is the venous ventricle and gives rise to the PA
3. atrioventricular discordance and ventrcul-arterial discordance.
4. Aorta is anterior to PA - L-Looping TGA


List all the cyanotic lesions

Tetralogy of fallot
Tricuspid atresia
Transposition of great arteries
Total anomalous pulmonary venous return
Truncus arteriosus
hypoplastic left heart syndome


Name all the acynaotic lesions

atrial septal defect
ventricular septal defect
patent ductus arteriosus
atrioventricular canal
aortic stenosis
pulmonary stenosis


What is natural history of ASD

Spontaneous closure (85-90) within the first few years but rare to close after age 3-4

untreated, the average life expectancy is 40 to 50 years old

cause of death: CHF, PHTN, arrhythmia/sudden death


Investigations for ASD

ECG-Axis deviation, atrial arrhythmia
CXR- Vascularity
ECHO- ASD location, seixe, RV size, PAP, QP/QS, associated lesions
TEE: Device closure/sinus venosus
CATH: PVR, Device Closure, CAD


What are class I indications for ASD closure

Hemodynamically significant +/- symptoms (Qp:QS> 1.5)
Large secundum (>38 mm) not amenable to device closure
percutaneous closure
Sinus venosus/primum ASD cannot be closed by devices; should be repaired by congenital CVS
If AF/futter occurs, anticoagulation
atrial arrythmias: rate or rhythm control


When NOT to operate for ASD

PVR > 8 woods units and No response to vasodilators
If the L-R shunt Qp/QS is < 1.5


What are class IIa indications for ASD closure

1. paradoxical emboli
2. orthodeoxia-platypnea---clinical syndrome characterized by dyspnea and deoxygenation accompanying a change to sitting or standing
3. Undergoing TV repair/replacement
4. Cath ablation prior to device; Maze at ASD surg closure
5. Transvenous pacing avoided with unrepaired ASD
6. pHTN present and L-R shunt >1.5 or reactivity; care from a specialist


What are complications of DEVICE placement for ASD

Migration of device
Residual shunt
thromboembolic event
obstruction of coronary sinus
obstruction of pulmonary veins


Post op surgical closure of ASD complications

residual ASD
Arrhytmias--increased rates of SVT (AF/Aflutter)
Pericardial effusion or tamponade


What are associated anomalies for VSD

PDA (25%)
Aortic coartaction (10%)
LVOT obstruction (4%)
PFO/ASD, vascular ring, PLSCA


What is natural history of VSD

Close spontaneously: 24-40% by age 2 and 80% by 10 years of age

If unrepaired the subarterial and perimembranous VSD can lead to
b. Aortic insufficiency
c. Arrhythmia
e. Pulmonary vascular resistance


What are class I indications for VSD repair (CCS ACDS)

1. Significant VSD
deteriorating ventricular function due to volume (LV) or pressure (RV) overload
Qp/Qs > 2
Pulmonary artery systolic pressure > 50mmHg
2. Significant right ventricular outflow tract obstruction
cath or mean echo gradient > 50 mmHg
3. PM or subarterial VSD with greater then equal to mild Aortic insufficiency
4. Severe Pulmonary hypertension PAP > 2/3 MAP or PVR >2/3 SVR---net L-R shunt > 1.5 or PA reactivity


What are class IIa indications to repair a VSD

Before transvenous pacing
isolated trabecular muscular remove from TV and aortic valve
PM VSD far from aortic valve


What are indications for PA banding

Multiple muscular VSD
complex lesion with high pulmonary blood flow
infants with very low body weight who are not fit for CPB


What are Class IIa indications for re-intervention for TOF

Free PI with progressive/moderate-Severe RV dilation (RVEDVi 170cc/m2),
Moderate RV dysfunction
Moderate to severe TR
Residual VSD Qp/Qs 1.5
Residual PS with RVSP > 2/3 SABP
Significant AI with symptoms and/or progressive LV systolic dysfunction
Aortic root > 55 mm
A large RVOT aneurysm or evidence of infection/false aneurysm


What are advantages of complete repair for TOF

Promote growth and development of organs
Eliminate hypoxia
Less need for extensive right ventricular muscular excision
Better late Right ventricular function
decreased incidence of late Dysrhythmias


When do you consider a modified BT shunt for a TOF

1. Early symptoms (within first 3 months)
2. Branch PA are hypoplasia
3. Anomalous origin of LAD crossing RVOT
4. Severe associated non-cardiac disease
5. pulmonary atresia--insertion of a conduit
6. When you have no experience with neonatal complete repair


What are indications for TOF repair

Hypoxemia (sats are consistently in 75-80% range)
Hypoxeimc (Tet) spells
start propanolol
Timing (no specific indication)
elective complete repair by 1year of age but most are done between 3 and 6 months


What are associated anomalies for Adult coarctation

Bicuspid aortic valve
Aortic arch hypoplasia
Extensive collateral cicrulation
Circle of willis aneurysm


What is natural history adult coarctation

25% mortality by 20 years of age
50% mortality by 32 years of age
75% mortality by 45

causes of death HTN, premature CAD, LV failure, aortic dissection


Class I indications for Aortic coarctation repair

Significant CoA
for ReCoA
exercise induced severe arterial hypertension
Trans-Coarctation resting gradient of > 25mmHg


List associated anomalies for TGA

Left ventricular outflow tract obstruction
Aortic coarctation
Coronary arteries abnormalities


What are common clinical findings of TGA

Pa02 of (25-40mmHg) with CHF
Cath is rarely needed
CXR-egg shaped
ASO should be performed within first 3 weeks of life
Should be on PGE1 infusions
Balloon atrial septostomy--routine, performed with TTE,
If presentation of outside 1 month
PA and BT shunt
atrial switch


What are post op complication of TGA

supravalvular pulmonary stenosis
Coronary artery stenosis/kinks
Right ventricular failure (RCA)
Hospital mortallity (2-5%)
coronary artery related problems are the main cause


What are long-term results of TGA repair

Normal somatic growth
Similar average general health
Almost normal exercise capacity
5 and 10 year survival 95 and 92%
Mild late neurological deficits


Define and embryological development of truncus

failure of the truncal ridges and the aorto-pulmonary ridges to develop normally resulting in one great artery with a single semilunar valve. The truncus overrides both ventricles and there is a VSD


What is Aorto-pulmonary window

communication between the aorta and the PA with two separate semilunar valves


What is classification of APW

Type I: proximal defect between adjacent ascending aorta and main PA
Type II: distal defect usually posterior to the origin of the right PA
Type III: total defect involving the entire length of the PA.


What is Van Praagh classification of VSD

Conoventricular (membranous)
Conal (outlet)
Inlet (AV canal type)
Muscular (single or multiple)


What is most common VSD

1. Conoventricular (80%)
centered around the membranous septum
important landmarks for repair : anterior-septal commissure and noncoronary sinus of aortic valve


What is Eisenmenger complex

Fixed pulmonary hypertension
bidirectional shunting
right ventricular hypertrophy
Inoperable and require heart lung transplant for further survival


What is Health-Edwards classification of pulmonary vascular disease

Grade 1: medial hypertrophy without intimal proliferation
Grade 2: medial hypertrophy with cellular intimal reaction
Grade 3: intimal fibrosis and medial hypertrophy
Grade 4: generalized vascular dilation, an area of vascular occlusion by intimal fibrosis and plexiform lesions
Grade 5: other "dilation lesions" such as cavernous and angiomatoid lesions
Grade 6: Necrotizing arteritis

Grade 3 or above is not reversible


What is the rule of 50%

Heart is DORV if in addition to the PA, more then 50% of the aorta arises from the right ventricle
that a double conus (infundibulum is present) meaning there is no aortic to mitral continuity.


What is classification of DORV

always association with a VSD.
The classifcation centers on location of VSD


List the types of DORV

1. DORV with subarotic VSD (50%) *similar to TOF
2. DORV with subpulmonary VSD (30%)
3. Doubly committed VSD: VSD immediately beneath both the PA and aorta
4. Non committed VSD: any VSD that is located below the conal septum or the junction of conal and muscular interventricular septum.


What are the features of TOF

infundibular pulmonic stenosis
right ventricular hypetrophy
dextroposition of aorta (overidding)


All features of TOF are because of a single anatomical lesion

Anterior and leftward displacement of the infundibular septum

this narrows the RVOT, leading to subpulmonic obstruction resulting in the anterior malalignment VSD.
Also causes overidding of the aortic valve into the right ventricular cavity


What is a common feature of the pulmonic valve in TOF

75% stenotic with half to two thirds being bicuspid


What is management of hypercyanotic episode in TOF

administration of IV fluids
morphine and other sedatives
Increase SVR and increase pulmonary blood flow
a. knee to chest position
b. phenylephrine
c. possible beta blockers (allow more filling of LV)
Intubation and PEEP
Prompt surgery


What can you do surgically if TOF as an anomalous LCA coming across RVOT

1. Conduit reconstruction of the RVOT with origin from ventriculotomy placed below the anomalous coronary
2. Translocation of the pulmonary artery to a distal ventriculutomy and use of the native pulmonary artery as a composite conduit to create a double outflow from the right ventricle
3. Complete repair from a transatrial-transpulmonary approach
4. Palliative shunt and fix when older


What is the long term follow up and management of late pulmonary valve replacement

MRI is useful for extent of right ventricular enlargement
Right ventricular end-diastolic volume of 160 to 170ml/m


What are associations with etiology of truncus

Microdeletion of Chromosome 22q11 is associated with neural chest cell defect (DiGeorge)
Environmental risk factors include maternal diabetes and exposure to retinoic acid


List two classification for truncus

Collett and Edwards--based on the site of origin of the pulmonary arteries

Van Praagh-based on the degree of septation of the trunk and the presence or absence of a VSD


List Collett and Edwards Classification of Truncus

Type 1: Branch Pulmonary arteries arise from a segment of the main pulmonary artery off the common trunk
Type 2: Branch Pulmonary arteries arise in close proximity from the posterior aspect of the common trunk
Type 3: Branch Pulmonary arteries arise from separate, widely spaced origins
Type 4: Absent "true" branch pulmonary arteries with aortopulmonary collaterals


What is Van Praagh classification of Truncus

Type A: VSD present
Type B: VSD is absent
Type 1: partial development of aorticopulmonary septum
Type 2: absence of aorticopulmonary septum
Type 3: absence of one of the branch pulmonary arteries
Type 4: Coarctation, hypoplasia, or interruption of the aortic arch with a PDA


What are presenting features of AP window

present in CHF
wide pulse pressure
differential includes PDA, TA, VSD with AI, and ruptured sinus of valsalva


What is natural history of AP window

40% die in first year of life
All patients should undergo surgical repair to avoid the effects of PVR


What is D-TGA

The aorta is by definition anterior and to the right of the pulmonary artery

There is atrioventricular concordance but ventriculao arterial discordance.

results in systemic and pulmonary circulations performing in parallel rather than in series.

Deoxygenated blood goes to body and never passes through lung. Must be an obligatory shunt


Describe the senning operation

Atrial switch concept was first successful accomplished Senning in 1959
Relocating the walls of the right atrium and the atrial septum
Pulmonary and systemic venous return was rerouted by incising and realigning the atrial septum over the pulmonary veins and using the the right atrial wall to create a pulmonary venous baffle.


Describe the Mustard operation

In 1964 Mustard described an alternative intra-atrial repair
Excising the atrial septum and creating a large interatrial baffle of pericardium to redirect pulmonary and systemic venous blood flow.
Resulted in a larger atrial size then senning.
Creation of a virtual common atrium
Became most common in 1960s


What are features of both atrial switch operations

Placing the right ventricle and the tricuspid valve in the systemic circulation
Physiologically but not anatomically repaired
The standard surgical repair was balloon atrial septostomy in infancy followed by elective repair between ages 3 and 8 months
Operative mortality was 2 to 10%
Long term survival was 75% at 25 years


What are uniques features of failure for atrial operations

Progressive late right ventricular dysfunction
late arrhythmias
heart failure


What is the basic technique for the arterial switch procedures

Dividing the aorta and pulmonary artery, excising the origins of the coronary arteries with a button of aortic wall, repositioning the coronary arteries to the posterior great vessel (pulmonary artery), and re-constructing each ventricle outflow to the appropriate distal vessel


What is the Lecompte maneuver

The pulmonary artery bifurcation is brought anterior to the aorta


What is more common indication for surgery post arterial switch (long term)

Reoperation on the right ventricle outflow tract related to inadequate growth at the neo-pulmonary anastomotic site
progressive neo-aortic dilation and late coronary artery lesions


What is a Rasteli Operation (for TGA)

Performed in cases with a large conoventricular VSD and TGA.
Involves a synthetic semicircular patch sewn to the edge of the VSD inferiorly and around the aortic annulus.
This diverts flow from the left ventricle through the VSD into the aorta
The pulmonary root is closed and a conduit is inserted between the right ventricle and the pulmonary artery


What are the 10 commandements for Fontan physiology

Minimal age 4
Sinus rhythm
Normal caval drainage
Right atrium of normal volume
Mean pulmonary pressure < 15 mm Hg
Pulmonary to artery to aorta diameter ratio > 0.75
Normal ventricular function (ejection fraction > 0.6)
Competent left atrioventricular valve
No impairing effects of previous shunts


What is Bidirectional Cavopulmonary anastomosis

Divided Superior vena cava anastomosed to the right pulmonary artery supplying both lungs
Azygos and small venous branches are ligated
can be an alternative to a Fontan or as a stage procedure.


What is the 1.5 ventricle epair

Children with marginal pulmonary ventricle, the options are Fontane or biventricular repair
1.5 is recruiting hypoplastic pulmonary ventricle to manage part of the systemic venous return by closing the atria septum, enlarging the right ventricular outflow tract and adding a cavopulmonary anastomis
This means the right ventricle only pumps blood from the IVC and not the entire cardiac output


What is the purpose of the Fontan Fenestration

Fenestration in the the Fontan baffle, allows systemic venous blood to shunt to the pulmonary venous atrium achieving better preload for the systemic ventricle at the cost of some systemic arterial desaturation


List complications from Fontan

Protein losing enteropathy
Plastic Brnchitis
atrial arrhythmias
Venovenous colllaterals
Thromboembolic events
recurrent pleural effusions


What is mechanism and treatment for Protein-losing enteropathy associated with Fontan

Loss of protein in the GI tract
Mortality 50% at 5 years
Possibly related to abnormal mesenteric flow
Intermittent albumin
heparin therapy
some have tried pacing, viagara
Transplant is final cure


What is Carpentiers Classification of Ebsteins Anomaly

Based on morphology of the right ventricle and tricuspid valve
Type A: RV valve adequate
Type B: RV valve inadequate with mobile anterior leaflet
Type C: RV valve inadequate with restricted anterior leaflet
Type D: Severe atrilialization of the RV and RVOT obstruction (Uhl's disease)


What are indications for Surgical intervention for Ebsteins

Severe CHF
Severe symptomatic TR
Severe Cardiomegaly due to to the increased risk of sudden death


What are surgical options for Ebsteins Anomaly

Tricuspid valve replacement
Starn: closure of the TV, RV to PA conduit and BT shunt
Carpentier: detaching the anterior leaflet, sliding it up and reattaching it with reinforcement with a synthetic ring
Mayo: ASD patch, caudal-cranial annuloplasty (monoleaflet valve)


What are the 3 main types of complete atrioventricular septal defects

Common AV valve orifice and a large interventricular communication
Rasteli A: Septal Bridging leaflet attached to chordae from crest of the ventricular septum (mild bridging)
Rasteli B: SBL attached to chordae from RV of papillary muscles (moderate bridging)
Rasteli C: SBL free floating with no chordal attachments (severe bridging)


What is a partial AVSD

Interatrial communication, two AV orifices, and no interventricular communication. A tongue of of tissue connects the ABL and PBL


What are Non-cardiac anomalies associated with AVSD

Asplenia (28%)
Down's syndrome (13%)
Polyspenia (6%)


What are the goals of repair complete AVSD

Eliminate atrial and ventricular shunts
Devide common AV valve into competent non-stenotic left and right sided AV valves
Preservation of sinus and rhythm

The most common complication is AVV regurgitation


What are the anatomical features of congenitally corrected transposition of the great arteries

AV and VA discordance
L-looping (L-TGA) aorta anterior and to the left of the PA
Blood flow: RA to LV to PA, and LA and to RV to Aorta
Subpulmonary VSD in 85%
TV anomalies in 90%, severe TR in 30%
Complete heart block (CHB) in 15%


What is a physiological repair of CCTGA

Accept the current ventricular assignment optimize the physiology
Close the VSD
Repair the systemic AV valve
Correct the pulmonary stenosis


What is a anatomic repair of CCTGA

A mustard procedure combined with arterial switch or a Raseli
this may have better long term outcomes


What is a Taussig-Big Anomaly

DORV with D-TGA.

In the absence of LVOT ASO and VSD patch may be adequate


When should you NOT do an arterial switch operation

Late presentation of TGA--requires training the systemic ventricle with PA bands
LVOT obstruction--think Rasteli
Patient with Previous Mustard
Taussig-Big Anomaly


List features of ideal Fontan circulation

Low PA pressure (PAP < 15)
PVR < 2
Transpulmonary gradient < 8
A competent AVV
Good ventricular function


What is Shone's complex

Parachute mitral valve
Subaortic stenosis
Coarctation of the aorta
Mitral suptra-vavular fibrous ring


What are components of the Norwood Operation

Atrial septectomy
Division of PA and enlarging the aorta with the PA patch
A modified BY shunt or an RV to PA conduit
Arch augmentation with a patch


What are the goals of a norwood operation

unobstructed systemic blood flow
unobstructed coronary blood flow
controlled pulmonary circulation
unobstructed pulmanar drainage (ASD)


What is Down's syndrome associated with

Complete AVSD
mental retardation
facial and skeletal anomalies


What is DiGeorge Syndrome associated with

Type B interrupted Aortic Arch
Thymic aplasia


What is Williams Syndrome associated with

Supravalvular AS, Elfin facies, hypercalcemia


What is Turners syndrome associated

Coarctation of the Aorta
Webbed Neck
Increase distance between nipples


What is Hyterotaxy syndrome

PA or PS


What are indications for biventricular repair in Hypertrophic Left Heart syndrome

LV volume > 20-25 ml/M2
AV annulus > 5.5mm
MV annulus > 9 mm

In addition: Non-apex forming LV and endocardial fibro-elastosis are contraindicated to biventricular repair


How does Anomalous Left Coronary artery from Pulmonary artery present

when PA pressure drops after birth left coronary steel occurs and blood is directed to the lower pressure pulmonary system leading to massive coronary ischemia.

Treatment is urgent reimplantation of the left coronary to the aorta


What is mean survival for Coarctation and what are causes of death

Mean survival is 42 year

aortic rupture, endocarditis, CHF, stroke


What are risk factors for developing an aneursym post coarc repair

Use of a prosthetic patch---medial necrosis in the aorta opposite the patch (risk is 38%)
Older age at repair
Females have a greater risk (during pregnancy)
Aortic diameter at the repair site to the aortic diameter at the diaphragm ratio > 1.5


What are the options for Coar and VSD

CoA repair followed by VSD closure if needed
CoA repair + PA band followed by VSD closure if needed
CoA repair and VSD closure in one operation


What is Sealy's syndrome

Abdominal pain post CoA repair


What are specific complications of CoA

Paradoxical HTN
Recurrent laryngeal nerve injury leading to vocal cord paralysis
Aortic thrombosis


What are types of partial anomalous venous connection

Sinus venosus syndrome
Right superior PV to SVC without ASD
Right sided PV to right atrium directly
Right sided PV to IVC --Scimitar syndrome--needs a concentric vein that drains all or most of the right lung to the IVS (right lung hyooplasi)
Right PV to Azygous vein or coronary sinus
Left PV to innominate vein


What are the type of total anomalous pulmonary vein connections

Supracardiac--(45%) Left vertical vein drains into the innonminate vein or the SVC, rarely to the azygous vein
Cardiac (25%) Common vein drains to the coronary sinus or rarely directly into the RA
Infracardiac (25%) common vein drains to the portal vein in most or to the ductus venous, gastric vein, hepatic vein or IVC in some
Mixed (5%) most commonly the left PV drains to a left vertical vein that drains to the innominate vein the remainder of the PV drain to the coronary sinus


What factors are involved in obstruction with TAPVC

Infracardiac TAPVC it occurs 100% and usually at the level of the portal vein
65% if the common vein drains to the SVC
40% if the common vein drains to the innominate vein
In mixed type TAPVC 40%

In the absence of a true ASD there is functional obstruction to pulmonary blood flow


What is natural history of TAPVC

untreated pts have median survival of 2 month
Supracardiac 2.5 month
Infracardiac it's 3 weeks
Post repair survival is 85% at 10 years


List indications to Close PDA

- respiratory failure or congestive heart failure in pts who have failed medical therapy (indomethacin)
- presence of PDA can lead to pulmonary vascular disease, endocarditis and Eisenmenger’s syndrome
- in adults, if pulmonary vascular resistance > 6-8 Wood units then do not close PDA, instead consider lung or heart-lung Tx


What lesions are commonly associated of CoArch

- Bicuspid Aortic Valve.p
- VSD, usually posterior malalignment type.
- PDA.
- Mitral prolapse due to choral elongation.
- Shone’s complex, multilevel left sided obstructive lesions.
- Turner’s syndrome, 15–35% of CoA have this


What is Natural history and indications for surgery in CoArc

90% of pts left untreated will eventually die by age 50 d/t cardiac or stroke complications
surgical repair indicated when gradient 20-30mmHg @ rest and loss of ≥50% diameter. If grad is < and anatomical obstruction severe, exercise test will reveal more severe gradient and repair is indicated


List 7 repair techniques for CoArc

End to end anastamosis
Extended end to end anastamosis
Subclavian flap
Reverse subclavian flap
Patch aortoplasty
Tube graft replacement
Extra anatomic conduits


7 day old infant presents to ER with resp distress, severe metabolic acidosis, absent femoral pulses. What are 4 causes

Acyanotic, ductal dependent lesions with L-sided obstructions:
hypoplastic arch
interrupted arch (posterior malalignment VSD)


What is relationship of Turners Syndrome to CoArc

There is a higher incidence of coarctation in females with Turner’s. They present with proximal hypertension (headaches, epistaxis, ruptured cerebral aneurysm) or claudication (poor distal perfusion with exercise-like swimming)

10% association of coaractation of the aorta in Turners syndrome.


List the Classification of Interrupted Arch

Type A (40%) Interrupted at isthmus just distal to the left subclavian
Type B (55%) Interrupted between left carotid and the left subclavian
Type C (5%) Interrupted at prox arch between the inominate and the left carotid

Most have a large VSD and varying degrees of other left sided obstruction and hypoplasia. 40-50% have bicuspid aortic valve. Subaortic obstruction in many Type Bs.

There must be an associated anomaly, such as a PDA to supply blood to the descending aorta and thereby permit survival, even in utero.


The causes of LVOT obstruction in IAA are:

Posterior malalignment of the conal septum.
Prominent muscle of Moulaert.
Bicuspid or unicuspid aortic valve.
Commissural fusion.
Annular hypoplasia.
The interruption


Most common congenital arch anomaly?

Aberrant Right subclavian artery off (left) descending aorta


5 year old kid with L-R shunt on echo; name 5 indications for surgical intervention.

Pulmonary-to-systemic flow ratio (Qp:Qs) of 1.5:1 or Qp:Qs > 2:1 (depending on the reference).
Uncontrolled CHF, including growth failure and recurrent respiratory infection.
Large, asymptomatic defects associated with elevated PA pressure (PAPs > 40-50 mmHg).
Ventricular enlargement/RV dysfunction is usually considered evidence of a clinically significant left-to-right shunt and an indication for surgical closure.
After 1st or 2nd episode of endocarditis
If the shunt results from a VSD, prolapse of an aortic valve cusp may be an indication for surgery, since early repair may prevent progression of the aortic insufficiency.


55 y.o. male, progressive SOB, palpitations. A right and left heart cath is performed. Saturations are as follows: LA=99%, Aorta=98%, SVC=70%, PA=85%. PAP is 75/40 and the LAP is 10 mm Hg
a) Calculate the shunt fraction

Qp:Qs = SAO2 – MVO2 : PVO2 – PAO2
Qp=pulmonary blood flow, Qs=systemic blood flow, MVO2=mixed venous, PVO2=pulmonary venous, PAO2=pulmonary arterial, SA=systemic arterial
ideally MVO2 is calculated by averaging O2 sat from IVC and SVC
PVR ={PA pressure (mean) – LA pressure (PCWP)}/ Qp (CO)
Therefore PVR in woods units = (PAP – PCWP)/ CO
Dynes = Wood units x 80
Normal PVR = 100-200 dynes  1.25=2.5 wood units.


What are contraindications to NOT closing

Hemodynamic contra indications to operation are fixed pulmonary hypertension, PVR > 6-8 Woods units, flow reversal
If Qp:Qs is <1 then there is flow reversal across VSD from PHTN. This pt. needs to be cathed in the presence of pulmonary vasodilators to determine if shunt is reversible
If PVR > 6-8 woods units then cath with NO, SNP, O2 to determine reversibility of PHTN
Qp:Qs > 1.5:1
> 8 Wood units → no repair
6-8 → vasodilatory challenge (FiO2 100% or NO 30ppm)


What are indications for PFO closure

1. recurrent stroke: despite compliant medical treatment
inability to comply safely w/ medical treatment
2. known circulating hypercoagulable state w/ ↑ risk of thromboembolism despite recommended warfarin treatment
3. lupus anticoagulant/antiphospholipid antibody
4. persistent procoagulant risk despite best medical treatment
5. “high risk PFOs”
atrial septal aneurysm
spontaneous R→L shunting
“tunnel-like” appearance
6. divers


List Complications of untreated VSD

Bacterial Endocarditis – 0.1 –0.3% per yr
Congestive heart failure
Failure to thrive
RVOTO 2° to infundibular muscular hypertrophy (RV muscle bundles)
RVH/Right heart failure
Tricuspid regurg
Aortic insufficiency
Eisenmenger’s complex


Describe Membranous VSD

Perimembranous (80%): When the membranous area of the ventricular septum fails to form completely, a VSD results adjacent to the commisure btw the anterior and septal leaflets of the tricuspid valve. The conduction system (Bundle of His) is in the posterior/inferior rim


What are indications for VSD repair

Severe heart failure at age < 2-3 months, except w/ swiss cheese VSD
Serious symptoms at age 2-6 month
Failure to thrive beyond 6 months of age
Subarterial VSD at any age
endocarditis after 1st or 2nd episode
pacemaker: to avoid paradoxical emboli
closure is contraindicated if PVR is > 8u/m2(Woods units) or it fails to respond to Isoproterenol w/ a drop to < 7 Woods units


What is classification of Pulmonary atresia with VSD

Type A: Confluent central PAs and no MAPCAs.
Type B: Confluent central PAs and MAPCAs.
Type C: Non-confluent central PAs and MAPCAs (usually 2 to 6).
MAPCAs or Major Aorto-Pulmonary Collaterals are alternative sources of blood flow to the lungs. The types of MAPCAs are:
- Primitive intersegmental arteries that failed to involute.
- Indirect arteries arising from the aortic arch.
- Bronchial arteries.


What are types of AV canal defects

AV canals:
Complete: ASD, VSD, one common annulus (balanced or unbalanced)
correct before 4-6mo
one patch or two patch or Australian repair (stick valve on crest)
close mitral cleft when possible
Partial: Primum ASD
- correct preschool or if symptomatic
Intermediate: ASD, VSD and 2 annulus
depends on size of VSD
if symptoms correct


Associated lesions that influence the prognosis of AV canal repair (7

MR or cleft MV
LVOTO/arch anomalies
Multiple VSD’s
Conotruncal TOF, DORV, TGA
Heterotaxia, single ventricle


Causes of post-op AV canal repair metabolic acidosis, decrease urine output and hypotension (6)

Malpositioning of ETT w/ hypoventilation
Dehiscence of the repair of atrioventricular valve
Residual VSD
Pulmonary hypertension


What are the boundaries of the VSD in TOF

superiorly: antroseptal commissure of TV and right fibrous trigone just under right coronary cusp of AV
posteroinferiorly: posterior limb of Trabiculum Septo Marginalis (TSM), which contains bundle of His inferior to the muscle of Lamchizi (papillary muscle of conus)
anteriorly: anterior limb of TSM


What is the unifying aspect of TOF

The unifying mechanism (monology) is anterior and leftward displacement of the infundibular septum.


What is a formula for determining if you are going to be able to save an infants pulmonary valve based on the pre -op size of echo

transannular patching vs PA valve sparing: adequate size of valve annulus is baby’s wt (kg) + 3mm (e.g. 4kg baby → 7mm annulus


TOF repair. You open the pericardium and there is an anomalous vessel crossing the RVOT from right to left:
a) What is this vessel?
b) List three options for dealing with this scenario.

a) anomalous LAD coming from RCA (could also be a large conus branch)
The extent of the RV ventriculotomy will be limited, such that myomectomy, release of muscular bands, etc. through this exposure may not be feasible.
Palliative shunt
Transatrial transpulmonary approach is usually effective, still allowing a short transannular incision (if required) to relieve RVOTO.
RV-PA conduit
**Mobilization of the coronary artery with patch placement beneath it is not recommended.


TGA in 5 week old. List 3 components of Rastelli repair. What would prevent you from doing repair immediately?

VSD and PS @ 6-12months
take down shunt if previously shunted or divide PDA
baffle VSD to aorta
RV to PA conduit (homograft)


What are contraindications to Arterial Switch for TGA

Pulmonary stenosis
Aortic stenosis
Poor LV function which cannot support systemic pressures
Intramural coronaries (relative contraindication)


What are options for a pt presenting with late TGA

With non restrictive VSD, probably ok to do arterial switch
Otherwise based on LV function
If leftward deviation of septum on echo, high probability of failure post-op
Patient will likely need sequential PA banding to 80% of systemic pressure plus or minus systemic to arterial shunt
When LV is ‘conditioned’ the arterial switch can be perform


5 complications of Senning/Mustard

1-Baffle leaks: , 28% incidence r
2-SVC obstruction: 19% incidence rate (higher with Dacron). Treated with balloon dilatation
3-IVC obstruction: 1-2 % (higher with Dacron) .
4- Pulmonary Venous obstruction: .
5-Stenosis of Left Pulmonary Venous Channel: 1-2% incidence
6-Dysrhythmias: Sinus node dysfunction. 9% incidence.
7-RV dysfunction and TR: Reduced RV contractility (RV and TV not able to support systemic pressures indefinitely) Increased RVEDV, decreased RVEF post atrial switch


5 Complications of bidirectional Glenn (7)

- SVC syndrome
- cerebral edema
- hemothorax
- chylothorax
- pulmonary artery thrombosis
- sinus node injury/arrhythmias
- protein-losing enteropathy


Management of baby with absent thymus. How to manage post op.

Digeorge Anomaly (Deletion 22q):
Irradiated blood products
To destroy lymphocytes and prevent graft-versus-host reaction
Deficit in T-cell mediated immunity (cellular immunity)
Hypoplasia or aplasia of the parathyroid glands
Increase risk of infection
Normal immunoglobulin count but decrease reaction often seen (IgG and IgA especially)


How are vascular rings classified

All rings are essentially interruption of the embryonic double arch with persistence of parts of the non-dominant artery


List types of complete Arch

Complete rings:
1) Double arch persistence of both left (anterior to trachea) and right arch (posterior to trachea)
Left (anterior) arch dominant 25%
Right arch dominant 75%
Cervical aorta syndrome
Right aortic arch with left ligamentum
Type 1: no retroesophageal segment
Type 2: mirror image w/ retroesophageal segment (Komerell)
Type 3: retroesophageal subclavian artery (most common)
Type 4: retroesophageal innominate artery


List the type of incomplete Arch

Left arch with aberrent retroesophageal right subclavian artery (0.5% of population)
usually asymptomatic


What are vascular rings

Incomplete vascular rings that surround the trachea but not the esophagus.

Anomalous left PA originating from the right PA instead of the main PA. The left PA
passes between the trachea and esophagus causing tracheal compression and an indentation on esophagogram.


List 4 features of Scimitar

sinus venosus ASD usually close to jct of IVC w/ RA
anomalous pulmonary venous drainage of most of the right lung connecting to the IVC/RA jct
aortopulmonary collateral blood supply to the right lower lobe
hypoplasia of right lung


What factors influence the management of pulmonary atresia with intact ventricular septum

RV dependent coronary artery circulation
Size of RV
Size of tricuspid pulmonary valve
degree of RV hypoplasia:


The potential causes of low cardiac output in a Fontan circulation include:

- Cardiac Tamponade.
- Hypovolemia.
- Depressed ventricular function.
- AVV regurgitation.
- Increased PVR.
- Arrhythmia.
- Systemic venous pathway obstruction


The ideal fontan would have

- Low PA pressure, PAPsys < 15-18mmHg, Fontan would be contraindicated if > 20.
- PVR < 2 woods units, Fontan would be contraindicated if > 4.
- Transpulmonary gradient < 8mmHg, Fontan would be contraindicated if > 10.
- A competent AVV.
- Good ventricular function, EF > 50%, contraindicated if EF < 40%
- Good ventricular compliance LVEDP < 10mmHg contraindicated if LVEDP > 12mmHg.


What are important features of TAPVR

“The most important anatomic factors in determining the clinical status of the patient include the presence and location of the right-to-left shunt, and the presence or absence of obstruction in the pulmonary venous pathway”

Excerpt From: Carlos M. Mery & Joseph W. Turek. “TSRA Review of Cardiothoracic Surgery.” Feedbooks, 2011. iBooks.
This material may be protected by copyright.


cyanotic 4 month old infant undergoing TOF, large coronary branch seen cross LVOT. What is it it?

What are 3 surgical options

Left anterior descending artery (possible large conal branch)

1. palliative shunt: deferring repair until later
2. transatrial, transpulmonary incison
3. Repair with a more inferiorly placed ventricultomy and right ventricle to pulmonary artery homograft conduit
4. translocation of pulmonary artery onto right ventriculotomy placed inferior to anomalous vessel

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