Congenital questions Flashcards
(200 cards)
1
Q
Understand how to calculate the estimated left to right shunt for an ASD
A
Aortic Saturation - SVC saturation/ Pulmonary venous saturation - Pulmonary artery saturation
A 2:1 shunt should be repair with elevated PA pressure
2
Q
What are two most appropriate or preferred repair techniques for coarctation in the neonatal age group
A
Extended end to end anastomosis
left subclavian flap
3
Q
What are the 3 most appropriate or preferred repair techniques for coarctation in the adult age group
A
Interposition graft
resection and end to end anastomosis
balloon dilation with intralumenal stent
4
Q
List 5 complications of coarctation surgery
A
paraplegia chylothorax hemorrhage phrenic nerve injury/palsy Horner's syndrome post operative/residual hypertension recurrent coarctation Postcoartecomy syndrome/abdominal pain/mesenteric arteritis
5
Q
Describe 3 features that preclude percutaneous ASD device closure
A
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
6
Q
List 5 complications of ASD closure
A
Residual shunt migration of shunt obstruction of coronary sinus obstruction of pulmonary veins thromboembolic event cardiac chamber performation
7
Q
List 6 potential causes of hypoxemia folllowing bidirectional cavopulonary anastomosis
A
pulmonary artery stenosis stenotic anastomosis elevated pulmonary vascular resistance cardiac tamponade left ventricular dysfunction, pulmonary edema anemia atrioventricular valve dysfunction/mitral regurgitation runoff through venous collaterals- azygos vein hemo/pneumothorax hypovolemia excessive PEEP Vq.Q mismatch, atelectasis
8
Q
Describe the anatomical features/locations of the following types of ventricular septal defects: Perimembranous subaortic ASD Inlet VSD Muscular VSD Doubly comitted subarterial VSD
A
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
9
Q
Describe the pathophysiology of subvalvular subaortic stensosis
A
Fixed fibromuscular discrete membrane. Tunnel diffuse subarotic stenosis Hypertrophic cardiomyopathy (IHSS) Abnormal insertion of mitral valve chordae Hypoplastc aortic valve ring
10
Q
List 4 complications of subaortic stenosis repair
A
Residual obstruction
heart block
ventricular septal defect
aortic valve tearing
11
Q
What is natural history of perimembranous VSD if left unoperated
A
Progressive RV enlargement Congestive heart failure prolapse aortic valve and aortic valve insufficiency risk of endocarditis adult pulmonary hypertension
12
Q
Patient wishes to have device closure of VSD . What anatomtomicl structures are importance to assess before closure
A
Tricuspid valve
aortic valve
size of defect
distance from aortic valve to superior edge of defect
13
Q
What are complications following closure of perimembranous VSD device closure
A
Left ventricular outflow tract obstruction
residual shunt
device embolization/migration
tricuspid/mitral/aortic valve regurgitation
Arrhythmia/heart block
14
Q
List the 5 complications or pitfalls specifically associated with surgery for Ebstein’s anomaly
A
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
15
Q
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
A
presence of chylomicrons
WBC > 1000
Lymphocyte > 80%
Triglyceride > 1mM
16
Q
List 4 treatment options in the sequence that normally would be employed for chylothorax
A
Pleural drainage NPO or low fat or medium change triglyceride diet total parenteral nutrition surgical ligation of thoracic duct octreotide treatment
17
Q
List 5 complications of unrepaired congenital ventricular septal defect
A
Congestive heart failure
Aortic insufficiency
Endocarditis
Pulmonary hypertension (Eisenmenger’s syndrome)
right ventricular outflow tract (RVOT) obstruction
severe failure to thrive
18
Q
Start CPB and the PA pressure remain high? What is this? what can be done?
A
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
19
Q
Classify interrupted aortic arch
A
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
20
Q
What are associated lesions with interrupted arch
A
Isolated VSD (most common) Truncus arteriosus transposition of great arteries with VSD including taussig-bing anomaly aortopulmonary window single ventricle subaortic obstruction
21
Q
What is DiGeorge Syndrome? What 2 things must be done postop?
A
No thymus. Watch of this when doing sternotomy.
Monitor Calcium level
Blood products must be irrigated
22
Q
In a 30 year adult should you close an ASD?
A
Yes, reduces the risk of paradoxical embolism but this controversial.
Yes, because of mortality benefit if closed before age of 40?
23
Q
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?
A
Transposition of Great Arteries
Pulmonary artery atresia with or without VSD
Obstructed total anomalous pulmonary venous drainage
tricuspid atresia
24
Q
What is immediate pharmacological treatment for a new born in extreme cyanosis
A
PGE1 infusion should be started immediately to restore ductal patency. Most important thing regardless of diagnosis!
25
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).
26
What are classical infow and outflow "from" that constitutes the collateral circulation around a classical coarctation of the aorta in an adult partnre]
```
Inflow;
Internal mammary artery
vertrbral artery
costocervival artery
scapular vessels
thryocervival trunk
Outflow
superior epigastric/inferior epigastric
first two pair of intercostal arteries distal to coarctation (3rd and 4th intercosteal arteries)
Descending aorta
```
27
What is most common aortic arch congenital anomaly
anomalous origin of the right subclavian artery
28
List 10 complications of PDA closure
```
Hemorrhage
recurrent laryngeal nerve injury
chylothorax
ductal recanalization or persistent ductal patency
left pulmonary artery occlusion
accidental aortic ligation
infection
esphageal injury
pulmonary injury
vagus nerve injury
phrenic nerve injury
pneumothorax
```
29
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.
30
what are the 3 most common cardiac causes of cyanosis in a newborn
Tetralogy of Fallot-particulary with pulmonary stenosis
d-TGA
Tricuspid atresia and pulmonary stenosis
Truncus
31
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
32
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
33
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
34
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
35
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
36
15 year old who had TOF with transannular patch. List 4 tests you require to assess cardiac function
```
EKG
Cardiac MRI
echocardiogram
exercise stress test
CT angiogram
```
37
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
arrhymias
new onset of TR in the presence of severe pulmonary insufficiency
QRS > 180 msec
38
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
39
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
```
40
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
41
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
42
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.
43
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
44
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
45
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
46
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
47
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
```
48
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.
49
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
50
List the classification for congenital ventricular septal defects
Conoventricular (membranous)
Conal (outlet)
Inlet (AV canal)
Muscular (VSD)
51
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
52
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
53
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.
54
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
55
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
56
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
57
What is Carpentier classification of Ebsteins
a reproducible classification is not possible because of variability.
There is a Carpentier classifcation in the literature.
58
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
59
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
60
What are components of complete AV canal defect
Primum ASD
Unrestrictive inlet VSD
Common atrioventricular valve
61
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
62
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.
63
What lesions are associated with Coarc
```
VSD
Bicuspid
PDA
AS
Hypoplasia of arch
```
64
List congenital lesions of mitral valve
Cor-Triatriatum
Anomalous pulmonary venous drainage (total or partial)
Mitral stenosis--supravalvular and parachute
Mitral atresia
65
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.
66
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
67
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
```
68
Name all the acynaotic lesions
```
atrial septal defect
ventricular septal defect
patent ductus arteriosus
atrioventricular canal
coarctation
aortic stenosis
pulmonary stenosis
```
69
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
70
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
```
71
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
72
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
73
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
74
What are complications of DEVICE placement for ASD
```
Migration of device
Residual shunt
thromboembolic event
obstruction of coronary sinus
obstruction of pulmonary veins
```
75
Post op surgical closure of ASD complications
Bradycardia
residual ASD
Arrhytmias--increased rates of SVT (AF/Aflutter)
Pericardial effusion or tamponade
76
What are associated anomalies for VSD
PDA (25%)
Aortic coartaction (10%)
LVOT obstruction (4%)
PFO/ASD, vascular ring, PLSCA
77
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
a. Endocarditis
b. Aortic insufficiency
c. Arrhythmia
d. RVOTO and LVOTO
e. Pulmonary vascular resistance
78
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
79
What are class IIa indications to repair a VSD
```
Endocarditis
Before transvenous pacing
Device
isolated trabecular muscular remove from TV and aortic valve
PM VSD far from aortic valve
```
80
What are indications for PA banding
Multiple muscular VSD
VSD/CoA
complex lesion with high pulmonary blood flow
infants with very low body weight who are not fit for CPB
81
What are Class IIa indications for re-intervention for TOF
Free PI with progressive/moderate-Severe RV dilation (RVEDVi 170cc/m2),
Moderate RV dysfunction
Symptoms
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
82
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
83
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
84
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
85
What are associated anomalies for Adult coarctation
```
Bicuspid aortic valve
Aortic arch hypoplasia
Extensive collateral cicrulation
VSD
Circle of willis aneurysm
MS, MR, PDA
```
86
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
87
Class I indications for Aortic coarctation repair
Significant CoA
for ReCoA
exercise induced severe arterial hypertension
Trans-Coarctation resting gradient of > 25mmHg
88
List associated anomalies for TGA
```
Left ventricular outflow tract obstruction
VSD
Aortic coarctation
Coronary arteries abnormalities
PFO, PDA
```
89
What are common clinical findings of TGA
```
Pa02 of (25-40mmHg) with CHF
Cath is rarely needed
CXR-egg shaped
Echo
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
```
90
What are post op complication of TGA
```
supravalvular pulmonary stenosis
Coronary artery stenosis/kinks
Right ventricular failure (RCA)
Bleeding
Hospital mortallity (2-5%)
coronary artery related problems are the main cause
```
91
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
```
92
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
93
What is Aorto-pulmonary window
communication between the aorta and the PA with two separate semilunar valves
94
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.
95
What is Van Praagh classification of VSD
Conoventricular (membranous)
Conal (outlet)
Inlet (AV canal type)
Muscular (single or multiple)
96
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
97
What is Eisenmenger complex
Fixed pulmonary hypertension
bidirectional shunting
right ventricular hypertrophy
Inoperable and require heart lung transplant for further survival
98
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
99
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
or
that a double conus (infundibulum is present) meaning there is no aortic to mitral continuity.
100
What is classification of DORV
always association with a VSD.
| The classifcation centers on location of VSD
101
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.
102
What are the features of TOF
VSD
infundibular pulmonic stenosis
right ventricular hypetrophy
dextroposition of aorta (overidding)
103
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
104
What is a common feature of the pulmonic valve in TOF
75% stenotic with half to two thirds being bicuspid
105
What is management of hypercyanotic episode in TOF
```
administration of IV fluids
morphine and other sedatives
oxygen
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
```
106
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
107
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
108
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
109
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
110
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
111
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
112
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
113
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
114
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
115
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.
116
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
117
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
118
What are uniques features of failure for atrial operations
Progressive late right ventricular dysfunction
late arrhythmias
heart failure
119
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
120
What is the Lecompte maneuver
The pulmonary artery bifurcation is brought anterior to the aorta
121
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
122
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
123
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
```
124
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.
125
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
126
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
127
List complications from Fontan
```
Protein losing enteropathy
Plastic Brnchitis
atrial arrhythmias
Venovenous colllaterals
Thromboembolic events
recurrent pleural effusions
```
128
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
Treatment
Diuretics
Intermittent albumin
steriods
heparin therapy
some have tried pacing, viagara
Transplant is final cure
```
129
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)
130
What are indications for Surgical intervention for Ebsteins
Cyanosis
Severe CHF
Severe symptomatic TR
Severe Cardiomegaly due to to the increased risk of sudden death
131
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)
132
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)
133
What is a partial AVSD
Interatrial communication, two AV orifices, and no interventricular communication. A tongue of of tissue connects the ABL and PBL
134
What are Non-cardiac anomalies associated with AVSD
Asplenia (28%)
Down's syndrome (13%)
Polyspenia (6%)
135
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
136
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%
137
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
138
What is a anatomic repair of CCTGA
A mustard procedure combined with arterial switch or a Raseli
this may have better long term outcomes
139
What is a Taussig-Big Anomaly
DORV with D-TGA.
In the absence of LVOT ASO and VSD patch may be adequate
140
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
141
List features of ideal Fontan circulation
```
Low PA pressure (PAP < 15)
PVR < 2
Transpulmonary gradient < 8
A competent AVV
Good ventricular function
```
142
What is Shone's complex
Parachute mitral valve
Subaortic stenosis
Coarctation of the aorta
Mitral suptra-vavular fibrous ring
143
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
144
What are the goals of a norwood operation
unobstructed systemic blood flow
unobstructed coronary blood flow
controlled pulmonary circulation
unobstructed pulmanar drainage (ASD)
145
What is Down's syndrome associated with
Complete AVSD
mental retardation
facial and skeletal anomalies
146
What is DiGeorge Syndrome associated with
Type B interrupted Aortic Arch
Hypothyroid
Thymic aplasia
Truncus
147
What is Williams Syndrome associated with
Supravalvular AS, Elfin facies, hypercalcemia
148
What is Turners syndrome associated
Coarctation of the Aorta
Webbed Neck
Increase distance between nipples
lymphedema
149
What is Hyterotaxy syndrome
```
Polysplenia
asplenia
TAPVC
TGA
AVSD
PA or PS
```
150
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
151
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
152
What is mean survival for Coarctation and what are causes of death
Mean survival is 42 year
aortic rupture, endocarditis, CHF, stroke
153
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
154
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
155
What is Sealy's syndrome
Abdominal pain post CoA repair
156
What are specific complications of CoA
```
Paradoxical HTN
Recurrent laryngeal nerve injury leading to vocal cord paralysis
Chylo
Infection
Paraplegia
Bleeding
Aortic thrombosis
```
157
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
158
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
159
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
160
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
161
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
162
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
163
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
164
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
```
165
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:
Coarctation
hypoplastic arch
interrupted arch (posterior malalignment VSD)
AS (LVOTO)
MS
HLHS
166
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.
167
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.
168
The causes of LVOT obstruction in IAA are:
```
Subvalvular:
Posterior malalignment of the conal septum.
Prominent muscle of Moulaert.
Valvular:
Bicuspid or unicuspid aortic valve.
Commissural fusion.
Annular hypoplasia.
Supravalvular:
The interruption
```
169
Most common congenital arch anomaly?
Aberrant Right subclavian artery off (left) descending aorta
170
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.
171
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.
172
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)
173
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
174
List Complications of untreated VSD
```
Bacterial Endocarditis – 0.1 –0.3% per yr
Congestive heart failure
Failure to thrive
PHTN
RVOTO 2° to infundibular muscular hypertrophy (RV muscle bundles)
RVH/Right heart failure
Tricuspid regurg
Aortic insufficiency
Eisenmenger’s complex
```
175
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
176
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
177
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.
178
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
```
179
Associated lesions that influence the prognosis of AV canal repair (7
```
MR or cleft MV
LVOTO/arch anomalies
Coarctation
Multiple VSD’s
Conotruncal TOF, DORV, TGA
PAPVD
Heterotaxia, single ventricle
```
180
Causes of post-op AV canal repair metabolic acidosis, decrease urine output and hypotension (6)
```
Hypovolemia
Malpositioning of ETT w/ hypoventilation
Tamponade
Dehiscence of the repair of atrioventricular valve
Residual VSD
Pulmonary hypertension
```
181
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
182
What is the unifying aspect of TOF
The unifying mechanism (monology) is anterior and leftward displacement of the infundibular septum.
183
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
184
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.
Options:
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.
185
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)
186
What are contraindications to Arterial Switch for TGA
RVOTO
Pulmonary stenosis
LVOTO
Aortic stenosis
Poor LV function which cannot support systemic pressures
Intramural coronaries (relative contraindication)
187
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
188
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
189
5 Complications of bidirectional Glenn (7)
- SVC syndrome
- cerebral edema
- hemothorax
- chylothorax
- pulmonary artery thrombosis
- sinus node injury/arrhythmias
- protein-losing enteropathy
190
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)
Hypocalcemia
Hypoplasia or aplasia of the parathyroid glands
Increase risk of infection
Normal immunoglobulin count but decrease reaction often seen (IgG and IgA especially)
191
How are vascular rings classified
All rings are essentially interruption of the embryonic double arch with persistence of parts of the non-dominant artery
192
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
193
List the type of incomplete Arch
Left arch with aberrent retroesophageal right subclavian artery (0.5% of population)
usually asymptomatic
194
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.
195
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
196
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:
197
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
198
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.
199
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.
200
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
