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Congenital Flashcards

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1
Q

What is a PFO?

A
  • Patent foramen ovale
    • one of two fetal cardiac shunts, allowing blood to bypass the fetal lungs, which cannot work until they are exposed to air
    • occurs when the foramen ovale fails to close after birth
    • later forms the “fossa ovalis”
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2
Q

What are the notable 22q11.2 deletion syndromes?

A

Multiple phenotypes

  • Tetralogy of Fallot
  • Pulmonary Stenosis
  • Interrupted arch
  • VSD
  • Double outlet right ventricle
  • D-transposition of the great arteries
  • DiGeorge syndrome: CATCH-22
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3
Q

Explain the features of Digeorge Syndrome

A

CATCH-22

  • Cardiac abnormality
    • commonly - interrupted aortic arch, truncus arteriosus, tetralogy of Fallot
  • Abnormal facies
  • Thymic aplasia and immune deficiencies
  • Cleft palate
  • Hypocalcemia/hypoparathyroidism
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4
Q

Chromosomal abnormality leading to:

  • Down syndrome
A

Trisomy 21

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5
Q

Chromosomal abnormality leading to:

  • Turner Syndrome
A

absence or abnormality in one of X chromosomes

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6
Q

Chromosomal abnormality leading to:

  • Williams Syndrome
A

Microdeletion on 7q and others

Supravalvular Stenosis

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7
Q

Mendelian gene/chromosomal mutation associated with:

  • Marfan Syndrome
A

Fibrillin-1 mutation on chromosome 15q21

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8
Q

Mendelian gene/chromosomal mutation associated with:

  • Loey-Dietz syndrome
A

TGF beta receptor disorder (TGFBR1 or TGFBR2)

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9
Q

Mendelian gene/chromosomal mutation associated with:

  • Holt-Oram Syndrome
A

TBX5 gene mutation

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10
Q

What congenital defect has the highest risk of transmission to progeny?

A
  • Bicuspid aortic valve and/or aorthopathy
    • up to 30% transmission rate
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11
Q

What is the general rate of transmission to offspring, for most congenital heart defects?

A

2-4%

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12
Q

What is/are the most common congenital heart pathology:

  • Down syndrome
A
  • 60% have some congenital heart lesion
  • AV septal defects (complete or partial)
    • ASDs
    • VSD’s
    • Both ASD and VSD’s
    • Cleft AV leaflets
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13
Q

What is/are the most common congenital heart pathology and features:

  • Holt-Oram Syndrome
A
  • Secundum ASD’s (occassionally others)
  • Abnormal digits, usually thumbs; can be both upper limbs
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14
Q

What is/are the most common congenital heart pathology and features:

  • Noonan Syndrome
A
  • Dysplastic pulmonary valve, hypertrophic cardiomyopathy, ASD’s
  • Short stature, Web neck, hypertelorism, low set ears, micrognathia, pectus excavatum, triangular facies,
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15
Q

What is/are the most common congenital heart pathology:

  • Marfan Syndrome
A
  • Aortic aneurysm
  • MVP
  • Aortic valve prolapse
  • Pulmonary artery dilatation
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16
Q

Which echocardiographic scan plane is most optimal to define a secundum ASD?

A
  • Subcostal 4-chamber view
    • view which is optimally perpendicular to the atrial septum
    • eliminates the greatest degree of potential drop out
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17
Q

What is the most common associated anatomic lesion found with a sinus venosus ASD?

A

Anomalous right pulmonary venous connection

  • either a single RUPV or the RU and middle pulmonary veins insert anomalously to the SVC or the SVC-right atrial junction
  • these can also be located inferiorly near the entrance of the IVC into the RA
  • sinus venosus ASD’s are most commonly found in the superior portion of the atrial septum creating a “biatrial” insertion of the SVC
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18
Q

What is the most common associated anatomic lesion found with a inlet VSD’s?

A

AV septal defects

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19
Q

What is the most common associated anatomic lesion found with bicuspid aortic valve?

A

coarctation of the aorta

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20
Q

What is the most common associated congenital defect in a patient with Down Syndrome and an AV septal defect (AVSD)?

A

Tetralogy of Fallot

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21
Q

What is the most common anatomic finding in a complete AVSD?

A

LVOT is “sprung” anteriorly

  • LV inflow is shortened and LVOT is elongated (“goose-neck deformity”) –> LV inlet / LV outlet ratio < 1
  • Presence of a common AV valve –> Aortic Valve no longer wedged between AV valves and is pushed anteriorly (“sprung”)
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22
Q

What are the anatomic hallmarks of AVSD’s?

A
  • Cleft in the anterior leaflet of the left AV valve
  • Lateral rotation of the LV papillary muscles
  • Attachments of the left and right AV valves at the same level at the cardiac crux
  • LV inlet / LV outlet ratio < 1 (“goose-neck deformity)
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23
Q

What is the best echo view to delineate a subpulmonary (supracristal, doubly committed) VSD?

A

parasternl short axis view

  • can also be demonstrated from subcostal and apical windows with appropriate angulation
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24
Q

What is the most characteristic acquired lesion resulting from a subpulmonary (supracristal, doubly committed) VSD?

A

Aortic Insufficiency

  • occurs as a result of prolapse of the aortic cusp into the subpulmonary VSD
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25
What is the most characteristic physiologic effect of a large VSD?
**Equalization of the RV and LV pressures** as well as elevated pulmonary arterial pressure
26
What is the most common anatomic type of subaortic stenosis?
Discrete membrane * located proximal to the aortic valve within the LVOT * most often circumferential and can be adherent to both the aortic valve as well as the anterior leaflet of the mitral valve
27
When are the Glenn and Fontan procedures employed?
whenever a congenital anatomy requires routing blood from the systemic venous system to the pulmonary arterial system
28
Describe the Glenn procedure:
creation of a cavopulmonary connection between the SVC and the disconnected right pulmonary artery (RPA)
29
What is the major complication of the Glenn procedure? What is done to correct this complication?
* patients only perfused the right lung via the SVC --\> * pulmonary AV malformations (sometimes quite large) * cyanosis * Correction --\> bidirectional Glenn procedure which allowed blood to go to both lungs * attaching SVC to RPA + oversewing of pulmonary valve
30
Describe the Fontan procedure
* multiple ways to perform the procedure, all involve routing * IVC --\> lungs * Classic Fontan = IVC --\> right atrial appendage --\> main PA
31
Why is it important that the Glenn and Fontan procedures performed at different times?
* prevent competitive flow conflict * prevent increased pulmonary resistance * lung will not be accustomed to the flow
32
What is a solution to the initial high pressure in the Fontan conduit at placement?
* "fenestrated" release into the right atrium to allow "pop-off" flow to allow decreased pressure in the conduit * once lungs have adapted to the new increase in flow --\> fenestration is generally closed with a closure device
33
What pathophysiology causes the Fontan conduit to fail? What is required to allow the Fontan conduit to function correctlly?
* Pulmonary hypertension (from any cause) * pulmonary vascular disease, elevated pulmonary venous pressure from ventricular dysfunction, AV valve regurgitation * Systemic venous pressure must exceed the PA pressure and active early diastolic relaxation of the systemic ventricle be normal
34
What is the most common associated cardiac abnormality in a patient with coarctation of the aorta?
**Bicuspid Aortic vavle** * 80% of patients * ASD's and VSD's are also common
35
In patients with coarctation of the aorta, when is systemic arterial pressure significantly affected in regards to the aortic lumen narrowing?
**50%**
36
What is the most common type of VSD associated with coarctation of the aorta?
**Perimembranous** ## Footnote ​
37
What can posterior malalignment VSD cause?
Severe coarctation or Interruption of the aortic arch
38
What is a characteristic finding in patients with: * coarctation of the aorta * muscular VSD or inlet VSD
**unbalanced RV-dominant AVSD**
39
When are anterior malalignment VSD's commonly seen?
RV outflow obstruction * most notably - ToF
40
What syndrome can present with interruption of the aortic arch?
DiGeorge Syndrome * deletion in chromosome 22q11
41
Define aortic arch interruptions
* Type A * occurs distal to the left subclavian artery * Type B * occurs between the left common carotid and left subclavian arteries * Type C * occurs between the right inonimate and left common carotid arteries
42
Describe the findings and diagnosis
**Large membranous VSD** * PSAX * classic demonstration of membranous VSD adjacent to the TV * betweeen 10-12 oclock * PLAX * seen just below aortic valve
43
What is the best view to visualize muscular VSD's?
A4C and PLAX / PSAX
44
What anatomic type of VSD has been closed in this image?
**Trabecular muscular VSD**
45
What anatomic type of VSD is demonstrated in the PSAX view?
**Subpulmonary (supracristal) VSD** * defect in the subpulmonary region (supracristal) of the ventricular septum adjacent to the pulmonary valve * "infundibular" or "conal" VSD
46
What is the best way to differentiate mebranous and subpulmonary (supracristal, infundibular, conal) VSD's on PSAX?
* Membranous - adjacent to the TV * Inlet VSD also close proximity to TV * Subpulmonary - adjacent to PV
47
2 day old infant with respiratiory distress Describe the findings and diagnosis
**PDA with bidirectional shunting** * High left PSAX view demonstrates PDA * Color doppler * consistent with bidirectional shunt from aorta-to-PA (right-to-left shunting in systole and left-to-right shunting in diastole) * CW doppler * confirms bidirectional low-velocity shunting consistent with pulmonary hypertension
48
Describe the findings and diagnosis
**Subarotic membrane with moderate stenosis**
49
Describe the findings and diagnosis
**Primum ASD** * Large left-to-right shunt (arrow) * Both AV valves are inserted at the same level at the cardiac crux --\> consistent with AVSD with large primum component
50
Describe the findings and diagnosis
**Coarctation of the aorta** * Suprasternal long axis of the aortic arch * Juxtaductal coarcation of the aorta
51
Describe the diagnosis in this Doppler pattern of the abdominal aorta
**Coarctation of the aorta** * PW Doppler in the descending aorta * Classic findings: * delayed arterial upstroke * prominent diastolic runoff * absence of early diastolic flow reversal (hallmark of significant obstruction)
52
Describe normal Doppler pattern of the abdominal aorta
rapid arterial upstroke in systole brisk return to the Doppler baseline in early diastole brief diastolic flow reversal
53
What two conditions would demonstrate holodiastolic Doppler flow reversal in the abdominal aorta?
Severe AR Large PDA
54
Describe the findings and diagnosis
**Pulmonary Valve Stenosis**
55
Describe the Doppler pattern in a dynamic RV infundibular obstruction
Classic late peaking (dagger-shaped)
56
When is VSD closure not recommended (class III)?
**severe irreversible pulmonary arterial hypertension**
57
What is an additional finding (related to VSD) with perimembranous VSD?
* ventricular septal aneurysm * consisting of tricuspid tissue that has closed or partially closed the VSD
58
Describe view and location of color jet: * Supracristal VSD
* PSAX * between 12-2 oclock * AI with prolapse of right coronary cusp
59
Describe the difference (in flow) of VSD's
* Restrictive * RVSP \< 50% of the LV pressure * Nonrestrictive * RVSP \> 50% of the LV pressure
60
Describe the difference (in size) of VSD's
* Small (large gradient) * Qp/Qs \< 1.4 / 1.0 * Moderate * RV pressure ≤ 2/3 LV pressure * Qp/Qs = 2.2 / 1.0 = 1.4 * Large * RV pressure \> 2/3 LV pressure * Qp/Qs \> 2.2
61
Describe the diagnosis and findings What is the peak gradient based upon the Doppler velocity displayed?
**Small anterior muscular VSD with left-to-right shunting** * CW doppler velocity of 3 m/s --\> restrictive defect **36 mmHg**
62
What are the class I recommendations for closure of VSD?
* Qp / Qs flow ratio ≥ 2 and * Clinical evidence of LV volume overload * May be indicated with history of infective endocarditis
63
What percentage of muscular VSD's close spontaneously by late childhood?
**80-90%**
64
Describe the findings and diagnosis
**Secundum ASD**
65
What anatomic / hemodynamic factors dictate the direction in which blood flows across an ASD?
**Compliance of the ventricles** * RV is typically more compliant than the LV, which leads to L-to-R shunting Factors that also contribute (less important): * SVR * PVR * Atrial pressures * Size and morphology of ASD
66
What type of ASD's are amenable to device closure?
**Secundum ASD**
67
Describe the findings and diagonsis
**Coarctation of the aorta** * PW Doppler of the descending aorta * diastolic runoff consistent with significant proximal obstruction (juxtaductal coarctation of the proximal descending aorta)
68
What is the most common site of coarctation of the aorta (in infants and children)?
**Juxtaductal** * opposite the insertion site of the ductus arteriorsus * accompanited by a posterior infolding ("ledge") of thickened aortic wall media tissue
69
What is a typical scenario of neonatal collapse in the setting of sever coarctation of the aorta?
PDA closure
70
Describe the findings and diagnosis?
**PDA flow in the descending aorta** * following administration of Prostaglandin E
71
What will eliminate the classic findings on the abdominal aortic Doppler tracing of coarctation of the aorta?
Large PDA
72
Describe the findings and diagnosis
**Large Primum ASD** and small inlet VSD * AV valves are inserted at the same level at the cardiac crux --\> absence of the atrioventricular septum --\> large ASD * No VSD shunting
73
Describe the findings and diagnosis
**Complete AVSD** * Primum ASD and a large inlet VSD
74
What is one post-balloon valvotomy complication in pulmonic stenosis?
**Dynamic RVOT (infundibular) obstruction** * PE: loud, late-peaking systolic murmur + PR * Treatment: BB and eventual regression of RV hypertrophy --\> decreased osbstruction
75
What is the most common cyanotic congenital heart disease?
**Tetralogy of Fallot** * 4th most common of congenital heart disease (but most common cyanotic)
76
What are the class IIa recommendations for VSD closure?
* Reasonable when net left-to-right shunting is present: * Qp/Qs \> 1.5 and * Pulmonary artery pressure \< 2/3 of systemic pressure and * PVR \< 2/3 of SVR * Reasonable when net left-to-right shunting is present: * Qp/Qs \> 1.5 and * LV systolic or diastolic decompensation
77
What are the most common cyanotic congenital heart disease?
* Tetralogy of Fallot (10%) * Transposition of the great arteries (5%) * Truncus arteriosus (1-2%) * Tricuspid atresia (1-2%)
78
Patients with complete AVSD (unrepaired in childhood) are at risk for this adverse condition?
**Eisenmenger syndrome** * Irreversible pulmonary hypertension * Occurs when the pulmonary vascular bed is subjected to elevated pressures (as seen with large VSD)
79
What is the diagnosis: * Large VSD * malalignment with overrid of the semilunar valve * Overriding great vessel * Single large great artery giving rise to Aorta and PA
Truncus arteriosus * malalignment with overrid of the semilunar valve = "Truncal Valve"
80
What syndrome is associated with truncus arteriosus?
**Digeorge syndrome** * accounts for **33% of cases**
81
What is one lesion which is ductal (PDA) dependent?
Aortic or pulmonary valve atresia * defects which compromise great artery flow
82
In the setting of unrepaired Tetraology of Fallot, describe findings: * pressure in ventricles * TR regurgitant velocity * Pulmonary pressures
* Large VSD (present in these patients) --\> no real chance of closure or restriction --\> **pressures in the R and L ventricles will equalize at systemic levels** * **high tricuspid regurgitant velocity** is expected in all cases * unlikely (not impossible) that pulmonary hypertension could develop --\> pulmonary valve and subvalvular stenosis generally protect the pulmonary arterial bed
83
What is the diagnosis and cause of cyanosis: * large VSD (with right-to-left shunt) * posterior great artery appears to bifurcate into two arteries * PFO with small lef-to-right shunt * large PDA (with bidirectional shunt)
**Transposition of the great arteries and VSD** * VSD is usually large --\> pulmonary pressures = systemic pressures --\> PH is present
84
What cyanotic congenital heart defect is most likely to escape detection in childhood?
**Ebstein's anomaly of TV** * may be very mild and patients may have little, if any murmur * If no ASD present --\> will not be cyanotic or have much exercise intolerance * Ebstein's can present with severe cyanosis as a newborn, but is rare
85
Describe how these conditions will be detected in childhood: * Tetralogy of Fallot * Supracardiac total anomalous pulmonary venous return * Transposition of the Great Arteries * Tricuspid valve atresia
* Tetralogy of Fallot * asymptomatic murmur - not subtle, diagnosis within first few weeks of life * Supracardiac total anomalous pulmonary venous return * soft and subtle murmur * mild, if any, clinical cyanosis * rare to go undetected into adulthood * Transposition of the Great Arteries * usually presents with profound cyanosis as a newborn * Rare - in presence of VSD --\> can present later with heart murmur * Tricuspid valve atresia * murmur in childhood * rare to go undetected into adulthood
86
Cyanotic congenital heart disease is most frequently produced by what abnormality?
**Decreased pulmonary blood flow**
87
Saline contrast IV injection into the left antecubital vein yield early appearance of cavitations in the left atrium in a patient with a pulse oximetry reading of 90%. What is the most likely anatomic abnormality?
**Persistent left SVC to unroofed coronary sinus** * key element is unroofing of the coronary sinus --\> allows for right-to-left shunting of systemic venous blood flow directlyinto the left atrium --\> desaturation * also at risk for paradoxical embolism
88
Describe PAPVR connections
* Supracardiac * Left Inonimate (subclavian) vein * SVC * Azygos vein * Cardiac * RA * Coronary sinus * Infracardiac * IVC * Hepatic veins * Portal vein * Left gastric vein \*\*\*\*mixed connections
89
What is the next step in diagnosis: * large VSD * overriding great vessel
**Identify the pulmonary artery connection -** Tetraology of Fallot * the great vessel is not always the aorta - key is identifying the pulmonary artery * key for making diagnosis and predicting clinical course
90
What is the differential in a patient: * large malalignment VSD * overriding great vessel
* Tetralogy of Fallot * Truncus arteriosus * Double-outlet RV * D-transposition of the great arteries with VSD * Pulmonary atresia with VSD
91
What is one situation in which tricuspid atresia will be ductal (PDA) dependent?
Tricuspid atresia with transposition of the great arteries Frequently aortic coarctation present
92
Tricuspid Atresia will present in these two forms
* Normally Great Arteries (75%) * Transposition of the Great Arteries (25%)
93
What must all forms of tricuspid atresia have present for survival?
**ASD or PFO** * to allow egress of blood from the RA * does not have to be PDA dependent * more likely to occur in outflow obstruction rather than inflow obstruction
94
What is the name of the atrial switch procedure for transposition of the great arteries?
Mustard procedure
95
Describe the findings
Mustard procedure (Atrial switch)
96
What is a common complication of the Mustard Procedure (atrial switch)? What is the most common site?
* Pulmonary venous hypertension * **Obstruction to pulmonary venous return** can occur within the surgically created pulmonary venous baffle, which courses between the pulmonary veins and the TV
97
What is the name of the operation for Truncus Arteriosus?
Rastelli operation * separate pulmonary arteries form aorta * RV to pulmonary artery conduit * requires multiple replacements throughout lifetime * VSD patch closure
98
What is a common complication of Truncus arteriosus repair?
**Conduit stenosis** * does not grow with patient and subject to calcification * often detected as increasing TR or RVSP
99
What is the diagnosis and direction of shunting in the PDA? * O2 sat 60% * Large PDA * Large PFO * BP 70 / 50 mmHg * RVSP 90 mmHg (based on TR velocities)
Pulmonary hypertension - **continuous right-to-left PDA shunting** * normal cardiac anatomy and elevated pulmonary pressures are likely due to elevated pulmonary artery resistance --\> shunting away from pulmonary bed * occurs relatively frequntly in newborn nurseris --\> may be provoked by sever respiratory problems (Meconium aspirate)
100
Which area of the heart should be studied thoroughly with Echo that would most likely account for the problem in this patient? * Transposition of the great arteries (intact ventricular septum) * PDA present (maintained with Prostaglanding E1) * Cyanotic with arterial O2 60%-63%
**Atrial septum** * small or restricted ASD will result in cyanosis even in the presence of large PDA
101
How do patients with transposition of the great arteries survive?
* two parallel circulations and survive with areas where the systemic and pulmonary circulations can mix * Connections: * ASD (most effective) * VSD * PDA
102
Describe the findings and diagnosis
103
Describe the findings and diagnosis: * new born infant * O2 sat 90% * tachypneic
* Hypoplastic left heart syndrome (HLHS) * ductal dependent lesion * A4C * two atria with a single ventricle and a single AV valve * left atrium is small and there is no obvious second AV valve * tiny, diminutive ascending aorta (classic appearance when atretic aortic valve is present)
104
What are two things that are required in HLHS?
**ASD (left-to-right shunting)** **PDA must be maintained** * to supply blood to the systemic circulation * until Norwood (stage I) palliative surgery can be performed
105
Describe the findings and diagnosis
Ebstein anomaly
106
What is the diagnosis? Identify structures? * 1 day old newborn with cyanosis * O2 sat 75%, does not improve with 100% FiO2
**D-transposition of great arteries** **a = LV; b = RV; c = PV; d = AV** * defined by "ventriculoarterial discordance" and probably results from abnormal conotruncal separation
107
Describe the difference in transposition of the great arteries
* D-transposition ("dextro") * parallel course * aorta is located anterior and rightward of the centrally located pulmonary artery * L-transposition * parallel course * aorta is anterior and leftward of the centrally located pulmonary artery * Normal orientation * aorta and pulmonary artery are never in the same plane
108
Describe the diagnosis and findings What will determine the O2 saturation? * 4 week old girl with bluish discoloration (when crying) * O2 sat 70-80's * Systolic ejection murmur
* **Tetraology of Fallot** * Malalignment VSD with overriding aorta * narrowing of the RVOT, hypoplastic pulmonary valve and hypoplastic main PA * **Degree of RVOT obstruction** * saturation is determined by how much blood goes across the RVOT into the PA and thereby the degree of RVOT obstruction
109
What is the determining factor, in classifying ToF patients (clinically/anatomically)?
**RVOT obstruction (severity)** * "Pink" tetralogy * minimal narrowing/RVOT obstruction * Classic tetralogy * less flow to lungs, peripheral O2 sat will be lower than normal * may need shunt surgery (Blalock-Taussig) shunt) in early period * Pulmonary atresia/VSD (tetralogy with pulmonary atresia) * ductal dependent * very complex
110
Describe the findings and diagnosis
**Ebstein Anomaly** * TR + poor forward flow + potential for R-to-L shunt through an ASD or PFO = possibility of cyanosis
111
What is Uhl's syndrome?
extremely rare congenital heart defect ( \< 20 cases reported) characterized by almost total absence of the RV myocardium
112
Describe the findings and differential diagnosis for this image
**Large VSD with an overriding great vessel** * Differential includes defects with an anterior malalignment VSD * Coarcation of the aorta with D-malposed great arteries and VSD * Pulmonary atresia with VSD and right aortic arch * Tetralogy of Fallot with right aortic arch * Truncus arteriosus
113
What are findings in anterior malalignment of the ventricular septum?
* large VSD * some degree of obstruction of the anterior outflow
114
Describe the findings and diagnosis
**Truncus Arteriosus**
115
Describe the findings and diagnosis What is the next step? * newborn with O2 sat 80% on RA
**Pulmonary valve atresia** * no forward flow or regurgitation in the PV region ( = atresia) **Evaluation of the ventricular septum** * PV atresia divided into two categories: * Pulmonary atresia + intact ventricular septum ("hypoplastic right heart syndrome") * Pulmonary atresia + VSD * severe form of ToF
116
Describe the findings and diagnosis * tricuspid atresia following surgical palliation
**Atrial lateral tunnel that carries systemic venous blood flow to the pulmonary arteries** * Patient has undergone single ventricle palliation, consisting of a lateral tunnel Fontan operation * lateral tunnel always travels through the RA and connects the IVC to the pulmonary arteries *
117
Describe the findings and diagnosis * 3 week old female infant in respiratory distress * O2 sat - 80's
**Pulmonary venous confluence - TAPVR** * in TAPVR, pulmonary veins usually converge in the midline, posterior and superior to the left atrium * No direct connection to the LA = venous confluence
118
What is a major consideration in someone undergiong correction of ToF (relief of RVOT, transannular patch, closure of VSD)?
**Coronary anomalies** (5% of cases)
119
What are associated anomalies that require surgical planning when undergoing ToF repair?
* Valvular pulmonary stenosis (50-60%) * Right aortic arch (25%) - usually mirror image branching * ASD (15%) * Coronary anomalies (5% - especially left from right) * Additional muscular VSD (2%) * Unilateral absent pulmonary artery (rare)
120
What is the most common associated anomaly with D-TGA?
**VSD (40-50%)** * usually perimembranous
121
What are the two forms of D-TGA?
* Intact IVS * VSD (usually perimembranous)
122
What is the differential diagnosis for anterior malalignment VSD?
* Coarcation of the aorta with D-malposed great arteries and VSD * Pulmonary atresia with VSD and right aortic arch * Tetralogy of Fallot with right aortic arch * Truncus arteriosus
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