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Congenital Flashcards

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USMLE® Step 1 flashcards
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
Q

What is the most characteristic physiologic effect of a large VSD?

A

Equalization of the RV and LV pressures

as well as

elevated pulmonary arterial pressure

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26
Q

What is the most common anatomic type of subaortic stenosis?

A

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
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27
Q

When are the Glenn and Fontan procedures employed?

A

whenever a congenital anatomy requires routing blood from the systemic venous system to the pulmonary arterial system

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28
Q

Describe the Glenn procedure:

A

creation of a cavopulmonary connection between the SVC and the disconnected right pulmonary artery (RPA)

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29
Q

What is the major complication of the Glenn procedure?

What is done to correct this complication?

A
  • 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
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30
Q

Describe the Fontan procedure

A
  • multiple ways to perform the procedure, all involve routing
    • IVC –> lungs
  • Classic Fontan = IVC –> right atrial appendage –> main PA
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31
Q

Why is it important that the Glenn and Fontan procedures performed at different times?

A
  • prevent competitive flow conflict
  • prevent increased pulmonary resistance
    • lung will not be accustomed to the flow
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32
Q

What is a solution to the initial high pressure in the Fontan conduit at placement?

A
  • “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
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33
Q

What pathophysiology causes the Fontan conduit to fail?

What is required to allow the Fontan conduit to function correctlly?

A
  • 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
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34
Q

What is the most common associated cardiac abnormality in a patient with coarctation of the aorta?

A

Bicuspid Aortic vavle

  • 80% of patients
  • ASD’s and VSD’s are also common
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35
Q

In patients with coarctation of the aorta, when is systemic arterial pressure significantly affected in regards to the aortic lumen narrowing?

A

50%

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36
Q

What is the most common type of VSD associated with coarctation of the aorta?

A

Perimembranous

​

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37
Q

What can posterior malalignment VSD cause?

A

Severe coarctation

or

Interruption of the aortic arch

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38
Q

What is a characteristic finding in patients with:

  • coarctation of the aorta
    • muscular VSD or inlet VSD
A

unbalanced RV-dominant AVSD

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39
Q

When are anterior malalignment VSD’s commonly seen?

A

RV outflow obstruction

  • most notably - ToF
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40
Q

What syndrome can present with interruption of the aortic arch?

A

DiGeorge Syndrome

  • deletion in chromosome 22q11
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41
Q

Define aortic arch interruptions

A
  • 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
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42
Q

Describe the findings and diagnosis

A

Large membranous VSD

  • PSAX
    • classic demonstration of membranous VSD adjacent to the TV
    • betweeen 10-12 oclock
  • PLAX
    • seen just below aortic valve
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43
Q

What is the best view to visualize muscular VSD’s?

A

A4C and PLAX / PSAX

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44
Q

What anatomic type of VSD has been closed in this image?

A

Trabecular muscular VSD

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45
Q

What anatomic type of VSD is demonstrated in the PSAX view?

A

Subpulmonary (supracristal) VSD

  • defect in the subpulmonary region (supracristal) of the ventricular septum adjacent to the pulmonary valve
  • “infundibular” or “conal” VSD
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46
Q

What is the best way to differentiate mebranous and subpulmonary (supracristal, infundibular, conal) VSD’s on PSAX?

A
  • Membranous - adjacent to the TV
    • Inlet VSD also close proximity to TV
  • Subpulmonary - adjacent to PV
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47
Q

2 day old infant with respiratiory distress

Describe the findings and diagnosis

A

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
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48
Q

Describe the findings and diagnosis

A

Subarotic membrane with moderate stenosis

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49
Q

Describe the findings and diagnosis

A

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
Q

Describe the findings and diagnosis

A

Coarctation of the aorta

  • Suprasternal long axis of the aortic arch
  • Juxtaductal coarcation of the aorta
51
Q

Describe the diagnosis in this Doppler pattern of the abdominal aorta

A

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
Q

Describe normal Doppler pattern of the abdominal aorta

A

rapid arterial upstroke in systole

brisk return to the Doppler baseline in early diastole

brief diastolic flow reversal

53
Q

What two conditions would demonstrate holodiastolic Doppler flow reversal in the abdominal aorta?

A

Severe AR

Large PDA

54
Q

Describe the findings and diagnosis

A

Pulmonary Valve Stenosis

55
Q

Describe the Doppler pattern in a dynamic RV infundibular obstruction

A

Classic late peaking (dagger-shaped)

56
Q

When is VSD closure not recommended (class III)?

A

severe irreversible pulmonary arterial hypertension

57
Q

What is an additional finding (related to VSD) with perimembranous VSD?

A
  • ventricular septal aneurysm
    • consisting of tricuspid tissue that has closed or partially closed the VSD
58
Q

Describe view and location of color jet:

  • Supracristal VSD
A
  • PSAX
    • between 12-2 oclock
    • AI with prolapse of right coronary cusp
59
Q

Describe the difference (in flow) of VSD’s

A
  • Restrictive
    • RVSP < 50% of the LV pressure
  • Nonrestrictive
    • RVSP > 50% of the LV pressure
60
Q

Describe the difference (in size) of VSD’s

A
  • 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
Q

Describe the diagnosis and findings

What is the peak gradient based upon the Doppler velocity displayed?

A

Small anterior muscular VSD with left-to-right shunting

  • CW doppler velocity of 3 m/s –> restrictive defect

36 mmHg

62
Q

What are the class I recommendations for closure of VSD?

A
  • Qp / Qs flow ratio ≥ 2 and
  • Clinical evidence of LV volume overload
  • May be indicated with history of infective endocarditis
63
Q

What percentage of muscular VSD’s close spontaneously by late childhood?

A

80-90%

64
Q

Describe the findings and diagnosis

A

Secundum ASD

65
Q

What anatomic / hemodynamic factors dictate the direction in which blood flows across an ASD?

A

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
Q

What type of ASD’s are amenable to device closure?

A

Secundum ASD

67
Q

Describe the findings and diagonsis

A

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
Q

What is the most common site of coarctation of the aorta (in infants and children)?

A

Juxtaductal

  • opposite the insertion site of the ductus arteriorsus
  • accompanited by a posterior infolding (“ledge”) of thickened aortic wall media tissue
69
Q

What is a typical scenario of neonatal collapse in the setting of sever coarctation of the aorta?

A

PDA closure

70
Q

Describe the findings and diagnosis?

A

PDA flow in the descending aorta

  • following administration of Prostaglandin E
71
Q

What will eliminate the classic findings on the abdominal aortic Doppler tracing of coarctation of the aorta?

A

Large PDA

72
Q

Describe the findings and diagnosis

A

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
Q

Describe the findings and diagnosis

A

Complete AVSD

  • Primum ASD and a large inlet VSD
74
Q

What is one post-balloon valvotomy complication in pulmonic stenosis?

A

Dynamic RVOT (infundibular) obstruction

  • PE: loud, late-peaking systolic murmur + PR
  • Treatment: BB and eventual regression of RV hypertrophy –> decreased osbstruction
75
Q

What is the most common cyanotic congenital heart disease?

A

Tetralogy of Fallot

  • 4th most common of congenital heart disease (but most common cyanotic)
76
Q

What are the class IIa recommendations for VSD closure?

A
  • 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
Q

What are the most common cyanotic congenital heart disease?

A
  • Tetralogy of Fallot (10%)
  • Transposition of the great arteries (5%)
  • Truncus arteriosus (1-2%)
  • Tricuspid atresia (1-2%)
78
Q

Patients with complete AVSD (unrepaired in childhood) are at risk for this adverse condition?

A

Eisenmenger syndrome

  • Irreversible pulmonary hypertension
  • Occurs when the pulmonary vascular bed is subjected to elevated pressures (as seen with large VSD)
79
Q

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
A

Truncus arteriosus

  • malalignment with overrid of the semilunar valve = “Truncal Valve”
80
Q

What syndrome is associated with truncus arteriosus?

A

Digeorge syndrome

  • accounts for 33% of cases
81
Q

What is one lesion which is ductal (PDA) dependent?

A

Aortic or pulmonary valve atresia

  • defects which compromise great artery flow
82
Q

In the setting of unrepaired Tetraology of Fallot, describe findings:

  • pressure in ventricles
  • TR regurgitant velocity
  • Pulmonary pressures
A
  • 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
Q

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)
A

Transposition of the great arteries and VSD

  • VSD is usually large –> pulmonary pressures = systemic pressures –> PH is present
84
Q

What cyanotic congenital heart defect is most likely to escape detection in childhood?

A

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
Q

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
A
  • 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
Q

Cyanotic congenital heart disease is most frequently produced by what abnormality?

A

Decreased pulmonary blood flow

87
Q

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?

A

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
Q

Describe PAPVR connections

A
  • Supracardiac
    • Left Inonimate (subclavian) vein
    • SVC
    • Azygos vein
  • Cardiac
    • RA
    • Coronary sinus
  • Infracardiac
    • IVC
    • Hepatic veins
    • Portal vein
    • Left gastric vein

****mixed connections

89
Q

What is the next step in diagnosis:

  • large VSD
  • overriding great vessel
A

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
Q

What is the differential in a patient:

  • large malalignment VSD
  • overriding great vessel
A
  • Tetralogy of Fallot
  • Truncus arteriosus
  • Double-outlet RV
  • D-transposition of the great arteries with VSD
  • Pulmonary atresia with VSD
91
Q

What is one situation in which tricuspid atresia will be ductal (PDA) dependent?

A

Tricuspid atresia with transposition of the great arteries

Frequently aortic coarctation present

92
Q

Tricuspid Atresia will present in these two forms

A
  • Normally Great Arteries (75%)
  • Transposition of the Great Arteries (25%)
93
Q

What must all forms of tricuspid atresia have present for survival?

A

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
Q

What is the name of the atrial switch procedure for transposition of the great arteries?

A

Mustard procedure

95
Q

Describe the findings

A

Mustard procedure (Atrial switch)

96
Q

What is a common complication of the Mustard Procedure (atrial switch)?

What is the most common site?

A
  • 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
Q

What is the name of the operation for Truncus Arteriosus?

A

Rastelli operation

  • separate pulmonary arteries form aorta
  • RV to pulmonary artery conduit
    • requires multiple replacements throughout lifetime
  • VSD patch closure
98
Q

What is a common complication of Truncus arteriosus repair?

A

Conduit stenosis

  • does not grow with patient and subject to calcification
  • often detected as increasing TR or RVSP
99
Q

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)
A

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
Q

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%
A

Atrial septum

  • small or restricted ASD will result in cyanosis even in the presence of large PDA
101
Q

How do patients with transposition of the great arteries survive?

A
  • two parallel circulations and survive with areas where the systemic and pulmonary circulations can mix
  • Connections:
    • ASD (most effective)
    • VSD
    • PDA
102
Q

Describe the findings and diagnosis

A
103
Q

Describe the findings and diagnosis:

  • new born infant
  • O2 sat 90%
  • tachypneic
A
  • 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
Q

What are two things that are required in HLHS?

A

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
Q

Describe the findings and diagnosis

A

Ebstein anomaly

106
Q

What is the diagnosis? Identify structures?

  • 1 day old newborn with cyanosis
  • O2 sat 75%, does not improve with 100% FiO2
A

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
Q

Describe the difference in transposition of the great arteries

A
  • 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
Q

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
A
  • 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
Q

What is the determining factor, in classifying ToF patients (clinically/anatomically)?

A

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
Q

Describe the findings and diagnosis

A

Ebstein Anomaly

  • TR + poor forward flow + potential for R-to-L shunt through an ASD or PFO = possibility of cyanosis
111
Q

What is Uhl’s syndrome?

A

extremely rare congenital heart defect ( < 20 cases reported)

characterized by almost total absence of the RV myocardium

112
Q

Describe the findings and differential diagnosis for this image

A

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
Q

What are findings in anterior malalignment of the ventricular septum?

A
  • large VSD
  • some degree of obstruction of the anterior outflow
114
Q

Describe the findings and diagnosis

A

Truncus Arteriosus

115
Q

Describe the findings and diagnosis

What is the next step?

  • newborn with O2 sat 80% on RA
A

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
Q

Describe the findings and diagnosis

  • tricuspid atresia following surgical palliation
A

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
Q

Describe the findings and diagnosis

  • 3 week old female infant in respiratory distress
  • O2 sat - 80’s
A

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
Q

What is a major consideration in someone undergiong correction of ToF (relief of RVOT, transannular patch, closure of VSD)?

A

Coronary anomalies (5% of cases)

119
Q

What are associated anomalies that require surgical planning when undergoing ToF repair?

A
  • 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
Q

What is the most common associated anomaly with D-TGA?

A

VSD (40-50%)

  • usually perimembranous
121
Q

What are the two forms of D-TGA?

A
  • Intact IVS
  • VSD (usually perimembranous)
122
Q

What is the differential diagnosis for anterior malalignment VSD?

A
  • 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

Medical (69 decks)

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