Pacemaker Congenital Heart Disease

Front 1

patent ductus arteriosus
when what does not close after birth

Back 1

ductus arteriosus

Front 2

patent ductus arteriosus
what is the percent of common anomalies in children

Back 2

10%

Front 3

patent ductus arteriosus
stats
male to female ratio
____ in 1000 live births
premature percentage

Back 3

2-3 times more likely than males
8 in 1000 live births
40%

Front 4

patent ductus arteriosus

what drug is given to close PDA after birth in most cases

Back 4

indomethacin

Front 5

past methods of diagnosis

Back 5

physical exam and echo

Front 6

patent ductus arteriosus

present method for diagnosis of PDA

Back 6

Echo and cath

Front 7

corrections can be done by?

Back 7

surgery-surgical
during cath procedure

Front 8

patent ductus arteriosus

Where are PDA's almost always located?

Back 8

Off the underside of the aortic arch just distal to the orgin of the left subclavian artery, left of the trachea and proximal to the left main stem bronchus

Front 9

patent ductus arteriosus

What common shape do they have?

Back 9

Hourglass shape with a prominent aortic diverticulum and a narrowing near the PA end

Front 10

patent ductus arteriosus

PDA's are rarely large with exception to what two conditions

Back 10

Downs Syndrome and
living at high altitude

Front 11

patent ductus arteriosus

What is the "usual" PDA characterized by what three things

Back 11

A stepup in PA blood oxygenation saturation
Some pulmonary hypertension
No change in aortic or RV blood oxygen saturation

Front 12

When (age wise) will a pt with a small PDA likely to become symptomatic

Back 12

60's 70's

Front 13

patent ductus arteriosus

catherization technique

Back 13

standard right sided catheter study to est hemodynamic effect of PDA

Front 14

patent ductus arteriosus

How would you close a PDA?

Back 14

Transcatheter closure is accomplished either from venous approach for double umbrella

Coil occlusion from a retrograde arterial route

Front 15

patent ductus arteriosus
Signs and symptoms

Back 15

If the lesion is hemodynamicall insignificant the pt is asymptomatic (50%)
Left to right shunt=Aorta to pulm artery
DOE
In 1/2 cases machine murmur high chest below clavicle

Front 16

patent ductus arteriosus
oximetry date

Back 16

Technically difficult
Left PA usually higher in oxygen that that of Right PA

Front 17

patent ductus arteriosus with right to left shunt, descending aortic blood is ____ than ascending aortic blood

Back 17

bluer

Front 18

Patnet Ductus Arteriosus

Shunt sizes are estimates at best and do not allow accurate calculation of _______

Back 18

Resistances

Front 19

The results of a PDA (5)

Back 19

Tachypnea
Increased workloaf of the heart-tachycardia
fluid in lung
right heart enlargement
increased lung workload

Front 20

Patent Ductus Arteriosus

Pressure Data-

Back 20

Most do not alter right/left pressures unless they are large

Front 21

Patent Ductus Arteriosus

Angiography

Back 21

Needed to provide accurate landmark for transcathater closure

Front 22

Ventricular Septal Defect

Two kinds:

Back 22

Congenital

Acquired/ dz process/ trauma

Front 23

Ventricular Septal Defect

When are VSD "usually" diagnosed?

Back 23

Early age, VSD murmurs are usually loud. Large defects produce symptoms early in life
Small defect tend to get smaller and close with age

Front 24

Ventricular Septal Defect
What are four types of VSD's

Back 24

Atrioventricular Canal type
Subpulmonary
Muscular
Swiss cheese

Front 25

Ventricular Septal Defect
Atrioventricular canal defect

Develelopmenttally what is the area related to

Back 25

Ostium primum defects

Front 26

Ventricular Septal Defect
Atrioventricular canal defect
where does atrioventricular canal type vsd occur

Back 26

In the posterior interventricular septum adjacent to the atrioventicular

Front 27

Ventricular Septal Defect
Atrioventricular canal defect

What occurs with EKG in atriventricular canal-type defect?

Back 27

counterclockwise superior QRS

Front 28

Atrioventricular canal defect
What syndrome is this defect most commonly associated with?

Back 28

Down syndrome

Front 29

Subpulmonary VSD
This kind results from a deficiency in _____ _______

Back 29

Conal septum

Front 30

Subpulmonary VSD
What race is more disposed to this?

Back 30

Asian

Front 31

Subpulmonary VSD

What is frequently associated with this-Aorta wise?

Back 31

Prolapse of the right coronary cusp and AR

Front 32

VSD
Muscular
Where can these occur?

Back 32

Anywhere in the interventricular septum--
Most are "Midmuscular"

Front 33

VSD
Swiss cheese
Which side of the septum has large openings and which has small?

Back 33

Left Large and right small

Front 34

VSD Physiology
When does shunting occur for a VSD? systole or diastole?

Back 34

Systole

Front 35

VSD
the afterload that each ventricle faces is related to ____ and _______ of shunting

Back 35

size
direction

Front 36

VSD
Most defects are closed or beomce small around what age?

Back 36

Age 5
So if they are not showing signs or symptoms they are managed medically

Front 37

VSD

Signes and symptoms

Back 37

Left to right shunt in right ventricle. Only the right atrial flow is normal
Increased pulmonary flow
Usually low artery pressure however PHTN is possible

Front 38

VSD
Symptoms in regards to the patient depend on what?

Back 38

Size of the defect and age of Pt

Front 39

VSD Symptoms with a small shunt

Back 39

no significant effect

Front 40

VSD symtoms with a large defect

Back 40

Pulmonary to systemic flow ratio of 1.5:1, may casue dyspnea after age 30

Large defects w/ flow ratios 3:1 or greater=rare in adult
usually exertional dyspnea
hx of heart murmur since infancy

Front 41

VSD
With a larger defect may cause dyspnea adter what age

Back 41

30-Uusally excertiona; and with a history of heart murmur since infancy

Front 42

VSD Cath technique

Back 42

Transcatheter double umbrella approach

Front 43

For atrioventricular canal types what is still mainstay of correction

Back 43

surgery

Front 44

Truncus Arteriosus

Back 44

Is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve

Front 45

Truncus Arteriosus
atatsics

5-15 of every ______ birth
without surgery 100% mortality by age 1
median death age

Back 45

100,000
2wks-3mos
male female equally

Front 46

Truncus Arteriosus
Signs and Symptoms

Back 46

DX sometimes by US intero
Poor feeding
Diaphorises
tachpnea
cyanosis

Front 47

Truncus Arteriosus
Diagnosis

Back 47

x-ray
echo
Cath
EKG

Front 48

Truncus Arteriosus
Treatment

Back 48

Surgica-Must
Additional surgical interventions highly probable

Front 49

Truncus Arteriosus
Post repair stats

Back 49

More than 80% lie 10-20- after surgery
Medications post surgery

Front 50

Aortic Stenosis

Back 50

Aortic stenosis remains a common form of congenital heart disease in both children and adults

Front 51

Aortic Stenosis

Where does the majoprity of Aortic stenosis occur

Back 51

Valvular level

Front 52

Aortic Stenosis
When is balloon valctomy recopmmended in neonate

Back 52

Whenever peak to peak transvalular gradient is higher than 50 mmHg and associated with mild AR in older children

Front 53

Aortic Stenosis
What are the tree types of Aortic stenosis

Back 53

valular aortic stenosis
subvalvar aortic stenosis
supravalvar stenosis

Front 54

Aortic Stenosis Valvar aortic stenosis

Back 54

75% of these case have bicommissural valve with leaflet infusion

Front 55

Aortic Stenosis
Valvar Aortic Stenosis

Back 55

progressive in 1/3 pt lots of followup

Front 56

Aortic Stenosis
Valvar Aortic Stenosis
Sounds hear

Back 56

murmur.... constant aortic ejection click at apex

Front 57

Aortic Stenosis
Subvalvar Aortic Stenosis

Back 57

The obstruction is subvalvar owing to
thin fibrous ridge
Fibromuscular dyplasia of LV outflow tract-progressive

Front 58

Aortic Stenosis
Subvalvar Aortic Stenosis
what is this know to casue when it has progresses

Back 58

AR

Front 59

Aortic Stenosis
Subvalvar Aortic Stenosis

Surgery is indicated for significan stenosis/symptoms to prtect aortic valve, but is delayed until after ___ decade

Back 59

first decade-growth

Front 60

Aortic Stenosis
Supravalvar Aortic Stenosis
What shape is this deformaty? Where?1

Back 60

hourglass? Just above the aortic valve

Front 61

Aortic Stenosis
Supravalvar Aortic Stenosis
Caused by

Back 61

At least partly by thickening of the supracoronary ridge, seen with Williams syndrome and branch PA stenosis

Front 62

Aortic Stenosis oximetry Data

Back 62

None

Front 63

Aortic Stenosis

Back 63

Peak to peak measurements of LV and AO greater than 50 mmHg

Angiography obtained in both LV Ao

Front 64

Coarctation of the Aorta

Back 64

Localized malformation resulting in narrowing of the Aorta-commonly associated with other congenital heart disease like VSD, aortic stenosis and mitral stenosis

Front 65

Coarctation of the Aorta
What makes this a commonly studied lesion

Back 65

Increased use of balloon dilatation

Front 66

Coarctation of the Aorta
Where do most coarcs occur?

Back 66

Just distal to the left subclavian artery, at or near the level of the old ductus arteriosus

Front 67

Aortic Stenosis oximetry Data

Back 67

None

Front 68

Aortic Stenosis

Back 68

Peak to peak measurements of LV and AO greater than 50 mmHg

Angiography obtained in both LV Ao

Front 69

Coarctation of the Aorta

Back 69

Localized malformation resulting in narrowing of the Aorta-commonly associated with other congenital heart disease like VSD, aortic stenosis and mitral stenosis

Front 70

Coarctation of the Aorta
What makes this a commonly studied lesion

Back 70

Increased use of balloon dilatation

Front 71

Coarctation of the Aorta
Where do most coarcs occur?

Back 71

Just distal to the left subclavian artery, at or near the level of the old ductus arteriosus

Front 72

Aortic Stenosis oximetry Data

Back 72

None

Front 73

Aortic Stenosis

Back 73

Peak to peak measurements of LV and AO greater than 50 mmHg

Angiography obtained in both LV Ao

Front 74

Coarctation of the Aorta

Back 74

Localized malformation resulting in narrowing of the Aorta-commonly associated with other congenital heart disease like VSD, aortic stenosis and mitral stenosis

Front 75

Coarctation of the Aorta
What makes this a commonly studied lesion

Back 75

Increased use of balloon dilatation

Front 76

Coarctation of the Aorta
Where do most coarcs occur?

Back 76

Just distal to the left subclavian artery, at or near the level of the old ductus arteriosus

Front 77

Coarctation of the Aorta

Describe the coarc

Back 77

Coarctations have discrete "curtains" of tissue indenting the POSTERIOR wall of the aorta-albeit may be assoc/with hypoplasi of the transvers aortic arch

Front 78

Coarctation of the Aorta

Rarely a coarctations invloved with what segments?

Back 78

Long segment of thoracic/abdominal aorta

Front 79

Coarctation of the Aorta

Physiology
What influences the gradient across a coarctation

Back 79

Degree of obstruction and degree of collateral flow around the obstruction

Front 80

Coarctation of the Aorta

For collateral flow what arterys mostly (4)

Back 80

Internal Mammary Artery
Intercostal Mammary
Scapular Artery
Epigastric Artery

Front 81

Coarctation of the Aorta

Rarely will a coarctation steal Blood flow from the ____

Back 81

Carotids

Front 82

Coarctation of the Aorta
What is a major "sign" of Coarctation of the Aorta?

Back 82

Upper Extremity Hypertension

Front 83

Coarctation of the Aorta
What are som things to remember about Coartation and hypertension

Back 83

Usually resolves after surgery but may persist in some children depite
Persistant is more commonif repair is done late in childhood

Front 84

Coarctation of the Aorta

What is recommended age of repair/DX

Back 84

Before 3 y/o

Front 85

Coarctation of the Aorta
Crossing a coarctation is usually not hazardous

Back 85

True

Front 86

Coarctation of the Aorta
Isolated coarctations have no abnormalties in O2 Sats

Back 86

True

Front 87

Coarctation of the Aorta

Pressure Data

Back 87

Mild- systolic gradient across site with only min diastolic-as obstruction increases a gradient is present through cardiac cycle

Front 88

Coarctation of the Aorta

Pts will have ____ upper extremity BP and _______ BP in lower extremity

Back 88

NORMAL

Decreased= FAINT Femoral found on PE

Front 89

Coarctation of the Aorta
What view will usually provide excellent visualization of the coarctation

Back 89

Straight lateral aortagram

Front 90

Coarctation of the Aorta

If the coarctation site is balloon-occluded and pressures are measured in the descending aorta you should get a _____ measurement of

Back 90

Precise measurement
Collateral adequacy

Front 91

Coarctation of the Aorta

Interventional Catherization

Back 91

Balloon dilation=reduction of gradient for unoperated coArc
Results in babies=Not as good as surgical

Front 92

Coarctation of the Aorta
Interventional Catherization

Back 92

Balloon w/ recurrent coArc invaluable w/ w/o stent
Surgical treatment the best

Front 93

Tetralogy of Fallot
Defined as

Back 93

Condition of the four elements
1)-VSD
2)-Enlarged Right Ventricle
3)-Narrowing of the Pulmonary Valve
4)-Displaced/deviated Aorta

Front 94

Tetralogy of Fallot
Symptoms at birth

Back 94

Cyanosis-Blue Baby

Front 95

Tetralogy of Fallot
catherization in these pts is hazard. Why? What is the alternative to diagnosis until surgery?

Back 95

Cathaters Blocks O2
Echo

Front 96

Tetralogy of Fallot
Besides the four defects what else can be a defect

Back 96

Branch PA stenosis
-Pulmonary Blood Flow
-Additional Muscular VSD's
-Aortopulmonary collateral areteries supplying blood flow to the lungs
-Coronary arterial anomalies

Front 97

Tetralogy of Fallot
What kind of shunt does the combination of pulmonary and a VSD create.

Back 97

Right to Left shunt

Front 98

Tetralogy of Fallot
Size of these shunts are unrelated to the size fo the VSD True or False

Back 98

TRUE

Front 99

Tetralogy of Fallot
TOF shunts are almost large and Restrictive

Back 99

TRUE Large and restrictive

Front 100

Tetralogy of Fallot
Right to left shunt degree of cyanosis determined by

Back 100

Degree of pulmonary obstruction

Front 101

Tetralogy of Fallot
Tetrad spells

Back 101

Hyperventalation
Acidosis
Extreme desaturations
Unconsciousness

Front 102

Tetralogy of Fallot
Tetrad Treatment

Back 102

Sedation
IV Volume infusion
Increasing the systemic vascular resistance

Front 103

Tetralogy of Fallot
With older TOF patients what tends to increase with time (2 things)

Back 103

The pulmonary obstruction, thus the degree of eight to left shunting

Front 104

Tetralogy of Fallot

What is done as a corrective procedure

Back 104

Blalock Taussing shunt used to relieve symptoms

Front 105

Tetralogy of Fallot
What should be noted about PA Pressures

Back 105

They are decreased because of stenosis in cyanotic pts presurgery

Front 106

Complete Transposition of the Great Vessels/Arteries

Back 106

In TGA the great arteteris arise from the wrong ventricles

The AOrta from the RV and the PA from the LV

Front 107

TGA
What are the two main types

Back 107

1)Dextro-TGA Most Common
2)Levo-TGA

Front 108

TGA
In LTGA(Levo-TGA) which is less common the ventricles are inverted as well what is this called

Back 108

L-loop

Front 109

Levo-TGA
waht alsmost always accompanies these

Back 109

VSD sub pulmonary stenosis

Front 110

DTGA

Back 110

Two closed symptoms without defect pts would die in minutes after birth

Front 111

ASD
The most common of the congenital heart defects
what are they

Back 111

Ostium secundum
Ostium primum
Sinus venosus

Front 112

ASD
What is the most common finding?

Back 112

Left to Right shunt of the pulmonary venous return into the RA = high tricuspid flow in increased BF to the lungs

Front 113

ASD what does the severity depend on?

Back 113

Size of hole and relative compliances of R & L ventricles

Front 114

ASD
About half of pts are____ when dx made

Back 114

asymtomatic

Front 115

ASD
Symptoms develop in 60% by age 30
T/F

Back 115

True

Front 116

ASD
Advocate closing ASD at dx to prevent future complication

Back 116

IE pulmonary hypertension

Front 117

ASD
Oximetry Data

ASD are characterized by a _______ in the 02 content of the blood in the RA

Back 117

stepup Rember in the RA

Front 118

ASD
What is thee eq pressure hallmakr of a large ASD?

Back 118

The equalization of atrial pressures

Front 119

Tricuspid Artresia

Back 119

ASD-All of the sytemic venous return reaches the left heart
LVH
RV is ABSENT or NOT functional
Other lesions possible

Front 120

Tricuspid Artresia
Treatment

Back 120

Palliative-Glenns
Corrective-Fontains

Front 121

Anomalous Venous Return

Back 121

All the blood returning from the lungs enters the right heart-there MUST be an ASD

Front 122

Anomalous Venous Return
What x-ray is characteristic of this

Back 122

Snowman heart

Front 123

Anomalous Venous Return
what is th emost common route to the right heart?(vessels)

Back 123

SVC
IVC

Front 124

Ebsteins Anomaly

Back 124

Downward displacement of a portion of the tricusid valve, with atrialization of a large part of the right ventricle ASD=optional, usually more harmful

Front 125

Ebsteins Anomaly
treatments

Back 125

Plication(folding)of the RA(palliative)
TV replacement

Front 126

Kawasaki Syndrome
Other name

Back 126

Mucocutaneous lymph node syndrome

Front 127

Kawasaki Syndrome

Back 127

inflammatory dx unknown etiology affecting children

Front 128

ASD
What is thee eq pressure hallmakr of a large ASD?

Back 128

The equalization of atrial pressures

Front 129

Tricuspid Artresia

Back 129

ASD-All of the sytemic venous return reaches the left heart
LVH
RV is ABSENT or NOT functional
Other lesions possible

Front 130

Tricuspid Artresia
Treatment

Back 130

Palliative-Glenns
Corrective-Fontains

Front 131

Anomalous Venous Return

Back 131

All the blood returning from the lungs enters the right heart-there MUST be an ASD

Front 132

Anomalous Venous Return
What x-ray is characteristic of this

Back 132

Snowman heart

Front 133

Pallitive

Back 133

Reducing the severity of
Alleveiating w/o cure

Front 134

Correctiv

Back 134

Counteracting, modifying, change what is injured or diseased

Front 135

Blalock Taussing shunt

Back 135

Subclavian artery to PA anastomosis

Front 136

Glenn Anastomosi-UNIdirectional

Back 136

The SVC is attached to the Right PA and the Right PA is seperated from the Main and Left PA

Front 137

Glenn Anstomosis bidirectional

Back 137

The SVC is attached to the Right PA and the Right PA remains in continuity with the Main PA and Left PA

Front 138

Watterson-Cooleyshunt

Back 138

A connection is formed between the back of the Aorta and the right branch of PA

Front 139

Potts shunt

Back 139

A direct side to side anastamosis between the Aorta and the Left Branch of the PA

Front 140

Rashkind Procedure

Back 140

A ballon tipped catheter is inserted through the ASD, inflated, then pulled back through to widen the ASD

Front 141

Mustard Procedure

Back 141

The surgeon uses synthetic material to redirect blood flow at the level of the atria. The new baffle or tunnel carries venous blood across the atrial septum to the left ventricle for oxygenation in the lungs then oxygenation is alloowed to flow around the baffle to the right ventricle, wherre it is sent throught the aorta to body

Front 142

Arterial switch-Janteen Procedure

Back 142

The aorta and PA are switched to provide a normal circulation

Front 143

Fontan

Back 143

2 stage first glenn second IVC attached to PA

Front 144

Rastelli Repair

Back 144

Holes in the heart are covered with fabric patches. Valves that were missing at birth may be replaced using a valved conduit that may contain either organ tissue or man made

Front 145

Pulmonary banding

Back 145

A narrow type band is placed around the PA and tightened so that its pressure is exactly one-half of the pressure of the aorta