Glossary of Pacemaker Congenital heart disease

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patent ductus arteriosus
when what does not close after birth
ductus arteriosus
patent ductus arteriosus
what is the percent of common anomalies in children
patent ductus arteriosus
male to female ratio
____ in 1000 live births
premature percentage
2-3 times more likely than males
8 in 1000 live births
patent ductus arteriosus

what drug is given to close PDA after birth in most cases
past methods of diagnosis
physical exam and echo
patent ductus arteriosus

present method for diagnosis of PDA
Echo and cath
corrections can be done by?
during cath procedure
patent ductus arteriosus

Where are PDA's almost always located?
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
patent ductus arteriosus

What common shape do they have?
Hourglass shape with a prominent aortic diverticulum and a narrowing near the PA end
patent ductus arteriosus

PDA's are rarely large with exception to what two conditions
Downs Syndrome and
living at high altitude
patent ductus arteriosus

What is the "usual" PDA characterized by what three things
A stepup in PA blood oxygenation saturation
Some pulmonary hypertension
No change in aortic or RV blood oxygen saturation
When (age wise) will a pt with a small PDA likely to become symptomatic
60's 70's
patent ductus arteriosus

catherization technique
standard right sided catheter study to est hemodynamic effect of PDA
patent ductus arteriosus

How would you close a PDA?
Transcatheter closure is accomplished either from venous approach for double umbrella

Coil occlusion from a retrograde arterial route
patent ductus arteriosus
Signs and symptoms
If the lesion is hemodynamicall insignificant the pt is asymptomatic (50%)
Left to right shunt=Aorta to pulm artery
In 1/2 cases machine murmur high chest below clavicle
patent ductus arteriosus
oximetry date
Technically difficult
Left PA usually higher in oxygen that that of Right PA
patent ductus arteriosus with right to left shunt, descending aortic blood is ____ than ascending aortic blood
Patnet Ductus Arteriosus

Shunt sizes are estimates at best and do not allow accurate calculation of _______
The results of a PDA (5)
Increased workloaf of the heart-tachycardia
fluid in lung
right heart enlargement
increased lung workload
Patent Ductus Arteriosus

Pressure Data-
Most do not alter right/left pressures unless they are large
Patent Ductus Arteriosus

Needed to provide accurate landmark for transcathater closure
Ventricular Septal Defect

Two kinds:

Acquired/ dz process/ trauma
Ventricular Septal Defect

When are VSD "usually" diagnosed?
Early age, VSD murmurs are usually loud. Large defects produce symptoms early in life
Small defect tend to get smaller and close with age
Ventricular Septal Defect
What are four types of VSD's
Atrioventricular Canal type
Swiss cheese
Ventricular Septal Defect
Atrioventricular canal defect

Develelopmenttally what is the area related to
Ostium primum defects
Ventricular Septal Defect
Atrioventricular canal defect
where does atrioventricular canal type vsd occur
In the posterior interventricular septum adjacent to the atrioventicular
Ventricular Septal Defect
Atrioventricular canal defect

What occurs with EKG in atriventricular canal-type defect?
counterclockwise superior QRS
Atrioventricular canal defect
What syndrome is this defect most commonly associated with?
Down syndrome
Subpulmonary VSD
This kind results from a deficiency in _____ _______
Conal septum
Subpulmonary VSD
What race is more disposed to this?
Subpulmonary VSD

What is frequently associated with this-Aorta wise?
Prolapse of the right coronary cusp and AR
Where can these occur?
Anywhere in the interventricular septum--
Most are "Midmuscular"
Swiss cheese
Which side of the septum has large openings and which has small?
Left Large and right small
VSD Physiology
When does shunting occur for a VSD? systole or diastole?
the afterload that each ventricle faces is related to ____ and _______ of shunting
Most defects are closed or beomce small around what age?
Age 5
So if they are not showing signs or symptoms they are managed medically

Signes and symptoms
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
Symptoms in regards to the patient depend on what?
Size of the defect and age of Pt
VSD Symptoms with a small shunt
no significant effect
VSD symtoms with a large defect
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
With a larger defect may cause dyspnea adter what age
30-Uusally excertiona; and with a history of heart murmur since infancy
VSD Cath technique
Transcatheter double umbrella approach
For atrioventricular canal types what is still mainstay of correction
Truncus Arteriosus
Is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve
Truncus Arteriosus

5-15 of every ______ birth
without surgery 100% mortality by age 1
median death age
male female equally
Truncus Arteriosus
Signs and Symptoms
DX sometimes by US intero
Poor feeding
Truncus Arteriosus
Truncus Arteriosus
Additional surgical interventions highly probable
Truncus Arteriosus
Post repair stats
More than 80% lie 10-20- after surgery
Medications post surgery
Aortic Stenosis
Peak to peak measurements of LV and AO greater than 50 mmHg

Angiography obtained in both LV Ao
Aortic Stenosis

Where does the majoprity of Aortic stenosis occur
Valvular level
Aortic Stenosis
When is balloon valctomy recopmmended in neonate
Whenever peak to peak transvalular gradient is higher than 50 mmHg and associated with mild AR in older children
Aortic Stenosis
What are the tree types of Aortic stenosis
valular aortic stenosis
subvalvar aortic stenosis
supravalvar stenosis
Aortic Stenosis Valvar aortic stenosis
75% of these case have bicommissural valve with leaflet infusion
Aortic Stenosis
Valvar Aortic Stenosis
progressive in 1/3 pt lots of followup
Aortic Stenosis
Valvar Aortic Stenosis
Sounds hear
murmur.... constant aortic ejection click at apex
Aortic Stenosis
Subvalvar Aortic Stenosis
The obstruction is subvalvar owing to
thin fibrous ridge
Fibromuscular dyplasia of LV outflow tract-progressive
Aortic Stenosis
Subvalvar Aortic Stenosis
what is this know to casue when it has progresses
Aortic Stenosis
Subvalvar Aortic Stenosis

Surgery is indicated for significan stenosis/symptoms to prtect aortic valve, but is delayed until after ___ decade
first decade-growth
Aortic Stenosis
Supravalvar Aortic Stenosis
What shape is this deformaty? Where?1
hourglass? Just above the aortic valve
Aortic Stenosis
Supravalvar Aortic Stenosis
Caused by
At least partly by thickening of the supracoronary ridge, seen with Williams syndrome and branch PA stenosis
Aortic Stenosis oximetry Data
Coarctation of the Aorta
Localized malformation resulting in narrowing of the Aorta-commonly associated with other congenital heart disease like VSD, aortic stenosis and mitral stenosis
Coarctation of the Aorta
What makes this a commonly studied lesion
Increased use of balloon dilatation
Coarctation of the Aorta
Where do most coarcs occur?
Just distal to the left subclavian artery, at or near the level of the old ductus arteriosus
Coarctation of the Aorta

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

Rarely a coarctations invloved with what segments?
Long segment of thoracic/abdominal aorta
Coarctation of the Aorta

What influences the gradient across a coarctation
Degree of obstruction and degree of collateral flow around the obstruction
Coarctation of the Aorta

For collateral flow what arterys mostly (4)
Internal Mammary Artery
Intercostal Mammary
Scapular Artery
Epigastric Artery
Coarctation of the Aorta

Rarely will a coarctation steal Blood flow from the ____
Coarctation of the Aorta
What is a major "sign" of Coarctation of the Aorta?
Upper Extremity Hypertension
Coarctation of the Aorta
What are som things to remember about Coartation and hypertension
Usually resolves after surgery but may persist in some children depite
Persistant is more commonif repair is done late in childhood
Coarctation of the Aorta

What is recommended age of repair/DX
Before 3 y/o
Coarctation of the Aorta
Crossing a coarctation is usually not hazardous
Coarctation of the Aorta
Isolated coarctations have no abnormalties in O2 Sats
Coarctation of the Aorta

Pressure Data
Mild- systolic gradient across site with only min diastolic-as obstruction increases a gradient is present through cardiac cycle
Coarctation of the Aorta

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

Decreased= FAINT Femoral found on PE
Coarctation of the Aorta
What view will usually provide excellent visualization of the coarctation
Straight lateral aortagram
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
Precise measurement
Collateral adequacy
Coarctation of the Aorta

Interventional Catherization
Balloon dilation=reduction of gradient for unoperated coArc
Results in babies=Not as good as surgical
Coarctation of the Aorta
Interventional Catherization
Balloon w/ recurrent coArc invaluable w/ w/o stent
Surgical treatment the best
Tetralogy of Fallot
Defined as
Condition of the four elements
2)-Enlarged Right Ventricle
3)-Narrowing of the Pulmonary Valve
4)-Displaced/deviated Aorta
Tetralogy of Fallot
Symptoms at birth
Cyanosis-Blue Baby
Tetralogy of Fallot
catherization in these pts is hazard. Why? What is the alternative to diagnosis until surgery?
Cathaters Blocks O2
Tetralogy of Fallot
Besides the four defects what else can be a defect
Branch PA stenosis
-Pulmonary Blood Flow
-Additional Muscular VSD's
-Aortopulmonary collateral areteries supplying blood flow to the lungs
-Coronary arterial anomalies
Tetralogy of Fallot
What kind of shunt does the combination of pulmonary and a VSD create.
Right to Left shunt
Tetralogy of Fallot
Size of these shunts are unrelated to the size fo the VSD True or False
Tetralogy of Fallot
TOF shunts are almost large and Restrictive
TRUE Large and restrictive
Tetralogy of Fallot
Right to left shunt degree of cyanosis determined by
Degree of pulmonary obstruction
Tetralogy of Fallot
Tetrad spells
Extreme desaturations
Tetralogy of Fallot
Tetrad Treatment
IV Volume infusion
Increasing the systemic vascular resistance
Tetralogy of Fallot
With older TOF patients what tends to increase with time (2 things)
The pulmonary obstruction, thus the degree of eight to left shunting
Tetralogy of Fallot

What is done as a corrective procedure
Blalock Taussing shunt used to relieve symptoms
Tetralogy of Fallot
What should be noted about PA Pressures
They are decreased because of stenosis in cyanotic pts presurgery
Complete Transposition of the Great Vessels/Arteries
In TGA the great arteteris arise from the wrong ventricles

The AOrta from the RV and the PA from the LV
What are the two main types
1)Dextro-TGA Most Common
In LTGA(Levo-TGA) which is less common the ventricles are inverted as well what is this called
waht alsmost always accompanies these
VSD sub pulmonary stenosis
Two closed symptoms without defect pts would die in minutes after birth
The most common of the congenital heart defects
what are they
Ostium secundum
Ostium primum
Sinus venosus
What is the most common finding?
Left to Right shunt of the pulmonary venous return into the RA = high tricuspid flow in increased BF to the lungs
ASD what does the severity depend on?
Size of hole and relative compliances of R & L ventricles
About half of pts are____ when dx made
Symptoms develop in 60% by age 30
Advocate closing ASD at dx to prevent future complication
IE pulmonary hypertension
Oximetry Data

ASD are characterized by a _______ in the 02 content of the blood in the RA
stepup Rember in the RA
What is thee eq pressure hallmakr of a large ASD?
The equalization of atrial pressures
Tricuspid Artresia
ASD-All of the sytemic venous return reaches the left heart
RV is ABSENT or NOT functional
Other lesions possible
Tricuspid Artresia
Anomalous Venous Return
All the blood returning from the lungs enters the right heart-there MUST be an ASD
Anomalous Venous Return
What x-ray is characteristic of this
Snowman heart
Anomalous Venous Return
what is th emost common route to the right heart?(vessels)
Ebsteins Anomaly
Downward displacement of a portion of the tricusid valve, with atrialization of a large part of the right ventricle ASD=optional, usually more harmful
Ebsteins Anomaly
Plication(folding)of the RA(palliative)
TV replacement
Kawasaki Syndrome
Other name
Mucocutaneous lymph node syndrome
Kawasaki Syndrome
inflammatory dx unknown etiology affecting children
Reducing the severity of
Alleveiating w/o cure
Counteracting, modifying, change what is injured or diseased
Blalock Taussing shunt
Subclavian artery to PA anastomosis
Glenn Anastomosi-UNIdirectional
The SVC is attached to the Right PA and the Right PA is seperated from the Main and Left PA
Glenn Anstomosis bidirectional
The SVC is attached to the Right PA and the Right PA remains in continuity with the Main PA and Left PA
A connection is formed between the back of the Aorta and the right branch of PA
Potts shunt
A direct side to side anastamosis between the Aorta and the Left Branch of the PA
Rashkind Procedure
A ballon tipped catheter is inserted through the ASD, inflated, then pulled back through to widen the ASD
Mustard Procedure
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
Arterial switch-Janteen Procedure
The aorta and PA are switched to provide a normal circulation
2 stage first glenn second IVC attached to PA
Rastelli Repair
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
Pulmonary banding
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

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