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: 10%
patent ductus arteriosus stats male to female ratio ____ in 1000 live births premature percentage: 2-3 times more likely than males
8 in 1000 live births
40%
patent ductus arteriosus what drug is given to close PDA after birth in most cases: indomethacin
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?: surgery-surgical
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
DOE
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: bluer
Patnet Ductus Arteriosus Shunt sizes are estimates at best and do not allow accurate calculation of _______: Resistances
The results of a PDA (5): Tachypnea
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 Angiography: Needed to provide accurate landmark for transcathater closure
Ventricular Septal Defect Two kinds:: Congenital

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
Subpulmonary
Muscular
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?: Asian
Subpulmonary VSD What is frequently associated with this-Aorta wise?: Prolapse of the right coronary cusp and AR
VSD Muscular Where can these occur?: Anywhere in the interventricular septum--
Most are "Midmuscular"
VSD 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?: Systole
VSD the afterload that each ventricle faces is related to ____ and _______ of shunting: size
direction
VSD 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
VSD 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
VSD 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
VSD 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: surgery
Truncus Arteriosus: Is characterized by a single arterial trunk arising from the normally formed ventricles by means of a single semilunar valve
Truncus Arteriosus atatsics 5-15 of every ______ birth without surgery 100% mortality by age 1 median death age: 100,000
2wks-3mos
male female equally
Truncus Arteriosus Signs and Symptoms: DX sometimes by US intero
Poor feeding
Diaphorises
tachpnea
cyanosis
Truncus Arteriosus Diagnosis: x-ray
echo
Cath
EKG
Truncus Arteriosus Treatment: Surgica-Must
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: AR
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 Physiology 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 ____: Carotids
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: True
Coarctation of the Aorta Isolated coarctations have no abnormalties in O2 Sats: True
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: NORMAL

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
1)-VSD
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
Echo
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: TRUE
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: Hyperventalation
Acidosis
Extreme desaturations
Unconsciousness
Tetralogy of Fallot Tetrad Treatment: Sedation
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
TGA What are the two main types: 1)Dextro-TGA Most Common
2)Levo-TGA
TGA In LTGA(Levo-TGA) which is less common the ventricles are inverted as well what is this called: L-loop
Levo-TGA waht alsmost always accompanies these: VSD sub pulmonary stenosis
DTGA: Two closed symptoms without defect pts would die in minutes after birth
ASD The most common of the congenital heart defects what are they: Ostium secundum
Ostium primum
Sinus venosus
ASD 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
ASD About half of pts are____ when dx made: asymtomatic
ASD Symptoms develop in 60% by age 30 T/F: True
ASD Advocate closing ASD at dx to prevent future complication: IE pulmonary hypertension
ASD Oximetry Data ASD are characterized by a _______ in the 02 content of the blood in the RA: stepup Rember in the RA
ASD 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
LVH
RV is ABSENT or NOT functional
Other lesions possible
Tricuspid Artresia Treatment: Palliative-Glenns
Corrective-Fontains
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): SVC
IVC
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 treatments: Plication(folding)of the RA(palliative)
TV replacement
Kawasaki Syndrome Other name: Mucocutaneous lymph node syndrome
Kawasaki Syndrome: inflammatory dx unknown etiology affecting children
Pallitive: Reducing the severity of
Alleveiating w/o cure
Correctiv: 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
Watterson-Cooleyshunt: 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
Fontan: 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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