Cardiac Congenital Defects Wong 25

Front 1

What are the 4 main circulatory changes at birth?

Back 1

1. pulmonary vasodilation
2. systemic pressure rises, pulmonary pressure falls
3. closure of the foramen ovale
4. closure of the ductus arteriosus and ductus venosum

Front 2

What is an atrial septal defect?

Back 2

abnormal opening between 2 atria (blood goes left to right); increases saturated blood on the right side and increases pulmonary blood flow

Front 3

What are the 3 types of atrial septal defect

Back 3

ASD 1 (ostium primum) opening at lower end of septum
ASD 2 (ostium secondum) opening at center of septum
sinus venosus defect (opening near junction of SVC)

Front 4

Signs/Symptoms of atrial septal defect

Back 4

May be asymptomatic
May develop CHF
Characteristic murmur
Risk of: atrial dyrhythmias, pulmonary vascular obstructive disease, emboli formation later in life

Front 5

What is the treatment for Atrial Septal Defect?

Back 5

Surgical: Dacron patch for large defects, may require replacement of mitral valve
Nonsurgical: ASD 2 may be closed using devices during cardiac catheterization

Front 6

What is ventricular septal defect? How is VSD classified?

Back 6

1. Abnormal opening between the 2 ventricles. Blood goes left to right--increase in pulmonary vascular resistance. Many VSDs spontaneously close during the 1st year of life.
2. Classified according to location: membranous (80%) or muscular.

Front 7

What are the s/s of ventricular septal defect? What are the complications of VSD?

Back 7

Clinically, you will hear a characteristic murmur. CHF is a common complication. Pt at risk for: 1) bacterial endocarditis, 2) pulmonary vascular obstructive disease and Eisenmenger syndrome

Front 8

What is the treatment for ventricular septal defect?

Back 8

Surgical treatment is closure of hole with purse string sutures or dacron patch repair. Non surgical closure during cardiac catheterization under clinical trials.

Front 9

How are Congenital Defects Classified (2)?

Back 9

1. Acyanotic: disorders where there is an increase of pulmonary blood flow or obstruction of blood flow from a ventricle
2) Cyanotic: disorders where there is a decrease in pulmonary blood flow or mixed blood flow

Front 10

What is Patent Ductus Arteriosus (PDA)?

Back 10

Failure of the ductus arteriosum to close completely--blood flows from the aorta to the pulmonary artery--extra blood then recirculates thru lungs and is returned to the left atrium. All this results in:
1) increased work for left ventricle
2) increase in pulmonary vascular congestion and resistance
3) increased right ventricular pressure

Front 11

What are the S/S of patent ductus arteriosus?

Back 11

1. machine like murmur
2. widened pulse pressure
3. bounding pulses

Front 12

What are the complications of patent ductus arteriosus?

Back 12

1. bacterial endocarditis
2. pulmonary vascular obstructive disease

Front 13

What are 3 treatments for patent ductus arteriosus?

Back 13

1. administration of indomethacin (prostaglandin inhibitor) sometimes closes pda
2. surgical intervention involves types of thoractomy (clipping ductus)
3. non-surgical use of coils to occlude pda during catheterization

Front 14

What is atrioventricular canal defect (AVC)?

Back 14

low atrial septal defect that is continuous with a high ventricular septal defect and clefts of the mitral and tricuspid valves, creating a large av valve that allows blood flow to all 4 chambers--most common defect of Downs syndrome

Front 15

What are the 4 signs and symptoms of atrioventricular canal defect?

Back 15

*moderate to severe CHF
* murmur
*cyanosis when crying
*high risk for pulmonary vas ob disease

Front 16

What is the treatment of atrioventricular canal defect?

Back 16

1. palliative: pulmonary artery banding with severe symptoms
2. complete surgical repair involves patch closure and reconstruction of valve tissue

Front 17

What are 5 post-op complications for pts with atrioventricular canal defect?

Back 17

1. heart block
2. chf
3. mitral regurgitation
4. dysrhythmias
5. pulmonary hypertension

Front 18

What s/s do patients with cardiac defects with increased pulmonary blood flow demonstrate?

Back 18

* s/s CHF
* risk of pulmonary vascular obstructive disease

Front 19

Describe defects with increased pulmonary blood flow

Back 19

Increased blood volume on the right side of the heart increases pulmonary blood flow at the expense of systemic blood flow.

Front 20

Describe obstructive defects

Back 20

Blood exiting the chamber meets an area of anatomic narrowing (stenosis). The pressure in the chamber before the obstruction is increased and the pressure beyond the obstruction is decreased. The end result is increased load on the ventricle and decreased cardiac output

Front 21

Describe the 3 main locations of obstructive defects (in relation to the valve)

Back 21

1. VALVULAR: at the site of the valve itself
2. SUBVAVLULAR: narrowing in the ventricle below the valve
3. SUPRAVALVULAR: narrowing in the great artery above the valve

Front 22

What signs/symptoms do you expect in patients with obstructive defects

Back 22

1. may be asymptomatic, but typically some level of CHF
2. severe may exhibit hypoxemia

Front 23

What is coarctation of the aorta

Back 23

Obstructive defect caused by a narrowing of the aorta near the insertion of the ductus arteriosis resulting in an increase in arterial pressure proximal to the defect (head and upper extremities) and decrease in pressure distal to the defect (body and lower extremities)

Front 24

What are the signs and symptoms of coarctation of the aorta?

Back 24

1.high blood pressure and bounding pulses in arms
2. weak/absent femoral pulses
3. CHF in infants
4. older children: dizziness, headaches, fainting & epitaxis (nosebleeds)

Front 25

What are 4 complications of coarctation of the aorta?

Back 25

Pts at risk for:
1. hypertension (risk of lifelong hypertension if not corrected prior to 2 years of age)
2. ruptured aorta
3. aortic aneurysm
4. stroke

Front 26

What is the treatment for coarctation of the aorta?

Back 26

Surgical: 1) resection of coarcted portion with anastomosis of aorta OR 2) enlargement of constricted portion with a prostetic graft or subclavian segment
NONSURGICAL treatment with balloon angioplasty

Front 27

What is aortic stenosis?

Back 27

Obstruction narrowing of the aortic valve causing resistance, decreased cardiac output, left ventricular hypertrophy, and pulmonary congestion.

Front 28

What are the 3 types of aortic stenosis?

Back 28

1. valvular--bicuspid or fusion of cusps
2. subvalvular:fibrous ring below valve
3. supravalvular: so rare, not described in book

Front 29

What are the three reasons why valvular aortic stenosis is so serious?

Back 29

1. obstruction is progressive
2. sudden episodes of ischemia, low cardiac ouput or MI result in sudden death
3. surgical repair rarely results in a normal valve

Front 30

What are the signs and symptoms of aortic stenosis

Back 30

Could range from asymptomatic to severe. S/S include decreased cardiac output, faint pulses, hypotension, tachycardia, poor feeding, exercise intolerance, chest pain and dizziness when standing for long periods

Front 31

What are the complications of aortic stenosis

Back 31

* Left vent. hypertrophy interferes with coronary artery perfusion which may result in MI, scarring of papillary muscles of l vent.
* also risk of bacterial endocarditis, coronary insufficiency and ventricular dysfunction

Front 32

What are the treatments for aortic stenosis

Back 32

1. Valvotomy: open the valve via balloon or surgery
2. Aortic valve replacement

Front 33

What are the 4 types of surgical valve replacement used for aortic stenosis

Back 33

1. Ross procedure: aortic valve replaced with pulmonary vavle--pulmonary valve replaced with donor valve
2. aortic valve replaced with donor valve
3. aortic valve replaced with mechanical valve
4. Konno procedure: part of the left ventricle is enlarged with aortic valve replacement

Front 34

What is pulmonary stenosis

Back 34

*Obstructive defect where a narrowing at the entrance to the pulmonary artery causes right ventricular hypertrophy and decreased pulmonary blood flow.
* If right vent failure, then right atr pressure increases and foramen ovale will open shunting deox blood into left atrium resulting in systemic cyanosis.
* If pulmonary stenosis is severe, CHF & systemic venous engorgement is evident
* Associated defect patent ductus arteriosis partially compensates for obstruction

Front 35

What are the s/s of pulmonary stenosis?

Back 35

*can range from asymptomatic to mild/mod cyanosis to CHF
*characteristic murmur present
*cardiomegaly seen on xray

Front 36

What are the complications of pulmonary stenosis

Back 36

* risk of bacterial endocarditis
* progressive worsening of symptoms (CHF)

Front 37

What is the treatment for pulmonary stenosis?

Back 37

Surgical treatment via Brock technique of valvotomy rare--more common nonsurgical technique of balloon angioplasty (few complications and highly effective)

Front 38

What is the Tetrology of Fallot (4 defects)?

Back 38

Cyanotic disorder characterized by decreased pulmonary flow due to the presence of 4 defects:
1. ventricular septal defect
2. pulmonary stenosis
3. overriding aorta
4. right ventricular hypertrophy
(the shunt direction depends on the difference between the systemic and pulmonary resistance)

Front 39

What are the s/s of Transposition of the Great Vessels

Back 39

Depends on the size and type of associated defects--some are severely cynaotic and depressed at birth--symptoms of CHF may be present

Front 40

What is the treatment for transposition of great vessels?

Back 40

1. initial administration of prostaglandin (or via balloon cath) to keep the ductus arteriosum patent
2. 2 main types of complete surgical correction:(1) creating a tunnel inside the atria to redirect blood to proper vessel--Rastelli procedure OR (2) switching the major arteries--

Front 41

What is the Transposition of the Great Vessels?

Back 41

When the aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle

Front 42

What are the s/s Tetrology of Fallot?

Back 42

Blue at birth or mild cyanosis that progresses over the 1st year of life as the pulmonic stenosis worsens
* Anoxic spells when the infant cries or eats

Front 43

What is the treatment for Tetrology of Fallot?

Back 43

1) palliative shunt to improve pulmonary blood flow
2) complete surgical repair