(MHD) Congenital Heart Disease

Question 1

What are the (3) key shunts to consider with congenital heart disease?

Answer 1

1. Atrial Septal Defect
2. Ventricular Septal Defect
3. Patent Ductus Arteriosus

Question 2

What are the (2) key atrial septa defects to know?

Answer 2

1. Fossa Ovalis (secundum type)
2. Primum type (persistent ostium primum)

Question 3

Define an Atrial Septal Defect secundum type

Answer 3

Defect in the fossa ovalis

Question 4

What are the key hemodynamic changes associated with a secundum type atrial septal defect (2)?

Answer 4

1. Left to Right atrial shunt
2. Increased pulmonary flow

Question 5

What is the pathological effect that a Secundum type ASD will have on the architecture of the heart? (2)

Answer 5

1. RA and RV become hypertrophied and dilated
2. Dilation of tricuspid and pulmonary orifices and pulmonary trunk

Question 6

Define the Primum type ASD.

Answer 6

Defect distal to fossa ovalis, close to the mitral and tricuspid valves

Question 7

What are the key hemodynamic changes associated with a primum type ASD? (3)

Answer 7

1. Left to right atrial shunt
2. Increased pulmonary flow
3. Increased pulmonary vascular resistance/pulmonary hypertension which may develop later in life.

Question 8

What is the pathological effect that primum type ASD can have on the architecture of the heart? (4)

Answer 8

1. Cleft aortic leaflet of the mitral valve
2. RA and RV hypertrophy/dilation
3. Dilation of tricuspid and pulmonic orifices
4. LV hypertrophy IF mitral regurg/subaortic stenosis are present

Question 9

What hemodynamic changes are associated with Ventricular Septal Defects (VSD)?

Answer 9

1. RV and PA pressures are increased IF the defect is large
2. Left to Right shunt
3. Increased pulmonary flow
4. Increased pulmonary vascular resistance/ pulmonary hypertension may develop causing Right to Left shunt and cyanosis (if defect is large)

Question 10

Define Patent Ductus Arteriosus

Answer 10

PDA

Communication between aorta and left pulmonary artery distal to isthmus

Question 11

Key hemodynamic changes associated with PDA (3)

Answer 11

1. L to R shunt at ductus level
2. Increased pulmonary flow
3. If pulmonary vascular resistance is high, may have bidirectional shunt at ductus level

Question 12

What is the pathological effect that PDA has on the heart architecture?

Answer 12

1. RA/RV hypertrophied and dilated
2. LA/LV vary
3. w/o pulmonary hypertension, LA and LV hypertrophied and dilated

OR

w/ pulmonary hypertension, dilation of PA

Question 13

What (3) factors can lead to pulmonary hypertension?

Answer 13

1. Flow beyond the distensibility of the lung vasculature
2. Vasoconstriction of the pulmonary bed
3. Secondary pathologic changes in the arteries/arterioles of the lung, leading to restriction of the pulmonary bed

Question 14

Name the (3) obstructive lesios that don’t inlcude shunts

Answer 14

1. Isolated pulmonary stenosis
2. Isolated aortic stenosis
3. Coarctation of the aorta

Question 15

Hemodynamic changes associated with Isolated Pulmonary Stenosis

Answer 15

1. Elevated RV systolic pressure
2. NO SHUNTS (unless it is severe in which R to L via foramen ovale)

Question 16

Pathologic changes in the cardiac architecture associated with isolated pulmonary stenosis

Answer 16

1. RA and RV hypertrophied
2. Poststenotic dilatation of PA often present

Question 17

What are the (3) congenital isolated aortic stenosis types?

Answer 17

1. Valvular/ring
2. Supravalvular
3. Subaortic

Question 18

What hemodynamic changes are associated with Isolated Aortic Stenosis?

Answer 18

1. LV pressure elevation
2. NO SHUNTS (usually)
3. Laege LV to aortic systolic gradient/decreased pulse pressure in severe stenosis.

Question 19

What pathological changes to the cardiac architecture are associated with aortic stenosis?

Answer 19

1. Hypertrophied LV/LA

Question 20

Define Adult Coarctation

Answer 20

Constrictive narrowing of aorta in region of ligamentum arteriosum

Question 21

Hemodynamic changes/key points associated with adult coarctation (4)

Answer 21

1. LV and proximal aortic pressure elevation
2. Normal or low distal aortic pressure
3. No shunts
4. Pressure in arms GREATER than pressure in legs

Question 22

Pathologic changes to cardiac architecture associated with adult coarctation

Answer 22

1. LA and LV hypertrophy
2. Dilatation of ascending aorta

Question 23

Define fetal coarctation

Answer 23

Non-constricting long narrowing of aorta with pulmonary hypertension

Question 24

Hemodynamic changes associated with fetal coarctation (5)

Answer 24

1. RV and PA pressures elevated
2. L to R shunt at atrial level
3. R to L shunt at ductus level
4. Increaed pulmonary flow
5. Syanosis of lower extremities may be present

Question 25

Pathologic changes to cardiac architecture associated with _fetal coarctation (_6)

Answer 25

1. RA/RV hypertrophied and dilated
2. LA/LV atrophied
3. Dilatation of PA
4. Hypoplasia of aorta
5. ASD
6. Usually PDA

Question 26

What are the (4) characteristics associated with tetralogy of fallot? Which is most important?

Answer 26

1. Infundibular Pulmonary Stenosis
2. Right Ventricular Hypertrophy
3. Ventricular Septal Defect
4. Overriding Aorta

Question 27

Hemodynamic changes associated with tetrology of fallot (4)

Answer 27

1. High RV systolic pressure
2. Low PA pressure
3. Large R to L shunt (and small L to R shunt)
4. Decreased pulmonary flow (rarely increased)

Question 28

Name the pathologic changes to the cardiac architecture associated with Tetralogy of Fallot

Answer 28

1. RA and RV hypertrophy
2. LA and LV normal/atrophied