The Long View- Congenital Heart Disease Natural History and Outcomes

Question 1

ASD - what type of shunt

Answer 1

Left to right

Question 2

Consequence of ASD

a) volume loads
b) volume deprives

Answer 2

a) volume loads
- Right atrium
- Right ventricle
- pulmonary artery
b) volume deprives
- left ventricle
- aorta

Question 3

Net consequence of ASD

Answer 3

• compromised CO

- cardiac and pulmonary work chronically increased

Question 4

Clinical manifestation of ASD

Answer 4

• relatively quiet (not as much pressure in atria vs ventricles)
• growth restriction
• respiratory symptoms
• but impact proportionate to size and dynamics change with age

Question 5

Holt Oram syndrome

Answer 5

-associated with ASD
Holt–Oram syndrome is an autosomal dominant disorder that affects bones in the arms and hands (the upper limbs) and may also cause heart problems -Wikipedia

Question 6

Long term impact of ASD (when, effects -4)

Answer 6

progressive functional impairment through 3-5th decades
Can cause:
1) Arrhythmia
2) RV dysfunction
3) Paradoxical embolic phenomena
4) Pulmonary vascular disease (?)

Question 7

Paradoxical embolic phenomena

Answer 7

• normally clot occurs in the veins breaks off and travels through right side of heart before being lodged in pulmonary circulation
• if there is a defect in either atria or ventricles then technically clot can bypass the lungs and pass to the left side of the heart and cause a stroke (or MI?)

Question 8

Intervention ASD

Answer 8

• percutaneous or surgical intervention in the first decade of life is ideal
• in adults repair not as successful (think of the permanent changes that will have occured)

Question 9

VSD - consequence of small defect

Answer 9

• usually clinically fine (maladie roger) and get spontaneous closure in many
• lifetime risk of infective endocarditis (5%, and risk increases with age)

Question 10

Consequences of moderate VSD

1) Volume loads:
2) Pressure loads:

Answer 10

1) Volume loads:
- pulmonary vasculature
- LA, LV
2) Pressure loads:
- RV/PA (depending on size/shunt)
- some may still become smaller and close

Question 11

Consequences of large VSD:

1) Volume & pressure loads
2) Net consequences

Answer 11

1) RV, PA –> LA, LV
2) CHF, failure to thrive in infancy, pulmonary vascular disease (in consequence to offset and balance of shunt), infection

Question 12

Treatment VSD

Answer 12

• need to operate early on
• typically done under 6 months
• highly successful if done in infancy (>95% cumulative long term survival)

Question 13

Risk for patent ductus arteriosus

Answer 13

Extreme premature infants - failure to signal closure

Question 14

Treatment patent ductus arteriosus + risks

Answer 14

-in premature infants - indomethacin (stimulates muscles in PDA to constrict)
-surgery :
PDA ligation (cardiac catherization - catheter inserted into bv guided up to heart and then coil passed into open PDA to stop blood flow through it)
Risk
-foreign body = risk of infection or embolization

Question 15

Pulmonary hypertension

a) what is it
b) what are its causes

Answer 15

• elevated pulmonary artery pressure
  either
  a) Primary PHT - a specific disease entity
  b) Secondary PHT - large VSD, direct pressure L –> R ventricle

Question 16

Why congestive heart diseases leads to pulmonary vascular disease

Answer 16

a) ASD -rarely
b) VSD - yes
c) PDA - yes
d) Tetralogy - No
e) TGA - yes
f) Coarctation -no

Question 17

Obstructive lesions (2 types)

Answer 17

• partial obstruction to flow caused by :
  a) stenosis (valves)
  b) coarctation (vessels)

Question 18

Consequences of obstructive lesions

Answer 18

• elevated afterload of the affected ventricle

- diastolic supply/demand problem for the hypertrophied mycardium

Question 19

Coarctation of aorta -consequence (where is causes pressure overload)

Answer 19

• leads to pressure load on:
  a) ascending aorta
  b) aortic valve
  c) left ventricle
  d) left atrium

Question 20

Consequence of coarctation of aorta - conditions

Answer 20

1) Chronic hypertension
2) Left ventricular hypertrophy
3) Cerebrovascular event
4) Rupture of aorta
5) INfective endocarditis
6) Angina (due to LV failure)

Question 21

Syndromes associated with coarctation

Answer 21

• turner (45XO)

- DiGeorge (22 Q deletion) syndrome

Question 22

Age morbidity coarctation

Answer 22

-early teens

Question 23

Coarctation interventions

Answer 23

1) Surgery
- neonatal or childhood repair
2) Angioplasty
- for recurrent coarctation or in teens/adults