Congenital Heart Disease

Question 1

Bicuspid aortic valves are often undetected at birth. TRUE/FALSE?

Answer 1

TRUE
children are normally asymptomatic

Question 2

Why do patients with bicuspid aortic valves eventually need valve replacement?

Answer 2

Develop aortic stenosis

May also develop aortic regurgitation predisposing to infective endocarditis +/- aortic dilatation/dissection

Question 3

What accelerates complications in patients with bicuspod aortic valves?

Answer 3

Intense exercise
=> beware in athletes

Question 4

Describe the two types of Atrial Septal Defect (ASD) and when they tend to present

Answer 4

Ostium SECUNDUM (80%)
- high in septum
- asymptomatic until adulthood when L to R shunt develops
- presents with SOB/HF age 40-60

Ostium PRIMUM (20%)
- low in septum near AV
- associated with Down’s Syndrome
- symptomatic in childhood

Question 5

Symptoms of Atrial Septal Defect (ASD)

Answer 5

chest pain
palpitations
SOB
arrhythmias

Question 6

Signs of ASD on examination

Answer 6

Increased JVP
Wide fixed split S2
Pulmonary systolic murmur
Pulm/Tricuspid Regurg.

Question 7

ECG findings in ASD

Answer 7

RBBB with left axis deviation (PRIMUM)
RBBB with right axis deviation (SECUNDUM)

Question 8

CXR findings in ASD

Answer 8

Small aortic knuckle
atrial enlargement

Question 9

Complications of ASD

Answer 9

Reversal of L to R shunt (Eisenmenger’s syndrome)

the L to R shunt causes chronic pulmonary HTN and high R heart pressures. These can then exceed the L and cause a R to L shunt

Question 10

Symptoms of Eisenmengers syndrome

Answer 10

Cyanosis
R to L shunt causes deoxygenated blood to enter systemic circulation

Paradoxical emboli
- venous clots enter arterial circulation via ASD

Question 11

Management of ASD

Answer 11

May close spontaneously
PRIMUM - need closed in childhood if symptomatic
SECUNDUM - closed if symptomatic or signs of RV overload

transcatheter closure

Question 12

Symptoms of VSD

Answer 12

severe HF in infancy
may be asymptomatic and found later in life

Question 13

Signs of VSD on examination

Answer 13

harsh pansystolic murmur at left sternal edge
systolic thrill
Left parasternal heave

Question 14

Complications of VSD

Answer 14

Aortic regurgitation
Infective endocarditis
Pulmonary hypertension
Eisenemenger’s syndrome
Heart failure

Question 15

ECG findings in VSD

Answer 15

Normal
Left axis deviation
LVH

Question 16

CXR findings in VSD

Answer 16

normal if VSD is small
cardiomegaly and marked pulmonary plethora if large VSD

Question 17

Treatment of VSD

Answer 17

Many close spontaneously
Surgery if symptomatic and medical tx not helping
if shunt >3:1
infective endocarditis

Question 18

What condition’s hallmark is a narrowing of the descending aorta just distal to the origin of the left subclavian artery?

Answer 18

Coarctation

Question 19

Is coarctation of the aorta more common in males or females?

Answer 19

Males

Question 20

Name 2 conditions that aortic coarctation is associated with

Answer 20

Bicuspid aortic valve
Turners syndrome

Question 21

Signs of coarctation of aorta on examination

Answer 21

radiofemoral delay
weak femoral pulse
raised BP
scapular bruit/systolic murmur
Cold feet

Question 22

Complications of aortic coarctation

Answer 22

Heart failure (increased afterload)
infective endocarditis
intracerebral haemorrhage

Question 23

CXR finding in aortic coarctation

Answer 23

rib notching

(blood diverted via intercostal arteries which dilate and erode local ribs)

Question 24

Treatment of coarctation

Answer 24

Surgery:
Balloon dialtation +/- stenting

Question 25

What causes Tetralogy of Fallot?

Answer 25

Abnormality in separation of truncus arteriosus into aorta and pulmonary arteries early in gestation

Question 26

4 features of Tetralogy of Fallot

Answer 26

VSD
Pulmonary stenosis
Overriding aorta
RVH

Question 27

How do children normally present with TOF?

Answer 27

Agitated and restless during hypoxic spells
They often “squat” to increase peripheral vascular resistance and reduce the R to L shunt

Question 28

Symptoms of TOF in an adult who has had surgical repair

Answer 28

SOBOE
palpitations
clubbing
RV failure
syncope
death

Question 29

ECG findings in Tetralogy of Fallot

Answer 29

RVH and RBBB

Question 30

CXR findings in Tetralogy of Fallot

Answer 30

may be normal
HALLMARK = boot shaped

Question 31

What investigation can be used to assess the degree of stenosis in Tetralogy of Fallot?

Answer 31

ECHO

Question 32

Treatment of Tetralogy of Fallot

Answer 32

Surgery at <1 year old
Closure of VSD and correction of pulmonary stenosis

Question 33

Without surgery, what is the mortality of TOF?

Answer 33

95% by age 20 without intervention