Congenital heart disease

Question 1

Investigations for congenital defects

Answer 1

Echo± bubble contrast is first line

CT+MR for anatomical/functional info

Question 2

Bicuspid aortic valve features

Answer 2

Work well at birth
Often develop aortic stenosis needing replacement
Intense exercise may accelerate complications so yearly echos

Question 3

ASD types

Answer 3

Ostium secundum (80%) - hole high in septum, asymptomatic until L->R shunt at 40-60yrs
Ostium primum - associated with AV valve abnormalities, present in childhood

Question 4

ASD presentation

Answer 4

Chest pain
Palpitations
Dyspnoea
Arrythmias
Inc migraine frequency

Question 5

ASD tests

Answer 5

ECG shows RBBB with LAD (primum) or RAD (secundum)

CXR shows small aortic knuckle, pulmonary plethora, atrial enlargement

Question 6

ASD complications

Answer 6

Left-to-right shunt reversal (Eisenmenger’s complex)

Paradoxical emboli

Question 7

ASD treatment

Answer 7

May close spontaneously
Primum closed in childhood
Secundum closed if symptomatic or RV overload
Trancatheter closure more common

Question 8

VSD causes

Answer 8

Congenital

Post-MI

Question 9

VSD presentation

Answer 9

Severe HF in infancy

Incidental later in life

Question 10

VSD signs

Answer 10

Harsh pansystolic murmur at left sternal edge with systolic thrill
Smaller holes give louder murmurs
Pulmonary HT signs

Question 11

VSD complications

Answer 11

AR
IE

Eisenmenger’s complex

HF from volume overload
Pulmonary HT

Question 12

VSD investigations

Answer 12

ECG normal, LAD, LVH or RVH
CXR large pulmonary arteries, cardiomegaly, pulmonary plethora
Cardiac catheter shows step up in RV O2 sats

Question 13

VSD management

Answer 13

Medical initially as may spontaneously close
Surgical closure if failed medical, symptomatic, shunt >3:1 or IE
Endovascular closure may be possible

Question 14

What is coarctation of the aorta

Answer 14

Congenital narrowing of descending aorta, usually just distal to left subclavian a. origin
More common in boys

Question 15

Coarctation of the aorta associations

Answer 15

Bicuspid aortic valve

Turner’s syndrome

Question 16

Coarctation of the aorta signs

Answer 16

Radiofemoral delay
Weak femoral pulse

Inc BP

Scapular bruit
Systolic murmur over left scapula

Cold feet

Question 17

Coarctation of the aorta complications

Answer 17

HF from high afterload
IE
Intracerebral haemorrhage

Question 18

Coarctation of the aorta investigations

Answer 18

CT/MRI aortogram

CXR may show rib notching (blood diverted down intercostal arteries)

Question 19

Coarctation of the aorta management

Answer 19

Surgery

Balloon dilatation ± stenting

Question 20

One-ventricle heart management

Answer 20

Fontan procedure so systemic circulation directly into PA
Gives signs and symptoms of RHF
Seek advice from specialist ACHD centres when managing

Question 21

Tetralogy of Fallot features

Answer 21

VSD
Pulmonary stenosis
RV hypertrophy
Aorta overrides VSD, accepting right heart blood
(ASD in some makes up pentad)

Question 22

Tetralogy of Fallot presentation

Answer 22

Most common cyanotic heart disorder
Severity depends on pulmonary stenosis
Toddlers squat to increase TPR
Exertional dyspnoea, clubbing + RV failure in repaired pts

Question 23

Tetralogy of Fallot investigations

Answer 23

ECG shows RV hypertrophy with RBBB
CXR normal or boot shaped heart
Echo shows anatomy + ˚ of stenosis
Cardiac CT+MR before surgery for planning

Question 24

Tetralogy of Fallot management

Answer 24

Surgery before 1 with VSD closure + pulmonary stenosis correction

Question 25

Tetralogy of Fallot prognosis

Answer 25

Without surgery 95% mortality before 20

After repair 85% survive to 35