Congenital Heart Diseases

Question 1

How does a bicuspid aortic valve differ from normal?

Answer 1

two cusps instead of three

Question 2

What happens as result of bicuspid aortic valve?

Answer 2

turbulent blood flow which results in the valve thickening and stiffening (is a common cause of aortic stenosis)

Question 3

What is an Atrial Septal Defect caused by and thus what’s the effect?

Answer 3

Patent foramen ovale in the septum –> higher pressure in the LHS than RHS –> L to R shunting of the blood –> RH deals with more blood than its meant to –> RH dilates

Question 4

Two treatment options for Atrial Septal Defect?

Answer 4

1. Percutaneous closure

2. Surgical closure

Question 5

4 classical characteristics of a Tetralogy of Fallot?

Answer 5

1. ventricular septal defect
2. overriding aorta (takes blood in from both L outflow and some deoxygenated from RV)
3. Pulmonary stenosis
4. Right ventricular hypertrophy (Caused by the pulmonary stenosis)

Question 6

Clinical presentation of tetralogy of fallot?

Answer 6

Infants: cyanosis,
Children: have ‘tetralogy spells’ of cyanotic, tired & sleepy –> unresponsive, irritable
spells are relieved by SQUATTING

Question 7

What is coarctation of aorta?

Answer 7

narrowing of the descending aorta.

Can present in infancy or present later in life as hypertension with a murmur

Question 8

4 Treatment options of heart failure

Answer 8

1. Medical therapy (ACE inhibitors, beta blockers, diuretics)
2. Cardiac resynchronisation therapy (CRT)
3. Left ventricular assist device (mechanically takes blood out of LH and pumps it into aorta)
4. Transplant

Question 9

Which valvular dysfunction is commonly seen in patients POST-REPAIR of tetralogy of fallot?

Answer 9

pulmonary regurgitation: have to put a patch over where the (ventricular septal) valve is - sometimes this stretches the valve annulus and causes regurgitation

Question 10

Example of atrial arrhythmia (3)

Answer 10

Atrial fibrillation
Atrial flutter
supraventricular tachycardia

Question 11

Medication given for tachyarrythmias

Answer 11

beta blockers
digoxin
amiodarone

Question 12

Heavy weights are specifically advised against in which congenital heart defect

Answer 12

Aortopathy: Dilated aorta (can increase the risk of dissection)

Question 13

Sports involving breath holding are specifically advised against in which congenital heart defect

Answer 13

Fontan circulation (reliant on breathing)

Question 14

Risks of maternal death in ACHD

Answer 14

Severe obstructive lesions (17% maternal mortality)

Severe pulmonary hypertension (50% mortality)

Question 15

Associations with bicuspid aortic valve

Answer 15

aortic dilatation

coarctation

Question 16

Treatment for bicuspid aortic valve

Answer 16

same as degenerative artic valve disease

AVR +/- aortic surgery

Question 17

when would closure of an atrial septal defect be indicated?

Answer 17

if the right heart is dilated (and they are becoming breathless on exertion)

Question 18

when would closure of ventricular septal defect by indicated?

Answer 18

if evidence of decompensation (dilated ventricles)

SAME two closure options as atrial (percutaneous or surgical)

Question 19

Repair of tetralogy of fallot

Answer 19

patch the ventricularseptal defect (this can stretch the valve annulus –> pulmonary regurgitation)
widen the R ventricular outflow tract

Question 20

adults who have tolerated coarctation of the aorta may present with what?

Answer 20

high BP

a murmur

Question 21

2 ways to repair coarctation of aorta?

Answer 21

stenting (but may require further stenting later in life)

surgical repair

Question 22

why do we follow up these defects?

Answer 22

surgery is repair not cure
some defects are progressive…
LT complications: HF, valvular dysfunction, arrythmias

Question 23

what does a melody valve replace?

Answer 23

the pulmonary

Question 24

2 types of ventricular arrythmia

Answer 24

ventricular tachycardia

ventricular fibrillation

Question 25

how do valvular dysfunction lead to arrythmias

Answer 25

atrial dilatation
ventricular dilatation
scar tissue following surgery

Question 26

treatment for bradyarrhythias

Answer 26

pacemaker

Question 27

what contraceptions are a problem for Fontan circulation?

Answer 27

IUCD as intense vasovagal (drop in HR or BP)

oestrogen-containing preps could be thrombogenic