Tetralogy of Fallot

Question 1

Associated disorder with TOF

Answer 1

Di George (CATCH22)
Poland Syndrome
Foetal ETOH syndrome
Downs Syndrome

Question 2

What are other causes of cyanotic heart disease

Answer 2

TOF
Tricuspid Atresia
Pulmonary Atresia
Transposition of great vessel
Ebstiens Anomaly
Eisenmenger’s
Severe Pulmonary stenosis

Question 3

Features of TOF

Answer 3

PROVe

Pulmonary stenosis (RVOT outflow obstruction)
RVH
Overriding aorta
VSD

Question 4

Clinical signs of TOF (not aucultation)

Answer 4

Thrusting apex
Left parasternal heave
Systolic thrill over VSD
Thrill over pulmonary area
Raised JVP
Sternotomy scar (from pulmonary valve replacement - required due to risk of pulmonary regurg following surgical management)
Lateral thoractomy scar - from blalock tousig shunt insertion (causes unequal pulses)
subclavicular scar
Raised JVP

Question 5

Other causes of thoracotomy scar

Answer 5

Aortic repair from coarctation
Pneumonectomy
Lobectomy

Question 6

Clinical presentation of TOF

Answer 6

Cyanosis
worsening cyanosis on crying/ feeding/ dehydration/ agigitation
failure to thrive
clubbing
exertional SOB

Question 7

How does squatting aid in cyanotic spells

Answer 7

Increased afterload and increases venous return to heart. This increase in left ventricular pressure mitigates the R -> L shunt by increasing left ventricular pressure. This subsequently measures blood on the right side will overcome the RVOT obstruction and go into pulmonary circulation.

Question 8

Complications of TOF repair

Answer 8

Pulmonary regurgitation (can lead to RVF + TR)
Arrhythmias (may need PPM)
Endocarditis
Polycythaemia
Paradoxical emboli

Question 9

Investigations of TOF

Answer 9

ECG (RAD/ RBBB/ Wide QRS (high incidence of death)
CXR - Boot shaped heart
Holter monitor - arrhythmias
TTE
Cardiac MRI - best for assessing RV dimensions/ function/ nature and severity of lesions)

Question 10

Management of TOF

Answer 10

go through PROVe

RVOT resections/ balloon valvuloplasty
VSD closure with decron patch

Management of complications such as pulmonary regurg - PV replacement/ percutaneous

Question 11

Is endocarditis prophylaxis required in dental surgery

Answer 11

No. no link between dental surgery and IE
Abx may be required to cover for IE in GI/GU surgery into an infected site

Question 12

Other thinks to consider in term of non pharma management of TOF

Answer 12

Genetic counselling as 15% have 22Q11 deletion (digeorge)

Question 13

What might you be mindful of with long term follow up of TOF

Answer 13

Yearly TTEs
Ensure no complications of repair
Ensure no functional TR
Any evidence of heart failure