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Tetralogy of Fallot (ToF) (Pathophysiology TOF (1) Overriding aorta …
Tetralogy
of Fallot (ToF)
Definition
Congenital structural disorder
of the heart with 4 distinct
structural defects
Epidemiology
Commonest cause of cyanotic
congenital heart disease
5% of congenital heart disease
Pathophysiology
1) Overriding aorta
Aorta overrides the ventricular septum
2) Subpulmonary stenosis
RV outflow tract obstruction
Reduced pulmonary perfusion
4) VSD
Often large
Get a R-L flow due to pulmonary
outflow obstruction leading to RV>LV pressure
3) RVH
Occurs as a result of RV tract defect
and backpressure into RV
Clinical
presentation
Cyanosis
(infancy)
Asymptomatic
(initially)
Diagnosis
Examination
Cardio
Clubbing (older children)
Murmur: loud ejection systolic LSE
Investigations
Bloods
FBC, U+E, LFT (baseline)
Imaging
CXR: small heart, boot-shaped (RVH) , right-sided aortic arch,
left sided concavity (absence of PA and outflow tract), reduced
pulmonary vascular markings
Bedside
Obs (sats, RR, HR, BP, temp)
ECG (normal initially, progressive RVH)
History
PMH
Growth and development
Known medical conditions
DH
Meds, allergies
POH
Antenatal: bloods, scans,, growth, complications
Perinatal: gestation, delivery, weight, complications
Postnatal: complications
SH
Living arragements
PC/HPC
Cyanosis
Management
Conservative
Information, advice, support
Refer to cardio/paeds ASAP
Intubation/ventilation if severe hypercyanosis
Surgical
Shunt
Indictation: temp measure for severe cyanosis <6m
MOA: surgical tube placement betw subclavian artery
and pulmonary artery or balloon dilatation of RV outflow
Corrective surgery
Indication: once 6m old
MOA: close VSD, relieve RV outflow obstruction
Medical
Analgesia
Indication: hyercyanotic spells
MOA: sedation, pain relief
A/B-agonist
Indication: hypercyanotic spells
E.g. propanolol
MOA: peripheral vasoconstriction
Complications
CVD
MI or stroke with phyercyanotic
spells (e.g. on exertion)
Hypercyanotic spells
Usually self limiting <15m
Followed by sleep usually
Screening
Antenatal screening programme
Foetal anomaly scan
Newborn examination
Murmur
