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Tetrology of Fallot
Tetrology of Fallot
Tetrology of Fallot
25-08-2015
History
Environmental factors
Maternal diabetes [threefold increased risk], Retinoic acids,
Maternal phenylketonuria (PKU), and Trimethadione
Syndromes and associations
DiGeorge/Velocardiofacial
syndrome, Down syndrome,
Alagille syndrome, Cat's-eye syndrome, recombinant
chromosome (or San Luis Valley) and Kabuki syndromes,
CHARGE and VATER/VACTERL associations, Poland
syndrome and Goldenhar’s syndrome(oculo-auriculo-
vertebral dysplasia).
Microdeletion on 22q11….seen in
15–35% of TOF patients;
45% of TOF + PA;
65% of those with TOF + APV.
Embryology
Aorta is displaced to the right over the VSD rather than the left
ventricle.
This results in blood flow from both ventricles into the aorta.
The degree of aortic override of the VSD can vary widely and is
one of the major factors used by some groups to differentiate
between TOF and DORV.
If one defines double outlet right ventricle as the presence
of aortic/mitral valve fibrous continuity, then the degree of
override is not relevant to diagnosis.
If, however, one defines double outlet right ventricle as a
condition with greater than 50 percent aortic override, then,
by definition, the degree of aortic override in TOF is limited.
Associated anomalies
Pathophysiology
Growth retardation
Mental retardation
Cerebral venous sinus thrombosis
Nasal speech
Brain abscess
Cereberal embolism
Infective endocarditis
Coagulopathy
Huperuricemia and Gout
Clinical features
Cyanosis, Clubbing
JVP is normal
Arterial pulses …normal in uncomplicated TOF
Wide pulse pressure (arterial diastolic runoff)….
aortopulmonary collaterals, palliative surgical shunt or
PDA
Accentuated precordial RV impulse
LV impulse will not be hyperactive (normal cardiac output)
S2 single& loud (anterior, dextroposed aorta)
S3/S4 are unusual
Aortic ejection click
Systolic murmur…crescendo-decrescendo @ LUSB.
The intensity of the murmur inversely parallels the degree
of pulmonic obstruction.
Diastolic murmurs are unusual..Rarely AR murmur
TOF with PA…..no harsh, obstructive precordial murmurs
A harsh diastolic murmur, with a harsh murmur of PS,
[harsh sawing, to-and-fro murmur ] ……TOF and APV
syndrome
Continuous murmurs….PDA, aortopulmonary collaterals,
may be best heard in the back.
ECG
CXR
Echocardiography
Pulm.
RCA Valve
RCC MPA
NCC
LCx
The anomalous LAD crossing the RVOT in TOF is identified when the transducer
is swept superiorly in the parasternal short -axis view .
This allows visualization of the anomalous LAD that is situated anterior to the
RVOT
Cardiac Catheterization
Pulmonary atresia
Aim of surgery
Residual VSD
Pulmonary regurgitation
Residual RVOT obstruction
RV wall motion abnormalities
RV dysfunction and RHF
Atrial tachycardia
Ventricular tachycardia (VT)
Conclusions