Radiography

On radiographs, the cardiac silhouette in patients with tetralogy of Fallot is normal in size; however,
right ventricular hypertrophy can elevate the left ventricle. Combined with a small or absent main
pulmonary artery segment, the heart can have the classic boot-shaped appearance (as seen in the
image below). Most children with tetralogy of Fallot do not have boot-shaped heart.
Radiograph of a boot-shaped heart in an infant with tetralogy of Fallot.
Typically, the appearance of the vascularity of the pulmonary artery is reduced, but it can also be
normal. Decreased pulmonary vascularity is frequently difficult for the general radiologist to
appreciate. Large collaterals may give the appearance of normal vascularity.
The enlarged aorta in children with a right-sided arch can cause airway compression that can be
identified on chest radiographs, as demonstrated in the images below. A right-sided arch is present in
25% of children with tetralogy of Fallot, and it can be identified by means of direct visualization, with
displacement of the trachea to the left or with increased opacity of the spinal pedicles on the
ipsilateral side of the aortic arch. The position of the aortic arch influences surgical planning because
Blalock-Taussig shunts are more easily placed on the contralateral side of the aortic arch. Modified
Blalock-Taussig shunts can be placed bilaterally.
Radiograph of an infant with tetralogy of Fallot and a right-sided aortic arch.
Radiograph of an infant with tetralogy of Fallot (same patient as in the previous image).
Note the anterior compression of the trachea by the large ascending aorta.
Degree of confidence
Confidence in conventional chest radiographic findings increases with the radiologist's reading
experience. The use of echocardiography has reduced the importance of chest radiography in the
initial diagnosis of congenital heart disease. Echocardiography should be used to confirm
radiographic findings that are suggestive of tetralogy of Fallot.
False positives/negatives
The boot-shaped heart is overlabeled in neonates, who normally have right ventricular hypertrophy. If
the chest radiograph shows lordosis, a normal heart may appear boot shaped.
A right-sided aortic arch in a child with congenital heart disease is most commonly associated with
tetralogy of Fallot. Children with a large right-sided aortic arch may have a double aortic arch or an
aberrant left subclavian artery without congenital heart disease. Other forms of cyanotic heart disease
that are associated with a right-sided aortic arch are usually hypervascular and associated with
cardiomegaly (eg, truncus arteriosus, transposition of the great arteries).
Although cyanosis and a right-sided aortic arch are associated with tetralogy of Fallot, the presence of
cardiomegaly and increased pulmonary vascularity make an admixture lesion the more likely
diagnosis. Transposition of the great vessels or truncus arteriosus is associated with increased
pulmonary vascularity, cardiomegaly, and cyanosis, as well as a right-sided arch. Approximately one
third of children with truncus arteriosus have a right-sided aortic arch. Only 5% of children with
transposition of the great vessels have a right-sided aortic arch, but this is a more common form of
congenital heart disease than truncus arteriosus.