Tetralogy of Fallot – Right Aortic Arch

TOF – Right aortic arch

The lifted up apex, cor en sabot – peasant’s boot shaped heart, due to the right ventricular hypertrophy is seen well in this chest X-ray PA view. The right sided aortic arch is seen intending the tracheal air column on the right side. There is mild cardiomegaly and right atrial enlargement as well in this adult person with tetralogy of Fallot and associated inferior wall myocardial infarction. The lung fields are oligemic due to the right ventricular outflow tract obstruction in Tetralogy of Fallot. Tetralogy of Fallot is the commonest cause of right aortic arch in an adult.

A pathological study of 10 cases of right aortic arch and tetralogy of Fallot was reported by Steffen et al. Mirror image branching was noted in 7, aberrant left subclavian in 2 and isolated left subclavian in one [1].

Friedberg described right aortic arch in tetralogy of Fallot in three successive generations. They could not confirm a typical mendelian inheritance pattern in this series. Still they suspected genetic factors in that familial occurrence of cases in three successive generations [2].

References

1. Steffen Craatz, Eberhard Künzel, Katharina Spanel-Borowski. Right-sided aortic arch and tetralogy of Fallot in humans–a morphological study of 10 cases. Cardiovasc Pathol. Jul-Aug 2003;12(4):226-32.
2. Friedberg DZ. Tetralogy of Fallot with right aortic arch in three successive generations. Am J Dis Child. 1974 Jun;127(6):877-8.