Demographics
The age of reported patients at their initial diagnosis, and at
necropsy, ranged from 1 day to 84 years, with a median of 71.5 months,
and an interquartile range from 14 to 498 months. The great majority
were below 18 years of age.1-80 The male to female
ratio in patients with congenital heart diseases was equal, but males
predominated by 1.7 to 1 in reported isolated cases. There was no
identifiable regional or ethnic predominance. Patients with isomerism of
either the right or left atrial appendages had an increased prevalence
of the anomaly, at around 13%.4,11-14 As emphasized,
it is exceedingly rare to find the lesion in the absence of congenital
heart diseases or aortic arch anomalies.14-17