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