Demographics
The age of patients with right isomerism at initial diagnosis, as
assessed from the literature, ranges from birth to 22.3 years, with a
median of 7.7 months. Among survivors, approximately nine-tenths
presented in the neonatal period, having been recognized at birth in
almost two-thirds. Half of these patients required prostaglandin
infusion at presentation to maintain oxygen
saturation.14-19,22 For those with left isomerism, age
at initial diagnosis ranged from birth to 35 years, with a median of 2
days. Of these two-thirds were females, whereas only half of those with
right isomerism were female. Up to one-sixth of reported patients with
left isomerism have extracardiac lesions but normal hearts. We have
excluded these individuals from further study. It remains the case,
nonetheless, that approximately one-fifth of reported patients with left
isomerism died shortly after birth, with about half not having surgery
and dying during infancy.16,17,20