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