Background and aim: On the basis of previously published
accounts, coupled with our own experience, we have assessed the surgical
approaches to patients with isomeric atrial appendages.
Methods: We reviewed pertinent published studies on surgical
treatment of individuals with isomeric atrial appendages, with the
pertinent surgical details provided by most of the manuscripts.
Results: Half of patients with right isomerism, and two-thirds
of those with left isomerism have bilateral superior caval veins. Azygos
extension of the inferior caval vein is reported in three-quarters of
those with left isomerism. The coronary sinus is universally absent in
right isomerism, along with totally anomalous pulmonary venous
connection, and is absent in two-fifths of those with left isomerism..
Univentricular atrioventricular connections are expected in up to
three-quarters of those with right isomerism. Atrioventricular septal
defect is reported in up to four-fifths, more frequently in right
isomerism, with such patients typically having discordant
ventriculoatrial connections or double outlet right ventricle. Reported
mortalities extend to 85% for those with right, and 50% for those with
left isomerism. In right isomerism, mortality is up to 54% for
systemic-to-pulmonary arterial shunting, up to 75% for univentricular
repair, and up to 95% for repair of totally anomalous pulmonary venous
connection itself. No more than one-quarter had undergone Fontan
completion, with reported mortalities of 21%. Conclusion:
Early surgical results are satisfactory in patients with left isomerism,
but disappointing for those with right. Recent advances in cardiac and
liver transplantation may offer improved survival.