Abstract
The morphological variations when one, or both, of the atrial chambers
is subdivided, are many and varied. We present a synthesis of 198
published investigations of this “family” of uncommon lesions. Almost
three-quarters of patients with divided atrial chambers present during
infancy with severe pulmonary hypertension and cardiac failure.
Associated cardiac and extra-cardiac defects are present in between half
and nine-tenths of cases. Acquired division of the left atrium has been
reported after the Fontan operation, orthotopic cardiac transplantation,
and complicated aortic valvar infective endocarditis. Surgery under
cardiopulmonary bypass remains the definitive treatment. Balloon
dilation may be considered in anatomically compatible variants in the
setting of cardiac failure and pregnancy as a bridge to definitive
treatment. Overall, mortality has been cited between nil to 29%.
Presentation during infancy, associated congenital anomalies, pulmonary
hypertension, and surgery in the previous era, have been the reported
causes of death. The operative survivors have long-term favourable
outcomes, with near normal cardiac dimensions and low risk of
recurrence. While asymptomatic patients with division of the right
atrium do not need treatment, surgical resection of the dividing
partition under cardiopulmonary bypass is recommended in symptomatic
patients with complex anatomy, the spinnaker malformation, or associated
cardiac anomalies. Balloon dilation may be considered in uncomplicated
patients with less obstructive lesions. Hybrid intervention and
endoscopic robotic correction also have been performed. We submit that
an increased appreciation of the anatomic background to division of the
atrial chambers will contribute to improved surgical management.