Ujjwal Chowdhury

and 8 more

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.

Lakshmi Sankhyan

and 7 more

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.