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Ann Thorac Surg 1998;66:681-682
© 1998 The Society of Thoracic Surgeons

Supplement

Total anomalous pulmonary venous connections and consideration of the fontan or one-ventricle repair

Robert M. Freedom, MDa, Aijaz Hashmi, MDa

a Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children and University of Toronto Faculty of Medicine, Toronto, Ontario, Canada

Address reprint requests to Dr Freedom, The Hospital for Sick Children, Rm 1503C, 555 University Ave, Toronto, ON, Canada, M5G 1X8

Presented at the Workshop on "One and One-Half Ventricle Repairs," Gubbio, Italy, Dec 6–7, 1996.

Abstract

There is now a considerable literature that babies with right atrial isomerism have a poor outcome. The reasons for this are complex and multifactorial, but may be related at least in part to intrinsically small and abnormal pulmonary veins. We reviewed a series of consecutive patients seen at a single institution and found that babies with right atrial isomerism, severe pulmonary outflow tract obstruction or atresia, and total anomalous obstructed pulmonary veins had a grim outlook, especially those requiring operation in the neonatal period. Others have reported a similarly concerning outcome.




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