Total anomalous pulmonary venous connections and consideration of the Fontan or one-ventricle repair.
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.
Freedom
R M
RM
Department of Paediatrics, The Hospital for Sick Children and University of Toronto Faculty of Medicine, Ontario, Canada.
Hashmi
A
A
D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000601:surgery; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D011667:Pulmonary Veins / Q000002:abnormalities*