The Superior Cavopulmonary Connection: History and Current Perspectives.

The development of the superior cavopulmonary connection is a rich illustration of international influences in congenital cardiac surgery. The bidirectional Glenn and hemi-Fontan procedures have improved survival as both definitive and staged functional single ventricle palliation. The optimal timing of the second-stage superior cavopulmonary procedures varies by center but for low- and intermediate-risk patients, this may be within three to six months after the Norwood procedure. The list of risk factors continues to grow but the most frequently cited factors include atrioventricular valve regurgitation, decreased ventricular function, need for reintervention, and failure to attain nutritional and growth goals. Ongoing prospective, multi-institutional studies, particularly those fostered internationally by the World Society for Pediatric and Congenital Heart Surgery and other associations, will hopefully provide further clarification of the complex management issues related to patients with functional single ventricle physiology.