The Superior Cavopulmonary Connection: History and Current Perspectives

Abstract

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.

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

“…The bidirectional cavopulmonary connection (BDCPC) has been utilized as an intermediate procedure prior to Fontan for patients with functionally single ventricle (SV) hearts since the 1980s [1]. The BDCPC is usually performed when a child is 3 to 6 months of age.…”

Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis

“…Patients who underwent Fontan surgery have gradually had improving survival since then. 12 However, the procedure would cause significant morbidity and mortality, especially in those with poor hemodynamics. 13,14 As Fontan operation creates a bottleneck in the circulation, upstream congestion and downstream decreased flow are inevitable consequences.…”

A Tribute to the Pioneers of Right Heart Bypass: An Historical Review

“…In consideration of the remarkable results that our scientific community has achieved in the last five decades, we all need to acknowledge our pioneers, Carlo Carlon, Francis Fontan, Guillermo Kreutzer, for having explored and introduced such a remarkable type of circulation and many other luminary surgeons who had subsequently further developed the concept of Fontan/ Kreutzer circulation. Like the Fontan registry which has been created among the Australian/New Zealand cardiological and surgical community, 45,46 it is now mandatory to create international registries among other existing societies (ie, ECHSA, Congenital Heart Surgeons’ Society, World Society for Pediatric and Congenital Heart Surgery) including multicentric studies, 47 in order to follow such a growing population of patients for life and to be able to answer in more details to Fontan’s patients most common question: “How long I will continue to be normal?”…”