Andersons pediatric cardiology 1801

SECTION 6 Functionally Univentricular Heart OUTLINE 68 Introduction The “Fontan Pathway” 69 Anatomic Considerations in the Functionally Univentricular Heart 70 Physiologic Principles to Maximize Outcome in Patients With a Functionally Univentricular Heart 71 Fontan Pathway From Birth Through Early Childhood 72 Interstage Management 73 Longer-Term Outcomes and Management for Patients With a Functionally Univentricular Heart 68 Introduction The “Fontan Pathway” Gil Wernovsky, James S Tweddell Keywords Fontan; Single Ventricle; Hypoplastic Left Heart Syndrome; Interstage; Congenital Heart Disease In the late 1960s, two surgical teams (one led by Franỗois Fontan in Bordeaux, France, the other by Guillermo Kreutzer in Sóo Paolo, Brazil)1,2 concurrently introduced what is perhaps the most disruptive surgical innovation in congenital cardiac surgerya procedure that simultaneously eliminated volume load and cyanosis among patients with a functionally univentricular heart These operations were initially applied to patients with right heart obstructive lesions (tricuspid atresia in particular) and were termed right heart bypass procedures The success of these pioneering operations has been followed by multiple surgical modifications of the original concept With a long-term palliative option now available for the patient with a functionally univentricular heart, additional ingenious and equally disruptive operations were created, including the Norwood and hybrid procedures, the superior cavopulmonary connection (the “bidirectional Glenn”), and others These newer procedures have continued to undergo modifications to better allow survival in infancy and minimize risk factors for the eventual modified Fontan (or perhaps more correctly FontanKreutzer) procedure In combination, these complex surgical procedures can be considered staged reconstruction Along with medical management and monitoring, they have come to be known colloquially as the Fontan pathway In this text and elsewhere, congenital cardiac anomalies are typically described morphologically, followed by a description of the physiology, surgical