Morphogenesis Annotated References References 70 Physiologic Principles to Maximize Outcome in Patients With a Functionally Univentricular Heart Abstract Introduction Guiding Principles of Staged Reconstruction Strategies and Physiologic Goals to Obtain the Optimal Outcome of Staged Reconstruction Physiology Immediately After Birth Clarifications and Proposed Changes to Terminology Used in the Neonate, Infant, and Child With a Functionally Univentricular Heart Physiologic Effects of Staged Reconstruction in the Patient With a Functionally Univentricular Heart Undergoing Superior Cavopulmonary Connection and Subsequent Fontan Procedure Annotated References References 71 Fontan Pathway From Birth Through Early Childhood Abstract Introduction Neonatal Stabilization, Surgical Strategies, and Postoperative Management Second-Stage Palliation: The Superior Cavopulmonary Connection Third-Stage Reconstruction: The Modified Fontan Procedure Outcomes Along the Fontan Pathway Future Considerations Closing Thoughts Annotated References References 72 Interstage Management Abstract Introduction Rationale and Metrics for Interstage Monitoring Inpatient Preparation for Interstage Management Outpatient Management Progression Through Stage II Palliation “Interstage II”—Beyond the Superior Cavopulmonary Connection References 73 Longer-Term Outcomes and Management for Patients With a Functionally Univentricular Heart Abstract Evolution of the Fontan Procedure Late Outcome With a Fontan Circulation Late Predictors Consequences of the Fontan Circulation Impaired Exercise Capacity (see also Chapter 23) Arrhythmia (see also Chapter 22) Summary Hematologic and Immunologic Complications Fontan-Associated Liver Disease Neurocognitive and Psychosocial Function Management of the “Well” Fontan Cardiac Catheterization and Transcatheter Intervention Management of Fontan Failure Future Directions Annotated References References Section 7 Beyond the Heart 74 Systemic Circulation Abstract