Andersons pediatric cardiology 1837

71 Fontan Pathway From Birth Through Early Childhood James S Tweddell, Ronald A Bronicki, Joshua W Salvin, Maryam Y Naim, Christine M Riley, Gil Wernovsky Abstract Functionally univentricular hearts, sometimes referred to as single ventricles, are a broad group of congenital heart defects in which division into separate pulmonary and systemic pumping chambers is not possible For individuals with a functionally univentricular heart, the ultimate goal is the Fontan circulation, in which the caval veins are directly connected to the pulmonary arteries, restoring in-series circulation and relieving cyanosis Treatment efforts from birth are directed at creating the ideal Fontan candidate and include preserving systolic and diastolic ventricular function, minimizing atrioventricular valve regurgitation, eliminating any mechanical obstruction to pulmonary blood flow, minimizing pulmonary vascular resistance, and ensuring atrioventricular sequential rhythm In the current era, palliation is accomplished in three stages of surgical intervention: the neonatal period, the superior cavopulmonary connection (accomplished during infancy), and the completion Fontan procedure, generally completed by 4 years of age This chapter details the management of the patient along the Fontan pathway, including neonatal stabilization and selection of the initial surgical palliation, the challenges of the interstage period, the superior cavopulmonary connection and completion Fontan, including patient selection and postoperative management Ultimately, long-term survival of the individual with functionally univentricular heart is possible, but life expectancy falls short of normal and there is an ongoing lifelong risk of heart failure due to the absence of a pulmonary ventricle Ongoing challenges include increasing the durability of the Fontan circulation, optimizing outcome of heart transplantation, and developing durable mechanical circulatory support for the patient with a functional univentricular heart Keywords Functionally univentricular heart; Single ventricle; Fontan; Bidirectional Glenn shunt; Hemi-Fontan; Norwood procedure; Damus-Kaye-Stansel Good judgement comes from experience Experience comes from bad