Andersons pediatric cardiology 1838

judgement ANONYMOUS Half of what I'm teaching you is wrong The problem is that I don't know what half it is ANONYMOUS Introduction Some of the most challenging forms of congenital heart disease (CHD) fall into the category of those with functionally one pumping chamber, colloquially known as “single ventricle” defects, although we prefer the term functionally univentricular heart (fUVH) A fUVH is defined as a heart in which division into separate pulmonary and systemic ventricular pumping chambers is not possible This includes malformations such as hypoplastic left heart syndrome, tricuspid atresia, double-inlet left ventricle, severe forms of pulmonary atresia and intact septum, unbalanced atrioventricular (AV) septal defects in which one of the ventricles is prohibitively hypoplastic or absent, as well as other malformations where two pumping chambers are present but division is not practical due to AV valve straddling or arrangement of the great vessels and remote location of a ventricular septal defect In the past 3 decades survival beyond the neonatal period has become a reality due to a broad range of innovations, including the use of prostaglandin to maintain ductal patency; improved diagnostic capabilities, particularly with echocardiography; innovations in surgery such as the Norwood procedure; improvements in postoperative management; improved postdischarge management; and the development of a standardized three-stage approach to completion of the Fontan procedure Favorable short- and long-term outcomes are now the expectation and survival into adulthood is anticipated With improved survival, the focus is now being directed at optimizing neurodevelopmental outcome, functional outcome, and quality of life among survivors of with fUVH anatomy The ultimate anatomic goal for the patient with a fUVH is a Fontan circulation in which the venous return through the superior and inferior caval veins is connected directly to the pulmonary arteries With this arrangement, blood circulates as closely as possible to the normal pattern A successful Fontan is more likely in individuals with normal pulmonary arteriolar resistance, no pulmonary artery stenoses, no obstruction to pulmonary venous return, good systemic ventricular function, and adequate function of the AV and semilunar valves and AV sequential rhythm The goal of surgical palliation of the infant with a fUVH is the creation of a good Fontan candidate Over time, based on the increasing knowledge of risk factors for the Fontan procedure, a number of surgical tenets have emerged (Box 71.1; see also Chapter 70) Box 71.1 Goals of Neonatal Surgical Reconstruction ■ Unobstructed systemic blood flow ■ Approximately limited pulmonary blood flow ■ Undistorted pulmonary arteries ■ Unobstructed pulmonary venous return ■ Unobstructed systemic venous return This chapter presents the combined experience of multiple centers in the United States (the Cincinnati Children's Hospital Medical Center, Texas Children's Hospital, Boston Children's Hospital, Children's Hospital of Philadelphia, and Children's National Medical Center) They are not meant to be all-inclusive, and strategies that are successful in one institution may not be as successful in others Multiple reports have shown considerable variability in practice; these are beyond the scope of this chapter.1–24 Where controversies exist, we attempt to describe areas of future research that may differentiate best practices