Fontan Procedure
Abstract The goal of staged reconstructive surgery for children with single-ventricle defects is ultimately to achieve a modified Fontan circulation. The modified Fontan circuit allows systemic venous blood from both…
Syndromes, Genetics, and Heritable Heart Disease
Abstract The field of human genetics has achieved dramatic growth since the publication of the second edition of this textbook. The capabilities of genetic testing technology to interrogate the genome…
Tetralogy of Fallot With and Without Pulmonary Atresia
Abstract This chapter provides an overview of the topic of tetralogy of Fallot with and without pulmonary atresia, covering areas from history and genetics to current management issues and methods,…
Palliative Procedures: Deciding on Clinical Pathway; One Versus Two Ventricles
Abstract: Biventricular repair requires two ventricles, each capable of supporting the systemic and pulmonary circulation. Septation has been interpreted as the pursuit of biventricular circulation rather than single-ventricle track. Although…
Double-Outlet Right Ventricle
Abstract Double-outlet right ventricle (DORV) describes a ventriculoarterial connection in which both great arteries associate primarily with the right ventricle in an array of possible arrangements that can produce physiologies…
Pulmonary Atresia With Intact Ventricular Septum
Abstract This chapter describes the disease spectrum of pulmonary atresia with intact ventricular septum (PA/IVS). It reviews the embryology, anatomy, physiology, treatment algorithms, and surgical techniques and outcomes. PA/IVS is…
Heterotaxy
Abstract Isomerism is a unique clinical entity in which there is duplication of one half of the body across the vertical axis, leading to a loss of laterality in the…
Pulmonary Valve Replacement: Indications and Options
Abstract This chapter focuses on the indications and treatment options of pulmonary valve replacement. Currently there are multiple options for pulmonary valve replacement. However, there is no “perfect” valve for…
Pulmonary Embolism
Abstract Pulmonary embolism (PE) is an uncommon but potentially fatal complication of venous thromboembolism (VTE). Congenital heart disease predisposes children to both VTE and, by association, PE. This is due…
Coronary Artery Anomalies
Abstract This chapter focuses on the medical and surgical management of coronary artery anomalies in the pediatric patient without other congenital heart defects. The vast majority of aberrant coronary arteries…
