Andersons pediatric cardiology 1145

43 Pulmonary Atresia With Intact Ventricular Septum Michael A Quail, Robert H Anderson, Diane E Spicer, Piers E.F Daubeney Abstract The right ventricle can be hypoplastic in various settings It can be small in the presence of deficient ventricular or atrioventricular septation, producing so-called left ventricular dominance The chamber can also be small and incomplete in the setting of univentricular atrioventricular connections such as double-inlet left ventricle or tricuspid atresia The ventricle can be hypoplastic when the ventricular septum is intact and there is critical stenosis of the pulmonary valve All of these entities are dealt with elsewhere in this book This chapter is concerned with pulmonary atresia in the setting of an intact ventricular septum (see Fig 43.1) and includes the situation in which the cavity of the right ventricle is dilated, as well as hypoplastic In two of the final encyclopedic reviews led by Freedom, attention was directed to the huge problems produced by lesions of the coronary arteries or tricuspid valve in this setting, making it one of the most lethal of current congenital cardiac malformations Keywords Pulmonary atresia The right ventricle (RV) can be hypoplastic in various settings It can be small in the presence of deficient ventricular or atrioventricular septation, producing socalled left ventricular dominance The chamber can also be small and incomplete in the setting of univentricular atrioventricular connections such as double-inlet left ventricle (LV) or tricuspid atresia The ventricle can be hypoplastic when the ventricular septum is intact and there is critical stenosis of the pulmonary valve All of these entities are dealt with elsewhere in this book This chapter is concerned with pulmonary atresia in the setting of an intact ventricular septum (Fig 43.1) and includes the situation in which the cavity of the RV is dilated, as well as hypoplastic In two of the final encyclopedic reviews led by Freedom,1,2 attention was directed to the huge problems produced by lesions of the coronary arteries or tricuspid valve in this setting, making it one of the most lethal of current congenital cardiac malformations It was not without reason that Freedom3 had previously commented, in one of his many perceptive reviews of this lesion, “How can something so small cause so much grief?”