Congenital Heart Disease with Anomalies of the Right Ventricular Outflow Tract

This chapter describes the pathology and surgical treatment of the cardiac diseases which among other parts of the heart also affect the right ventricular outflow tract. According to Richard Van Praagh, among the six distinct entities dealt with, three reveal a similar intracardiac anatomy; we consider them variants of tetralogy of Fallot (TOF): TOF/pulmonary stenosis (classical TOF), TOF/pulmonary atresia (PA), and TOF/absent pulmonary valve syndrome. In a continuous spectrum of ventricular overriding of the aorta, starting with TOF/PA on one end, passing classical TOF, double outlet right ventricle (DORV) could be considered being the other end of this anatomical spectrum of intracardiac TOF anatomy, making it number 4 TOF variant. In itself DORV may feature the whole spectrum of subpulmonary pathology or unobstructed anatomy seen in other forms of TOF. The fifth entity is pulmonary atresia with intact ventricular septum (PA/IVS) and critical neonatal pulmonary stenosis, associated with intact ventricular septum, is nowadays an interventional cardiology domain. Therefore, we do not have a separate surgical chapter. Neonatal failures of catheter intervention may go the surgical track for PA/IVS. The double-chambered right ventricle (DCRV), in simple functional terms a subpulmonary stenosis, deserves its own paragraph due to its distinct anatomical and clinical features. As for membranous subaortic stenosis, DCRV is supposed to be a congenital heart defect; however, its clinical course resembles an acquired disease.