Chapter 11 - Tetralogy of Fallot

Abstract

Tetralogy of Fallot is a complex cardiac malformation characterized by the presence of a multilevel right ventricular outflow tract obstruction, leading to right ventricular hypertrophy, and a malalignment ventricular septal defect with aortic override. The pulmonary valve is usually small and dysplastic, as is the pulmonary artery. In the tetralogy of Fallot with pulmonary atresia, which represents the extreme form of tetralogy of Fallot, the pulmonary valve is atretic. Tetralogy of Fallot with absent pulmonary valve is a rare variant of the disease, where only nonfunctional remnants of the pulmonary valve are present. The characteristic feature of this condition is a strong dilatation of the pulmonary artery and its branches, causing airway compression.