[Pulmonary atresia with interventricular communication. Review of concepts]

The purpose of this paper is to clarify the definition and anatomic characteristics of pulmonary atresia with ventricular septal defect. This complex congenital cardiac malformation produces great controversy in the literature. We analyze some embryological aspects, the right ventricular outflow tract anatomy and pulmonary arterial pattern with the various patterns of collateral blood supply. A good understanding of these anatomic characteristics is of great importance in the hemodynamic and angiographic diagnostic procedures and in the planning of surgical correction. The differential diagnosis with other congenital heart diseases with single outlet from the heart is discussed.