Atypical Tetralogy of Fallot: A Noncyanotic Form with Increased Lung Vascularity

Abstract

Only recently has the association of pulmonic stenosis with increased pulmonary blood flow been recognized. Four symptomatic infant cases are described in which the clinical diagnosis was large interventricular septal defect. Hemodynamic study showed the presence of associated pulmonic stenosis. Features suggesting that there is an overriding aorta in these patients, and that they, therefore, form an atypical group within the tetralogy of Fallot, are presented. The diagnosis has been confirmed in one case at autopsy. Their differentiation from clinically similar malformations as well as the surgical problem they pose is discussed.