Double-outlet Right Ventricle with Subaortic Ventricular Septal Defect and Severe Pulmonary Valve Stenosis, but Only Minimal Cyanosis and No Murmur: A Paradox?

We report the case of a complex congenital heart disease that accounts for unusual clinical presentation: subtle cyanosis and absence of murmur in a pediatric patient with double-outlet right ventricle, subaortic ventricular septal defect and severe pulmonary stenosis. Echocardiography revealed a large aorto-pulmonary window supplying the pulmonary arteries at systemic pressure.