For a number of years prior to the advent of widespread use of cardiac catheterization, the syndromes associated with pulmonary stenosis and right-to-left shunts through atrial and ventricular septal defects had been well described. In the past few years cardiac catheterization performed on a large number of patients has allowed recognition of the syndrome of pulmonary stenosis with left-to-right shunt. As expected from knowledge of the relative frequency of the various types of left-to-right shunts, the pulmonary stenosis is usually associated with atrial or ventricular septal defects or, less commonly, patent ductus arteriosus, anomalous pulmonary venous connection, or other rarer causes of left-to-right shunt.
Abrahams and Wood1 called attention to this syndrome in 1951 under the designation of pulmonary stenosis with normal aortic root. Since that time, one or two papers have appeared in the literature each year describing a small number of such cases. In 1952 Gøtzsche 2