Congenital stenosis of individual pulmonary veins

The case is reported of an infant with congenital stenosis of each of the four individual pulmonary veins near the left atrium. The characteristic features were failure to thrive, increasing cyanosis and dyspnea, and death in congestive cardiac failure at 7 months of age. A precordial systolic murmur and an early systolic ejection click were heard; the sounds over the base of the heart were diminished in intensity. Roentgenograms of the thorax revealed a heart of normal size with fullness of the main pulmonary artery segment and increased pulmonary vascular markings that had the reticulated appearance associated with pulmonary venous engorgement. Electrocardiography showed right axis deviation, right atrial enlargement, and a pattern of right ventricular systolic overload. Cardiac catheterization demonstrated increased right atrial pressure and markedly elevated right ventricular and pulmonary arterial pressures. Angiocardiographic studies revealed a right-to-left shunt at atrial level, enlargement of the right ventricle, and enlarged and tortuous pulmonary arteries. In the late films the left atrium appeared normal in size, but the pulmonary veins were tortuous and there was evidence of constriction of the pulmonary veins at the veno-atrial junctions, associated with delay in the passage of opaque material from the pulmonary veins to the left atrium. It is suggested that the definitive diagnosis most likely is to be made by a selective angiocardiogram performed from the main pulmonary artery. It would appear feasible, at least in theory, to obtain complete surgical correction of this anomaly.