Isolated atresia of the left pulmonary veins

Four cases of congenital isolated atresia of the left pulmonary veins were observed over a 12-year period. The diagnosis was established through radionuclide pulmonary perfusion studies together with cardiac catheterization and pulmonary arteriography. Both procedures showed an elevated pulmonary pressure wedge and decreased blood flow with late poor filling of the pulmonary capillary bed on the affected side. Arteriography revealed non-visualization of the left pulmonary veins. The possibility of congenital atresia of the pulmonary veins should be borne in mind when confronted by unexplained hemoptysis, recurrent lower respiratory infections with protracted infiltrates, or pulmonary arterial hypertension. Surgical treatment, though difficult, is feasible.