[Congenital pulmonary vein stenosis as a rare cause of pulmonary hypertension].

We report on the history, the diagnostic, and the operative procedure in an infant with congenital stenosis of all pulmonary veins. First symptoms such as failure to thrive, tachydyspnea, tachycardia and hepatomegaly occurred in the eighth week of life. Electrocardiography, 2-dimensional echocardiography and radiography of the chest were unspecific. After recurrent episodes of pulmonary oedema cardiac catheterization was performed: bilaterally elevated pulmonary artery wedge pressure and a normal left atrial pressure proved pulmonary venous obstruction. Severe stenosis of all pulmonary veins was apparent cineangiographically only by selective injections into the right and left pulmonary artery branches in wedge position. The operation (excision of the stenotic area and reimplantation using autologous pericardium) was unsuccessful as in most cases described in the literature. Congenital stenosis of all pulmonary veins is a rapidly progressive malformation. Death occurs usually in the first year of life with and without operation.