Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transvenous balloon dilation.

Congenital stenosis of the pulmonary veins is a rare but frequently lethal congenital cardiac abnormality. Eight patients with this malformation were diagnosed, evaluated and treated. All eight patients had associated congenital cardiac defects. Two of the eight died, one of sepsis and one after operative pulmonary venoplasty. In three patients who underwent transvenous balloon catheter dilation of the stenosis the procedure provided immediate but transient relief of the stenosis. The prognosis for symptomatic infants with pulmonary vein stenosis is poor and its treatment an enigma.