RESUMO
Topical congenital pulmonary vein stenosis is a uncommon defect, both isolated or associated to other cardiac abnormalities. Only the localization of the lesions seems to affect the survival, because 60 of survival cases has unilateral stenosis; the severity of associated cardiac lesions become the prognosis poor. We describe two cases: 1st case, a 43 days old boy presented with heart failure and convulsion and had a diagnosis of pulmonary hypertension, atrial septal defect and tricuspid regurgitation, without pulmonary abnormalities. He had recurrent pulmonary infections and a cerebral ischemia in the following months, and died at 15 months of age for sepsis. Autopsy revealed stenosis and atresia in all pulmonary veins, with venous and arterial hypertension. There was also aortic hypoplasia and aortic and tricuspid valves indifferentiation; 2nd case, a 7 days old girl had a diagnosis of aortic coarctation and atrial and ventricular septal defects. Surgical corrections, at 38 and 46 days old, firstly of the aortic coarctation and after for the septal defects, disclosed and relief a supra-valvar mitral stenosis, but she remained on heavy respiratory insufficiency. At 6 months old, she returned to the hospital with dyspnea and cianosis, heart failure and hemoptisis; a sepsis developed and she died. At autopsy, there were severe pulmonary vein stenosis on the left and in the superior right veins, with pulmonary hypertension and hemorrhage.