[Unilateral agenesis of the pulmonary artery. Experience with 4 cases]

Abstract

We present our experience with four cases of unilateral pulmonary agenesis, 3 of the right artery and the other one of the left. Two of the patients had pulmonary hypertension and one of them died at two years of age. All cases were diagnosed by using pulmonary angiography. Aortography demonstrated the absence of circulatory supply to the lung and a contralateral aortic arch to the absent pulmonary artery. We have performed a review of the literature with an emphasis on the diagnostic aspects of this condition, and discuss the considerations for possible surgery in symptomatic patients.