Isolated Unilateral Absence of the Pulmonary Artery

Abstract

A 10 mo. old boy is presented who had isolated unilateral absence of the right pulmonary artery. He suffered from hemoptysis and severe congestive heart failure. The patient underwent prosthetic anastomosis of the right to the main pulmonary artery. Although the hemodynamic response was favorable, his oxygenation did not improve due to diffuse pulmonary arteriovenous fistulae of the affected lung. The patient succumbed 3 mo. after operation due to massive uncontrollable hemoptysis from the right lung. Isolated unilateral absence of the pulmonary artery is a rare lesion. In a review of the world literature as of Nov. 1976, 47 cases (including this report) of the unilateral absence of the pulmonary artery were reported. Of these 25.5% had pulmonary hypertension and only 4 cases underwent successful repair of the lesion. Though repair of this defect can be carried out, the result may not be always gratifying. A lung biopsy before proceeding to the surgical repair of the lesion is suggested. If the affected lung shows arteriovenous abnormalities surgery is not recommended.