SPONTANEOUS ACQUISITION OF DISCONTINUOUS PULMONARY-ARTERIES

Background. Discontinuous pulmonary arteries have been considered a ra re complication of systemic-to-pulmonary shunt operations. We report a series of children who spontaneously acquired pulmonary artery discon tinuity. Methods. All children from 1989 through 1995 with congenital pulmonary atresia were reviewed. Results. Pulmonary artery discontinui ty developed in 29% (15 patients), none related to shunt operation. In 6 of 15 patients, the neonatal angiogram showed a pattern that seemed to predict subsequent discontinuity; in 9 of 15, pulmonary arteriogra phy was normal at birth. Two clinical patterns were identified: an ear ly rapid acquisition of discontinuity within hours to days, and a dela yed, more subtle development that occurred over months. Eight of 15 ha ve died. Pathologic studies in 6 children showed ductal tissue extendi ng along and into the pulmonary artery wall as well as intimal hypertr ophic reaction and maladaptive remodeling. Conclusions. Children with congenital pulmonary atresia may experience spontaneous acquisition of pulmonary artery discontinuity. Ductal tissue is responsible for loca l pulmonary artery distortion and discontinuity; this may be exacerbat ed by previous prostaglandin E(1) administration. Clinical algorithms are suggested for patients with pulmonary atresia.