Isolated right pulmonary artery discontinuity: An uncommon presentation and reperfusion complications. (March 2020)

• Record Type: Journal Article
• Authors: McAree, Daniel; Whiteside, Wendy; West, Caroline; Romano, Jennifer C.; Bates, Katherine E.
• Is Part Of: Progress in pediatric cardiology. Volume 56(2020)
• Publication Date: 2020-03
• Subjects: Isolated right pulmonary artery discontinuity -- Pulmonary artery discontinuity -- Absent pulmonary artery -- Spontaneous pneumothorax -- Pulmonary hypoplasia -- Pulmonary hypertension; Pediatric cardiology -- Periodicals; Cardiovascular Diseases -- Periodicals; Infant; Child; Cardiologie pédiatrique -- Périodiques; 618.9212005
• DOI: 10.1016/j.ppedcard.2019.101166
• Languages: English
• ISSNs: 1058-9813

Abstract

Abstract: Isolated unilateral pulmonary artery discontinuity is a rare diagnosis, estimated to occur in 1 in 200, 000 to 1 in 300, 000 individuals. The affected pulmonary artery is typically discontinuous from the main pulmonary artery, rather than entirely absent. It may present symptomatically in the neonatal period with respiratory distress and pulmonary hypertension, but oftentimes is diagnosed incidentally in adulthood. Isolated unilateral pulmonary artery discontinuity can lead to pulmonary hypoplasia of the affected lung, increased blood flow to the contralateral pulmonary artery, and high risk for pulmonary hypertension in the neonatal period. Only one case in the literature has reported a neonatal spontaneous pneumothorax in the setting of isolated right pulmonary artery discontinuity. We report a second case, which was also complicated by pulmonary hypoplasia of the ipsilateral lung and pulmonary hypertension. We also review the complex medical and surgical management in this case, given the lack of a consensus treatment for isolated right pulmonary artery discontinuity.