Background: Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with few published studies focusing on anatomy and outcome.
Objective: To assess the bronchovascular anatomy, lung volume and growth in treated and untreated patients with UAPA.
Materials and methods: Eighteen children with UAPA (isolated: n = 12; associated with congenital heart disease: n = 6) were retrospectively studied to assess the vascularization and lung segmentation and to appraise lung volume evolution in treated and untreated patients. Age at presentation: 1 day to 6 years; mean follow-up duration 13.6 years. Reperfusion of the affected pulmonary artery was attempted in 10 children (younger than 6 months: n = 7; older than 6 months: n = 3).
Results: Bronchovascular lung segmentation was complete in all cases. In children treated before 6 months of age, lung volume normalized in 3 and remained normal in 3, and hypoplasia progression was noted in 1. Hypoplasia persisted in children treated after 6 months of age. In untreated children, lung hypoplasia was unchanged in cases diagnosed after 7 months of age (n = 4) and progressive in cases diagnosed before 3 months (n = 4).
Conclusion: In UAPA, lung anatomy and volume are normal at birth. Revascularization of the affected pulmonary artery before 6 months of age seems to allow optimal lung growth and prevent postnatal lung hypoplasia and development of collaterals.