Unilateral absence of pulmonary artery in children: bronchovascular anatomy, natural course and effect of treatment on lung growth

Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly with few published studies focusing on anatomy and outcome. To assess the bronchovascular anatomy, lung volume and growth in treated and untreated patients with UAPA. 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). 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). 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.