Format

Send to

Choose Destination
See comment in PubMed Commons below
Pediatr Radiol. 2011 Apr;41(4):459-68. doi: 10.1007/s00247-010-1877-2. Epub 2010 Nov 27.

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

Author information

  • 1Department of Medical Imaging, CHU Sainte-Justine Mother and Child University Hospital Center, 3175 Cote Ste-Catherine Road, Montreal, QC, Canada, H3T 1C5.

Abstract

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.

PMID:
21113587
DOI:
10.1007/s00247-010-1877-2
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Springer
    Loading ...
    Support Center