Journal article
Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment
Pediatric cardiology, v 24(1), 73
01 Jan 2003
PMID: 12360396
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Three cases of unilateral right-sided pulmonary venous atresia were evaluated over an 18-year period. These bring the total number of cases to 25 in the literature. The clinical presentation of all these patients was similar and consisted of recurrent pulmonary infections, asthma-like symptoms, and exercise intolerance. The patients presented in 1982 (patient 1, a 12-year-old boy), 1994 (patient 2, a 9-year-old girl), and 1999 (patient 3, a 13-year-old boy). All patients were evaluated with a chest roentgenogram, and patients 1 and 2 had a ventilation and perfusion scan. Patients 1 and 3 also had cardiac catheterization and pulmonary angiography. Patient 2 had a magnetic resonance imaging study of the chest. Only patient 3 had wedge pulmonary angiography. Although a rare congenital defect, this diagnosis should be strongly suspected based on the typical clinical presentation and the preliminary studies, such as the chest roentgenogram and ventilation and perfusion scan. However, for definitive diagnosis, cardiac catheterization with wedge pulmonary angiography is necessary. Anastomosis of the atretic pulmonary veins to the left atrium is a theoretical consideration. However, this may not be feasible due to pulmonary venous anatomy or significant pulmonary dysfunction with pulmonary vascular changes. In these circumstances, we recommend performing pneumonectomy to remove the nidus for repeated bouts of pulmonary infections, to eliminate the left-to-right shunt, and to eliminate the dead space contributing to exercise intolerance.
Metrics
Details
- Title
- Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment
- Creators
- K K Pourmoghadam - St. Christopher's Hospital for ChildrenJ W Moore - St. Christopher's Hospital for ChildrenM Khan - St. Christopher's Hospital for ChildrenE M Geary - St. Christopher's Hospital for ChildrenN Madan - St. Christopher's Hospital for ChildrenB J Wolfson - St. Christopher's Hospital for ChildrenJ P de Chadarevian - St. Christopher's Hospital for ChildrenD S Holsclaw - St. Christopher's Hospital for ChildrenM L Jacobs - St. Christopher's Hospital for Children
- Publication Details
- Pediatric cardiology, v 24(1), 73
- Publisher
- Springer Nature
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000180337700018
- Scopus ID
- 2-s2.0-0038320975
- Other Identifier
- 991021838680004721
UN Sustainable Development Goals (SDGs)
This publication has contributed to the advancement of the following goals:
InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Cardiac & Cardiovascular Systems
- Pediatrics