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Pulmonary vascular disease in complete atrioventricular canal defect
The American Journal of Cardiology
|May 4, 1977
Summary
Pulmonary vascular disease progresses rapidly in infants with atrioventricular canal defects, often starting within the first year of life. Early surgical intervention by age 1 is crucial to prevent advanced disease.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
Background:
- Atrioventricular (A-V) canal defects are congenital heart conditions.
- Pulmonary vascular disease (PVD) is a significant complication in these children.
Purpose of the Study:
- To investigate the incidence and timing of PVD development in children with complete A-V canal defects.
- To compare PVD progression in children with and without trisomy-21.
Main Methods:
- Retrospective study of 67 children with complete A-V canal defects.
- Analysis of pulmonary vascular changes (intimal fibrosis, dilatation, plexiform lesions) based on age.
- Comparison with 40 children with large ventricular septal defects.
Main Results:
- Advanced PVD, including grade 3 and 4 changes, develops rapidly within the first year of life.
- PVD progression occurs even with hemodynamically less severe findings.
- Similar rapid PVD progression was observed in children with large ventricular septal defects.
- Trisomy-21 did not influence the speed of PVD progression.
Conclusions:
- Early surgical correction (by age 1 year) is recommended for A-V canal defects to prevent irreversible PVD.
- Aggressive management is needed due to the rapid onset and progression of PVD in this population.