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Hypertensive pulmonary vascular disease in alpha-transposition of the great arteries
Insights
Pulmonary arterial plexiform lesions, a sign of severe pulmonary hypertension, appear in children with d-transposition of the great arteries after 12 months. These lesions were found in 50% of patients with an intact ventricular septum and a closed ductus arteriosus.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
Background:
- Classic complete transposition (d-transposition) of the great arteries is a congenital heart defect.
- Pulmonary arterial plexiform lesions indicate chronic high pulmonary arterial pressure.
Purpose of the Study:
- To investigate the occurrence of pulmonary arterial plexiform lesions in pediatric patients with d-transposition of the great arteries.
- To determine the relationship between these lesions and ventricular septal integrity and ductus arteriosus status.
Main Methods:
- Histopathological examination of pulmonary arteries in subjects with d-transposition.
- Correlation of lesion presence with age, ventricular septal status, and ductus arteriosus patency.
Main Results:
- Pulmonary arterial plexiform lesions were not observed before 12 months of age.
- In patients aged 12-30 months, 50% (6/12) with an intact ventricular septum and closed ductus arteriosus showed the lesion.
- Only 1 of 4 patients with an associated ventricular septal defect had the lesion.
Conclusions:
- The study confirms the presence of pulmonary arterial plexiform lesions in d-transposition patients, particularly after 12 months.
- The unexplained occurrence of these lesions in patients with an intact ventricular septum and closed ductus arteriosus warrants further investigation.
- These findings support previous observations regarding the association of plexiform lesions with severe pulmonary hypertension in this population.
Abstract:
In subjects with classic complete transposition (d-transposition) of the great arteries, the pulmonary arterial plexiform lesion, characteristic of chronic high levels of pulmonary arterial pressure, was observed in those with an intact ventricular septum as well as in those with a ventricular septal defect. The lesion was not observed before age 12 months. Among 16 patients aged 12 to 30 months, the plexiform lesion was observed in 6 of 12 patients (50 percent) with an intact ventricular septum and a closed ductus arteriosus and in 1 of 4 patinets with an associated ventricular septal defect. The basis of the plexiform lesion, which is considered a sign of chronic severe pulmonary hypertension, occurring in subjects with an intact ventricular septum and a closed ductus arteriosus is not explained. The phenomenon observed in this study confirms the earlier observations of others.