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Pulmonary vascular disease in infants with complete atrioventricular septal defect
Insights
Pulmonary vascular disease is common in infants, especially those with Down syndrome, with severe cases occurring between 7-12 months. Early surgical intervention within 6 months is crucial for managing this condition.
Area of Science:
- Pediatric Pathology
- Cardiovascular Research
- Genetics and Disease
Background:
- Pulmonary vascular disease (PVD) is a significant concern in pediatric populations.
- Down syndrome is associated with an increased risk of cardiovascular abnormalities, including PVD.
- Histological assessment is crucial for grading the severity of PVD.
Purpose of the Study:
- To determine the incidence and timing of pulmonary vascular disease in pediatric patients.
- To correlate pulmonary vascular resistance with histological findings of PVD.
- To identify risk factors and optimal intervention times for PVD in children.
Main Methods:
- Histological examination of 49 lung specimens (34 autopsy, 15 biopsy).
- Patient cohort: 49 children (10 days to 14 years), 37 with Down syndrome.
- Correlation of pulmonary vascular resistance (PVR) from heart catheterization with PVD grade.
Main Results:
- Overall incidence of irreversible PVD (grade ≥3) under 1 year was 34.6%.
- Grade 4 PVD occurred exclusively in patients with Down syndrome.
- PVR > 7 units/m² strongly correlated with grade 4 PVD, particularly in infants under 1 year.
Conclusions:
- Significant PVD incidence occurs under 1 year, peaking between 7-12 months.
- High PVR (> 7 units/m²) is a reliable indicator of severe PVD (grade 4).
- Early surgical correction within 6 months is essential, especially for Down syndrome patients.
Abstract:
In order to assess incidence and time of occurrence of pulmonary vascular disease, the lungs of 49 patients were studied histologically. Thirty-four lung specimens were obtained at autopsy, and 15 were open lung biopsies. Patients consisted of 24 males and 25 females ranging in age from 10 days to 14 years (median 9 months); 37 had Down's syndrome. Overall incidence of irreversible pulmonary vascular disease (grade 3 or more) under 1 year of age was 34.6%; grade 4 of pulmonary vascular disease was observed only in Down patients. In correlating pulmonary vascular resistance, measured during heart catheterization, with pulmonary vascular disease severity at histology, 6 out of 7 patients with pulmonary vascular resistance over 7 units per metre squared showed grade 4 of pulmonary vascular disease, and of these 4 were under 1 year of age. These findings suggest that a significant rate of pulmonary vascular disease occurs under 1 year of age, with most severe degrees in the time interval 7-12 months. They show that good correlation exists between pulmonary vascular resistance over 7 units per metre squared and grade 4 of pulmonary vascular disease. They demonstrate that the most severe pulmonary vascular disease is seen in Down's syndrome. Finally, they indicate that early surgical correction is mandatory and should be accomplished within 6 months of age.