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Brain pathology in patients with congenital heart disease
Murad Alturkustani1, Linda Szymanski2
1King Abdulaziz University, Jeddah, Saudi Arabia.
Insights
Brain pathology in children with congenital heart disease (CHD) often involves diffuse gliosis, linked to neurodevelopmental delays. These changes are primarily due to cerebral hypoperfusion, highlighting the need for improved perfusion management in CHD patients.
Area of Science:
- Neuropathology
- Pediatric Cardiology
- Developmental Neuroscience
Background:
- Congenital heart disease (CHD) is linked to neurodevelopmental delays.
- Brain pathology, including white and gray matter lesions, is observed in CHD patients.
- Vascular etiologies are suspected for these brain lesions.
Purpose of the Study:
- To describe the specific pathological brain changes in pediatric patients with CHD.
- To compare neuropathological findings in CHD cases with control brains.
- To identify key histological features associated with brain injury in CHD.
Main Methods:
- Retrospective review of autopsy reports from 20 pediatric CHD cases.
- Histological evaluation using hematoxylin-eosin and special stains.
- Immunohistochemical analysis for glial fibrillary acidic protein (GFAP), amyloid precursor protein (APP), and HLA-DR, compared to controls.
Main Results:
- Diffuse gliosis was the most common finding (16/20 cases), often severe.
- Hypoperfusion-related changes were prevalent: 10 acute, 8 chronic.
- Focal white matter necrosis occurred in 4 cases, with evidence of emboli in 2.
- Amphophilic globules were present in 7 cases.
- Hemorrhagic lesions (subarachnoid, subdural, intraventricular) were noted in several cases.
Conclusions:
- Diffuse gliosis is a prominent neuropathological feature in pediatric congenital heart disease.
- Observed brain pathologies are largely consistent with cerebral hypoperfusion.
- Improved cerebral perfusion strategies are crucial for managing patients with CHD.
Introduction:
Brain pathology in patients with congenital heart disease (CHD) is associated with neuro-developmental delay. Imaging studies support vascular etiology for both white and gray matter lesions. In this retrospective study, we described the pathological changes in the brains of patients with CHD.
Material And Methods:
Last twenty autopsy cases in pediatric patients with CHD at our institution were retrieved and autopsy reports were reviewed. Available hematoxylin-eosin, special, and immunostains were evaluated, and at least one section from each case was stained with anti-glial fibrillary acidic protein (GFAP), anti-amyloid precursor protein (APP), and anti-HLA-DR antibody. Staining pattern of these immunostains was compared to staining pattern in five control cases. Control cases comprised of 2 cases with no significant pathological changes, and 3 cases with telencephalic leukoencephalopathy. The following histological features were assessed: necrotic cells in cortex, hippocampus, and cerebellum, APP and GFAP staining pattern, and the presence of focal lesions and amphophilic globules. Twenty patients (10 males, 10 females) were identified, with age range between 2 weeks and 19 years.
Results:
The pathological findings were as follows: 10 cases had changes consistent with acute global hypoperfusion, 8 cases showed features consistent with chronic global hypoperfusion, 4 cases presented focal white matter necrosis (2 with intra-vascular emboli), and 16 cases showed diffuse moderate to severe gliosis, including 7 cases with amphophilic globules. Subarachnoid hemorrhages were present in 5 cases, subdural hemorrhage in 4 cases, intra-ventricular hemorrhage in 2 cases, and germinal matrix hemorrhage in 1 case.
Conclusions:
In conclusion, diffuse gliosis is the prominent pathological feature in CHD cases. Most of the pathological changes are known to occur in cerebral hypoperfusion regardless of primary cause. Better techniques to improve cerebral perfusion are warranted in the management of these patients.
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