Affiliation:
1. University of Iowa Carver College of Medicine Iowa City IA
2. Department of Pathology University of Iowa Iowa City IA
3. Department of Pediatrics University of Iowa Iowa City IA
4. Department of Pathology and Laboratory Medicine Children's Hospital of Philadelphia PA
Abstract
Background
As a result of medical and surgical advancements in the management of congenital heart disease (
CHD
), survival rates have improved substantially, which has allowed the focus of
CHD
management to shift toward neurodevelopmental outcomes. Previous studies of the neuropathology occurring in
CHD
focused on cases preceding 1995 and reported high rates of white matter injury and intracranial hemorrhage, but do not reflect improvements in management of
CHD
in the past 2 decades. The purpose of this study is therefore to characterize the neuropathological lesions identified in subjects dying from
CHD
in a more‐recent cohort from 2 institutions.
Methods and Results
We searched the autopsy archives at 2 major children's hospitals for patients with cyanotic congenital cardiac malformations who underwent autopsy. We identified 50 cases ranging in age from 20 gestational weeks to 46 years. Acquired neuropathological lesions were identified in 60% (30 of 50) of subjects upon postmortem examination. The most common lesions were intracranial hemorrhage, most commonly subarachnoid (12 of 50; 24%) or germinal matrix (10 of 50; 20%), hippocampal injuries (10 of 50; 20%), and diffuse white matter gliosis (8 of 50; 16%). Periventricular leukomalacia was rare (3 of 50). Twenty‐six subjects underwent repair or palliation of their lesions. Of the 50 subjects, 60% (30 of 50) had isolated
CHD
, whereas 24% (12 of 50) were diagnosed with chromosomal abnormalities (trisomy 13, 18, chromosomal deletions, and duplications) and 16% (8/50) had multiple congenital anomalies.
Conclusions
In the modern era of pediatric cardiology and cardiac surgery, intracranial hemorrhage and microscopic gray matter hypoxic‐ischemic lesions are the dominant neuropathological lesions identified in patients coming to autopsy. Rates of more severe focal lesions, particularly periventricular leukomalacia, have decreased compared with historical controls.
Publisher
Ovid Technologies (Wolters Kluwer Health)
Subject
Cardiology and Cardiovascular Medicine
Cited by
6 articles.
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