This case highlights a rare and fatal presentation of left ventricular strangulation due to herniation through a congenital pericardial defect in a young child.
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Consider congenital pericardial defects in unexplained pediatric cardiac collapse; single case leaves open need for diagnostic strategies.
SYNOPSIS An apparently healthy 4 year old boy complained of sudden, persistent, severe back pain associated with vomiting and peripheral circulatory failure. The radiological findings were puzzling and he died, undiagnosed, 36 hours after the onset of symptoms. Necropsy revealed significant cardiac hypertrophy in addition to the findings described in the title. The mechanism of the strangulation and the advisability of prophylactic repair of partial pericardial defects are discussed.
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DADDS et al. (1972) studied this question.
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