Key result
Autopsy of a 13-month-old infant with hypertrophic cardiomyopathy and supraventricular tachycardia revealed myofiber disarray, nodoventricular fibers, and left bundle branch fibrosis.
Case Report (n=1)
This case report describes the pathologic findings of the cardiac conduction system in an infant with hypertrophic cardiomyopathy and SVT, suggesting that SVT may have utilized a concealed pathway or malformed AV junction leading to sudden death.
May inform sudden death mechanisms in pediatric HCM; leaves open need for systematic conduction studies in larger cohorts.
The cardiac conduction system from infants suddenly dying with hypertrophic cardiomyopathy has not been described. We studied by serial section examination the conduction system from a 13-month old infant also known to have had supraventricular tachycardia. At autopsy, there was cardiomegaly with asymmetric septal hypertrophy. Microscopic examination revealed myofiber disarray around an abnormally formed central fibrous body, numerous nodoventricular fibers to both sides of the ventricular septum, and fibrosis of the left bundle branch. We postulate that supraventricular tachycardia utilized a concealed pathway or the malformed AV junction. Death may have resulted from deranged hemodynamics secondary to supraventricular tachycardia.
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Kanter et al. (1997) conducted a case report in Hypertrophic cardiomyopathy and supraventricular tachycardia (n=1). Hypertrophic cardiomyopathy and supraventricular tachycardia was evaluated on Pathologic findings of the cardiac conduction system. Autopsy of a 13-month-old infant with hypertrophic cardiomyopathy and supraventricular tachycardia revealed myofiber disarray, nodoventricular fibers, and left bundle branch fibrosis.
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