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Subendocardial fibrosis in left ventricular hypertrabeculation-cause or consequence?

J Ker¹, L Du Toit-Prinsloo, W F P Van Heerden

¹Department of Physiology, University of Pretoria, Pretoria, South Africa.

Clinical Medicine Insights. Cardiology

|February 24, 2011

Summary

Left ventricular hypertrabeculation, a form of cardiomyopathy, can lead to sudden infant death. Subendocardial fibrosis in these cases may be a hidden cause of fatal arrhythmias in infants.

Keywords:

fibrosis hypertrabeculation noncompaction sudden infant death syndrome

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Area of Science:

Cardiology
Pathology
Genetics

Background:

Left ventricular noncompaction is a genetic cardiomyopathy.
It is characterized by a thickened myocardium with prominent trabeculations and recesses.
This spectrum includes left ventricular hypertrabeculation.

Observation:

A case of left ventricular hypertrabeculation presented as sudden infant death syndrome.
Histological examination revealed prominent subendocardial fibrosis.

Findings:

Left ventricular hypertrabeculation is associated with interstitial fibrosis.
Subendocardial fibrosis may serve as arrhythmogenic foci.

Implications:

Left ventricular hypertrabeculation could be an unrecognized cause of sudden infant death.
Identifying this condition may prevent future infant deaths.