Rare health conditions 49: sudden arrhythmic death syndrome (SADS), sudden infant death syndrome (SIDS), and Hansen's disease

The purpose of this series is to highlight a range of rare health conditions. Rare health conditions are those that affect no more and usually fewer than 1 person in every 2000. Many healthcare assistants and nurses will encounter some of these conditions, given the high number of them. This 49th article will explore three of these conditions: sudden arrhythmic death syndrome (SADS); sudden infant death syndrome (SIDS); and Hansen's disease.

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Sudden Infant Death Syndrome and Left Ventricular Hypertrabeculation-Hidden Arrhythmogenic Entity?

Clinical Medicine Insights Cardiology ◽

10.4137/cmc.s5933 ◽

2010 ◽

Vol 4 ◽

pp. CMC.S5933 ◽

Cited By ~ 1

Author(s):

J. Ker ◽

L. Du Toit-Prinsloo ◽

W.F.P. Van Heerden ◽

G. Saayman

Keyword(s):

Heart Failure ◽

Sudden Infant Death Syndrome ◽

Infant Death ◽

Sudden Infant Death ◽

Left Ventricular ◽

Sudden Infant ◽

Thromboembolic Events ◽

Left Ventricular Noncompaction ◽

Ventricular Noncompaction ◽

Arrhythmic Death

Left ventricular noncompaction/hypertrabeculation is a condition which is characterized by a highly trabeculated, “spongy” myocardium. It can present at any age with heart failure, arrhythmia and/or thromboembolic events. A wide variety of mutations have been found to be a cause of hypertrabeculation and it is possible that there is a continuum of hypertrophic cardiomyopathy, dilated cardiomyopathy and hypertrabeculation/noncompaction. We present a case of left ventricular hypertrabeculation which presented as sudden infant death syndrome and we propose that this entity may be a hidden cause of arrhythmic death in some infants presenting as sudden infant death syndrome.

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Subendocardial Fibrosis in Left Ventricular Hypertrabeculation-Cause or Consequence?

Clinical Medicine Insights Cardiology ◽

10.4137/cmc.s6507 ◽

2011 ◽

Vol 5 ◽

pp. CMC.S6507 ◽

Cited By ~ 5

Author(s):

J. Ker ◽

L. Du Toit-Prinsloo ◽

W.F.P. Van Heerden ◽

G. Saayman

Keyword(s):

Sudden Infant Death Syndrome ◽

Infant Death ◽

Interstitial Fibrosis ◽

Sudden Infant Death ◽

Left Ventricular ◽

Sudden Infant ◽

Genetic Origin ◽

Ventricular Noncompaction ◽

Arrhythmic Death ◽

Pathological Characteristics

Left ventricular noncompaction has been classified as a primary cardiomyopathy with a genetic origin. This condition is morphologically characterized by a thickened, two-layered myocardium with numerous prominent trabeculations and deep, intertrabecular recesses. Recently, it has become clear that these pathological characteristics extend across a continuum with left ventricular hypertrabeculation at one end of the spectrum. The histological findings include areas of interstitial fibrosis. We present a case of left ventricular hypertrabeculation which presented as sudden infant death syndrome. Histologically areas of subendocardial fibrosis was prominent and we propose that this entity may be a hidden cause of arrhythmic death in some infants presenting as sudden infant death syndrome., with areas of subendocardial fibrosis as possible arrhythmogenic foci.

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