Key result
Congenital isolated LV apical hypoplasia can manifest as sudden cardiac death in young patients.
Why the study?
Congenital isolated left ventricular apical hypoplasia is a recently recognized clinical entity with limited case descriptions and uncertain prognosis.
Case Report (n=1)
No
May signal sudden-death risk in isolated LV apical hypoplasia; extends phenotypic spectrum and leaves risk stratification open.
Congenital isolated left ventricular apical hypoplasia has recently been recognised as a discrete clinical entity with well-defined diagnostic criteria on cardiac magnetic resonance imaging. This spectrum has been described in four previous cases, three of which presented with relatively mild symptoms and one with pulmonary oedema. All of these patients responded to standard medical management. We describe a sudden and fatal presentation of this anomaly in a previously well 19-year-old male, confirming the fact that this is not a benign condition but a spectrum with the potential for significant complications.
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Irving et al. (2008) conducted a case report in Congenital isolated left ventricular apical hypoplasia (n=1). Congenital isolated left ventricular apical hypoplasia was evaluated on Sudden and fatal presentation. Congenital isolated left ventricular apical hypoplasia caused a sudden and fatal presentation in a previously well 19-year-old male, confirming it can lead to significant complications.
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