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An Unusual Case of a Bicuspid Aortic Valve and Non-Compaction of the Left Ventricle

並列摘要


Introduction: Myocardial noncompaction is thought to be a rare genetic disorder of myocardial morphogenesis. Case Report: We report the case of a 22-year-old male with left ventricular noncompaction, bicuspid aortic valve and ascending aortic dilation. We discuss the genetic basis of myocardial noncompaction and potential etiologies that manifest in hypertrabeculated myocardium in the presence or absence of extracardiac abnormalities. Conclusion: Myocardial noncompaction is a rare genetic disorder thought to arise from disruption of normal organogenesis during development. Many genetic abnormalities have been associated with myocardial noncompaction. Recent research prompts the question of whether various genetic mutations exert their effect via a common pathway which manifest in similar morphological findings.

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