Paracelsus Medical Private University Salzburg, Salzburg, Austria.
Am J Cardiol. 2012 Jul 15;110(2):314-5. doi: 10.1016/j.amjcard.2012.02.070. Epub 2012 Apr 12.
Left ventricular hypertrabeculation/noncompaction is associated with various neuromuscular and other rare genetic disorders. In a 53-year-old man with a Turner mosaic karyotype, regression of left ventricular hypertrabeculation/noncompaction was documented by cardiac magnetic resonance imaging and echocardiography after 7 years. During that time, coronary 3-vessel disease and severe left ventricular dys function developed, necessitating coronary bypass surgery. Postoperatively, left ventricular systolic function recovered to an ejection fraction of 40%. The patient died suddenly 6 months postoperatively. In conclusion, the disappearance of left ventricular hypertrabeculation/noncompaction was most likely due to scar formation.
左心室心肌致密化不全与各种神经肌肉和其他罕见的遗传疾病有关。在一名 53 岁特纳镶嵌核型患者中，经过 7 年的心脏磁共振成像和超声心动图检查，发现左心室心肌致密化不全消退。在此期间，患者发生了冠状动脉 3 支血管疾病和严重的左心室功能障碍，需要进行冠状动脉旁路手术。术后，左心室收缩功能恢复到射血分数 40%。患者术后 6 个月突然死亡。总之，左心室心肌致密化不全的消失很可能是由于瘢痕形成。
