Kwiatkowska Joanna, Aleszewicz-Baranowska Janina, Ereciński Jan
Klinika Kardiologii Dzieciecej i Wad Wrodzonych Serca, Gdański Uniwersytet Medyczny, 80-211 Gdańsk.
Kardiol Pol. 2010 Jan;68(1):72-5.
Non-compaction of the left ventricle (NCLV) was categorised as unclassified cardiomyopathy by the World Health Organization in 1995. Over the last decade this condition has been identified as a distinct form of cardiomyopathy and a genetically heterogeneous disorder. Clinically, this may be coupled with the loss of contractility, arrhythmia, and thromboembolism. The prognosis in a symptomatic patient is generally poor, with progression to chronic heart failure and death, including sudden death. We report a case of a child with NCLV and coexisting hemodynamic significant ventricular septal defect.
左心室心肌致密化不全(NCLV)在1995年被世界卫生组织归类为未分类的心肌病。在过去十年中,这种病症已被确认为一种独特的心肌病形式和一种基因异质性疾病。临床上,这可能伴有收缩力丧失、心律失常和血栓栓塞。有症状患者的预后通常较差,会进展为慢性心力衰竭并导致死亡,包括猝死。我们报告一例患有左心室心肌致密化不全并伴有血流动力学显著的室间隔缺损的儿童病例。