Cerar Andraž, Kšela Juš, Poglajen Gregor, Vrtovec Bojan, Kneževič Ivan
Department of Cardiology, Advanced Heart Failure and Transplantation Center,University Medical Center, Ljubljana, Slovenia.
Department of Cardiovascular Surgery, University Medical Center, Ljubljana, Slovenia.
Heart Surg Forum. 2016 Jun 24;19(3):E128-30. doi: 10.1532/hsf.1563.
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare hereditary cardiomyopathy characterized by the formation of an outer compacted and inner noncompacted layer of the myocardium. The latter is characterized by prominent trabeculations and deep intertrabecular recesses and is functionally inferior to the compacted myocardium. As there is no specific treatment for patients with LVNC who develop heart failure, the management of these patients is limited and many patients progress to advanced stages of the disease. For LVNC patients with advanced heart failure, the data regarding the use of mechanical circulatory support are scarce. We report a case of a 29-year-old patient with LVNC and advanced refractory heart failure, who was successfully bridged to heart transplantation using a long-term continuous-flow left ventricular assist device.
左心室致密化不全心肌病（LVNC）是一种罕见的遗传性心肌病，其特征是心肌形成外层致密层和内层非致密层。后者的特点是有明显的肌小梁和深陷的小梁间隐窝，其功能低于致密心肌。由于对于发生心力衰竭的LVNC患者没有特异性治疗方法，这些患者的治疗手段有限，许多患者会进展到疾病晚期。对于晚期心力衰竭的LVNC患者，关于使用机械循环支持的数据很少。我们报告一例29岁的LVNC合并晚期难治性心力衰竭患者，该患者使用长期连续流左心室辅助装置成功过渡到心脏移植。
