Bodian Malick, Jobe Modou, Lèye Mohamed, Ndiaye Mouhamadou Bamba, Kane Adama, Sarr Simon Antoine, Mbaye Alassane, Diao Maboury, Ba Fatimata Gatta, Ngaïde Aliou Alassane, Coly Sarah Mouna, Thiam Anna, Sarr Moustapha, Bâ Serigne Abdou
Department of Cardiology, Aristide Le Dantec Teaching Hospital, Dakar, Senegal.
Clin Med Insights Case Rep. 2013;6:9-13. doi: 10.4137/CCRep.S10466. Epub 2013 Jan 10.
Left ventricular noncompaction (LVNC) is classified as a genetic cardiomyopathy characterized by a progressive systolic dysfunction. It may occur alone or in association with congenital cardiac anomalies. The combination of left ventricular noncompaction with partial atrioventricular canal defect is rare and has not, to our knowledge, been described previously.
A 21-year-old male who traveled to our center from a neighboring country presented with signs of heart failure. Transthorarcic echocardiography showed prominent trabeculations in the left ventricle predominantly in the left ventricle involving the apical lateral and mid anterolateral segments associated with a partial atrioventricular canal defect. There was a biventricular systolic dysfunction. There was good response to medical treatment.
This case stresses the importance of maintaining a high degree of suspicion for this rare cardiomyopathy and the need to systematically look for other associated anomalies in order to institute proper short- and long-term managements.
左心室心肌致密化不全（LVNC）被归类为一种遗传性心肌病，其特征为进行性收缩功能障碍。它可单独发生，也可与先天性心脏异常相关联。左心室心肌致密化不全与部分房室通道缺损同时存在的情况较为罕见，据我们所知，此前尚未有过相关描述。
一名来自邻国的21岁男性前来我们中心就诊，表现出心力衰竭的症状。经胸超声心动图显示左心室有明显的肌小梁，主要累及左心室的心尖外侧和前外侧中段，并伴有部分房室通道缺损。存在双心室收缩功能障碍。药物治疗反应良好。
该病例强调了对这种罕见心肌病保持高度怀疑的重要性，以及为了进行适当的短期和长期管理而系统地寻找其他相关异常的必要性。
