双心室非压实性心肌病

Md Tariqul Islam Khan, Gobinda Kanti Paul, Ali Hossain, Md Shafiqul Islam Islam, Jahir Uddin Mohammed Sharif
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摘要

心肌不压实(NC)通常是孤立的;然而,有时其他先天性心脏异常可能伴随心肌不致密。左室非压实性(LVNC)是一种遗传性异常,其中心室壁被厚的心室小梁所取代,小梁间有深的凹陷,由一层薄的压实层结合在一起。最常见的受累部位是左心室,少数病例报告有右心室受累。孤立性右心室不压实(RVNC)是罕见的,但如果不及时治疗会危及生命。基因检测可以发现可能的基因突变。NC的早期诊断对疾病管理非常重要。同时处理相关的其他心脏病变将有助于改善非压实患者的症状和预后。我们在此报告一位60岁男性病患,因心肌病合并双心室不紧实导致心力衰竭。这个案例是作为一种学术兴趣提出的。中华医学杂志[j];6 (1): 44-47
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Cardiomyopathy Following Biventricular Noncompaction
Myocardial noncompaction (NC) is usually seen isolated; however, sometimes other congenital heart abnormalities may accompany the myocardial noncompaction. Left ventricular noncompaction (LVNC) is a genetic anomaly where the ventricular wall is replaced by thick ventricular trabeculations with deep intertrabecular recesses held together by a thin compacted layer. The most common site of involvement is the left ventricle, with right ventricular involvement being reported in a few cases. Isolated right ventricular noncompaction (RVNC) is rare yet life-threatening if left untreated. Genetic testing may identify possible mutation of gene. Early diagnosis of NC is very important for disease management. The management of associated other cardiac pathologies simultaneously will help improve the symptoms and prognosis in patients with noncompaction. Here we report a case of 60-year-old male patient presenting with heart failure due to cardiomyopathy with biventricular noncompaction. The case is being presented as an academic interest. Mugda Med Coll J. 2023; 6(1): 44-47
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