成年期诊断的孤立性左心室心肌致密化不全的临床和超声心动图特征：一项病例系列研究。
Clinical and Echocardiography Features of Diagnosed in Adulthood Isolated Left Ventricular Noncompaction: A Case Series Study.
作者信息
Huang Wen-Hung, Sung Kuo-Tzu, Tsai Jui-Peng, Lo Chi-In, Hsiao Chih-Chung, Kuo Jen-Yuan, Su Cheng-Huang, Chen Ming-Ren, Hung Chung-Lieh
机构信息
Department of Internal Medicine, Division of Cardiology, Mackay Memorial Hospital, Taipei, Taiwan.
Mackay Medicine Nursing and Management College, Taipei, Taiwan.
出版信息
J Med Ultrasound. 2018 Jan-Mar;26(1):37-41. doi: 10.4103/JMU.JMU_1_18. Epub 2018 Mar 28.
BACKGROUND
Left ventricular noncompaction cardiomyopathy (LVNC) is a primary genetic cardiomyopathy with morphologically unique characteristics, including loose "spongy" meshwork. Subjects carrying these disorders were typically presented with triad of heart failure, cardiac arrhythmias, and consequences of mural thrombi formation. The clinical and echocardiographic features regarding LVNC, however, are not widely known.
METHODS
A retrospective survey involving 11 patients who fulfilled echocardiographic criteria for LVNC defined by Jenni . was conducted at MacKay Memorial Hospital from January 2009 to March 2017. Parameters assessed by echocardiography and clinical data were further analyzed.
RESULTS
Significantly depressed left ventricular systolic function assessed by echocardiography was noticed in a majority of our adult study cases.
CONCLUSION
Considering the fatal complications LVNC may lead to, it is essential for clinical cardiologists to early identify suspicious individuals, and the establishment of definitive criteria and early treatment is essential.
背景
左心室心肌致密化不全（LVNC）是一种原发性遗传性心肌病，具有形态学上独特的特征，包括疏松的“海绵状”网状结构。患有这些疾病的患者通常表现为心力衰竭、心律失常三联征以及壁血栓形成的后果。然而，关于LVNC的临床和超声心动图特征并不广为人知。
方法
2009年1月至2017年3月在麦凯纪念医院对11例符合Jenni定义的LVNC超声心动图标准的患者进行了回顾性调查。对超声心动图评估的参数和临床数据进行了进一步分析。
结果
在我们大多数成年研究病例中，通过超声心动图评估发现左心室收缩功能明显降低。
结论
考虑到LVNC可能导致的致命并发症，临床心脏病专家早期识别可疑个体至关重要，建立明确的标准和早期治疗必不可少。
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