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| Clinical and UCG Features of a Family with Left Ventricular Noncompaction |
| ZHU Wen-jin, WU Jin-e, LI Guo-liang,et al |
| Department of Cardiovascular Medicine, the First Affiliated Hospital of Xi'an Jiaotong University Health Science Center, Xi'an 710061, China |
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Abstract 【Objective】 To analyze the pedigree and ultrasonic cardiogram (UCG) of a family with left ventricular noncompaction (LVNC) to provide more evidence for the diagnosis and treatment of LVNC. 【Methods】A family of LVNC was investigated, clinical manifestations and UCG were analyzed, and the pedigree of the family was drawn. The clinical manifestations and UCG characteristics of the family were observed and recorded. 【Results】Of the 15 LVNC families of 3 generations, 6 had clinical manifestations or UCG changes of LVNC. In this family, the main manifestation of adult patients was left ventricular dysfunction, while the minor patients had no clinical symptoms. UCG showed typical LVNC changes: abnormal large trabeculae in left ventricular regional myocardium, deep recesses between trabeculae, non-dense layer/dense layer of left ventricular myocardium at the end of systole (N/C) >2 , ejection fraction < 50%. Through the analysis of the family pedigree, it was found that both men and women in the family were susceptible to the disease. One of the parents of the patient must be a patient. There were patients in every generation of the family, suggesting that LVNC was inherited by autosomal dominant inheritance in the family.【Conclusion】The inheritance mode of LVNC is autosomal dominant inheritance. The main clinical manifestations of LVNC in adults are left ventricular insufficiency, and there are no clinical symptoms in minors. UCG plays an important role in the diagnosis of LVNC and can detect typical changes of LVNC.
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Received: 23 January 2019
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2019, Vol. 36 
