Non-compaction cardiomyopathy in a 5-generation Chinese family

• Published in: Zhōnghuá xīnxuèguănbìng zázhì Vol. 40; no. 4; p. 323
• Main Authors: Ding, Zhong-ru, Huang, Guo-ming, Wang, Hong-ru, Tu, Xiao-wen, Liu, Chuan-yin
• Format: Journal Article
• Language: Chinese
• Published: China 01.04.2012
• Subjects: Adolescent; Adult; Aged; Asian Continental Ancestry Group - genetics; Cardiomyopathies - epidemiology; Cardiomyopathies - genetics; Child; Child, Preschool; Death, Sudden, Cardiac; Female; Humans; Infant; Male; Middle Aged; Mutation; Pedigree; Ventricular Dysfunction, Left; Young Adult
• ISSN: 0253-3758
• DOI: 10.3760/cma.j.issn.0253-3758.2012.04.013

Familial left ventricular noncompaction(LVNC) is quite rare. We screened for the presence of LVNC and related clinical characteristics in a 5-generation Chinese family. Comprehensive medical history was obtained from 40 members in a 5-generation Chinese family. Systemic clinical investigations including echocardiography (UCG), routine and ambulatory electrocardiogram (ECG), X-rays were performed in 33 family members. Cardiovascular magnetic resonance image (MRI) was carried out in 2 family members. Sudden cardiac death (including 1 occurred while following-up) was reported in 7 family members (17.5%, 7/40). LVNC was diagnosed in 10 out of the 33 family members (30.3%) and heart enlargement was evidenced in 3, heart failure in 2, complete left branch conductive block in 3, serious sick sinus syndrome (SSS) treated with permanent pacemaker implantation in 1 and paroxysmal supraventricular tachycardia treated with radiofrequency ablation procedure in 1 out of these 10 LVNC patients. Primary pedigree analysis rev