Left Ventricular Non-Compaction: A Cardiomyopathy With Acceptable Prognosis in Children.
BACKGROUND
Data on children with left ventricular non-compaction (LVNC) is sparse. The purpose of this study was to evaluate its clinical profiles in a population of Chinese children.


METHODS
From January 2010 to March 2016, consecutive Chinese children (aged <18 years) with LVNC diagnosed by cardiovascular magnetic resonance (CMR) were prospectively recruited at Fuwai Hospital.


RESULTS
A total of 41 Chinese children (male: 28%; mean age: 14±4years) were included in this study. Left ventricular non-compaction was not detected in 13 (32%) patients at initial echocardiographic evaluation. Congenital heart disease (CHD) was found in 11 (27%) patients. Four (10%) patients had Wolff-Parkinson-White (WPW) syndrome. Mean left ventricular ejection fraction (LVEF) was 41±15%. Late gadolinium enhancement (LGE) was detected in eight (20%) subjects. During a mean follow-up of 2.9 years, four (9%) patients died or received heart transplantation. These patients had lower systolic blood pressure (91±10 vs. 108±14mmHg; p=0.02), diastolic blood pressure (57±7 vs. 68±8mmHg; p=0.007) and LVEF (19±7 vs. 44±12%; p=0.002) than the survivors. In addition, advanced heart failure (100% vs. 16%; p=0.002) and LGE (50% vs. 5%; p=0.04) were detected more in these subjects.


CONCLUSIONS
Left ventricular non-compaction is easily overlooked at echocardiographic assessment. Congenital heart disease and WPW syndrome were relatively common in LVNC children. The prognosis of children with LVNC seemed to be better than previous studies reported, and its long-term prognosis needs to be further investigated.
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Tao
T
Tian
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Yankun
Y
Yang
Department of Hematology and Oncology, The Second People's Hospital of Hefei, Hefei, Anhui, China.
Lanlan
L
Zhou
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Fang
F
Luo
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Yuehua
Y
Li
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Peng
P
Fan
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China.
Xueqi
X
Dong
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China. Electronic address: 13671185993@139.com.
Yaxin
Y
Liu
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China. Electronic address: doctorcjg@163.com.
Jingang
J
Cui
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Mediacal College, Beijing 100037, China. Electronic address: zhouxianliang0326@hotmail.com.
Xianliang
X
Zhou
D000293:Adolescent; D009202:Cardiomyopathies / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*; D002423:Cause of Death; D002648:Child; D002681:China / Q000453:epidemiology; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D009017:Morbidity / Q000639:trends; D011379:Prognosis; D011446:Prospective Studies; D013318:Stroke Volume / Q000502:physiology*; D015996:Survival Rate / Q000639:trends; D016277:Ventricular Function, Left / Q000502:physiology*
Children; Chinese children; Clinical presentation; Left ventricular non-compaction; Prognosis