Left ventricular noncompaction in children.
OBJECTIVE
Left ventricular noncompaction (LVNC) is an uncommon type of cardiomyopathy in children. We sought to determine the clinical presentations and outcomes of children diagnosed to have LVNC.


METHODS
The case records of children diagnosed to have LVNC between 1999 and 2007 were reviewed. The diagnosis was based on echocardiographic finding of a thick noncompacted myocardium layer characterized by a trabecular meshwork with deep endomyocardial spaces.


RESULTS
Ten patients (seven males) were diagnosed to have LVNC at a median age of 2 years (range, 7 days to 12 years). Seven patients had isolated LVNC while three had associated structural congenital heart diseases. The right ventricle was also involved in two patients. Clinical presentations included congestive heart failure in eight patients, asymptomatic heart murmur in one, and syncope with exercise intolerance in one. At presentation, cardiomegaly was found in nine patients, electrocardiographic abnormalities in nine, and impaired LV contraction in six. Eight patients received anti-heart failure medications. Three patients died at a median of 1.5 years after diagnosis, two died suddenly of unknown causes, and one of heart failure. The seven surviving patients were followed up for a median of 2 years (range, 2 months to 3 years). Three patients developed cardiac arrhythmias. The LV function improved in three patients and worsened in one on follow-up. None of the patients developed thromboembolic complications.


CONCLUSIONS
Left ventricular noncompaction in children is a heterogeneous condition. Long-term follow-up for development of progressive LV dysfunction and cardiac arrhythmias is indicated.
Koh
Carline
C
Department of Paediatrics and Adolescent Medicine, Division of Paediatric Cardiology, Grantham Hospital, The University of Hong Kong, Hong Kong, China.
Lee
Pui-Wah
PW
Yung
Tak-Cheung
TC
Lun
Kin-Shing
KS
Cheung
Yiu-Fai
YF
D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging* / Q000628:therapy; D006333:Heart Failure / Q000000981:diagnostic imaging* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D014463:Ultrasonography; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000628:therapy