Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study.
OBJECTIVE
Non-compaction of the left ventricular myocardium (NCVM) is reportedly exceedingly rare and associated with a high morbidity and mortality. A different genetic background has been suggested for NCVM with [non-isolated NCVM (ni-NCVM)] and without [isolated NCVM (i-NCVM)] other congenital heart defects. We prospectively evaluated both the NCVM subgroups regarding frequency of occurrence and cardiovascular complications in a paediatric population. Results In a prospective, single-centre study, 66/5220 consecutive patients (1.26%) were diagnosed (25 i-NCVM, 41 ni-NCVM). The median age was 4 years (range 0-21), the median follow-up 12 months (range 0-51). The occurrence of congestive heart failure (CHF) at follow-up was 68.0%. CHF was as frequently seen in i-NCVM and ni-NCVM patients (77.5 vs. 62.1%, P = 0.322). The occurrence of arrhythmias (20.0%) and thrombo-embolic events (13.9%) was not different between subgroups. The cardiomyopathy related mortality was 7.1%, with three and one deaths in the i-NCVM and ni-NCVM groups, respectively (P = 0.126).


CONCLUSIONS
When prospectively evaluated, NCVM appears to have been previously under-diagnosed. Whereas arrhythmias and thrombo-embolic events were rare, CHF was frequently found. An equally aggressive anticongestive treatment regimen would seem indicated for both the NCVM subgroups.
Lilje
Christian
C
Departments of Kinderkardiologie, Universitäts-Klinikum Hamburg-Eppendorf, Martinistr. 52, D-20246 Hamburg, Germany. lilje@europe.com
Rázek
Vit
V
Joyce
James J
JJ
Rau
Thomas
T
Finckh
Barbara F
BF
Weiss
Florian
F
Habermann
Christian R
CR
Rice
Janet C
JC
Weil
Jochen
J
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology* / Q000401:mortality; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011446:Prospective Studies; D013923:Thromboembolism / Q000209:etiology* / Q000401:mortality