AIMS: The clinical features, prognosis, and even definition of left ventricular 
non-compaction (LVNC) are still the subject of much debate. The aim of this 
registry was to describe the clinical, echocardiographic, and prognostic 
features of LVNC in France. The main endpoint was to assess clinical and 
echocardiographic predictors of adverse outcome, defined as death or heart 
transplantation.
METHODS AND RESULTS: Between 2004 and 2006, 154 suspected cases of LNVC were 
identified from a nationwide survey in France. The diagnosis of LVNC was 
confirmed in 105 cases by echocardiographic evaluation in a core laboratory. 
Clinical and echocardiographic data for the 105 cases of LVNC are presented. 
Left ventricular non-compaction was first detected from heart failure symptoms 
in 45 patients, rhythm disorders in 12, and familial screening in 8. Left 
ventricular ejection fraction (LVEF) was < 30% in 46% of patients, but ≥ 50% in 
16%. The latter had less symptoms of severe heart failure (11 vs. 54%, P = 
0.001), but similar extension of the NC zone. During 2.33 ± 1.47 years of 
follow-up, several complications occurred, including severe heart failure in 33 
patients, transplantation in 9, ventricular arrhythmia in 7, embolic events in 
9, and death in 12. Factors associated with death or heart transplantation were 
NYHA 3 or 4 (HR = 6.69; P = 0.0007), high LV filling pressures (HR = 7.59; P = 
0.001), LVEF (HR = 0.93; P = 0.006), and hospitalization for heart failure (HR = 
13.55; P < 0.0001).
CONCLUSION: In this large reported series of LVNC, we observed that: (i) Left 
ventricular non-compaction was detected by familial screening in asymptomatic 
patients in 8% of cases. (ii) Left ventricular non-compaction was frequently 
over-diagnosed by echocardiography. (iii) Patients identified as LVNC presented 
with a high risk of severe complications, transplantation or death and needed 
close follow-up.