Purpose Patients with left ventricular non-compaction cardiomyopathy (LVNC) and end-stage heart failure (HF) are transplanted less often than dilated cardiomyopathy (DCM) patients. We aimed to describe a cohort of LVNC patients and to identify risk factors associated with death or heart transplant (HTX). Methods We reviewed all patients < 18 years included in our HF database (2001 - 2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC) and at least mildly reduced ejection fraction (EF). Patients with DCM were included as controls for comparison. Descriptive statistics, multivariate analysis, and time-to-event analysis were used. Results We included 239 patients, 37 (15%) with I-LVNC, 38 (16%) with D-LVNC, and 164 (69%) with DCM. The overall median age at diagnosis was 4.8 years (IQR 1.1 - 10.1) with a median follow-up of 1.2 (IQR 0.2-4.1) years. I-LVNC patients presented less often with HF than D-LVNC and DCM patients (24%, 45%, 76%; p=0.0001). As expected, baseline LV dimensions were smaller in I-LVNC than in the D-LVNC and DCM groups (median z-scores 0.8, 3.84, 3.76; p < 0.0001). I-LVNC patients had higher baseline EF (median EF 47%, 30%, 22%; p<0.0001), however 64% of these patients did develop moderate to severe LV dysfunction during follow-up. I-LVNC patients had less admissions to ICU (46%, 61%, 70%, p =0.0005), 1 patient required ECMO and none underwent VAD implant (0%, 11%, 18%; p = 0.01). Significantly less patients from the I-LVNC group underwent HTX, as compared to their D-LVNC and DCM counterparts (11%, 21%, 33%; p = 0.02). This resulted in a significantly improved HTX-free survival for the I-LVNC group (p<0.0001) - Figure 1. On multivariable analysis, baseline EF and LV dimension, ACE inhibitor therapy and ICU admission during follow-up were significantly associated with death or HTX -Table 1. Conclusion In this cohort of LVNC and DCM patients, LV dilation with dysfunction was associated with death or HTX. Further studies on the outcomes of LVNC subtypes are warranted.