<h3>Purpose</h3> 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). <h3>Methods</h3> 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. <h3>Results</h3> 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. <h3>Conclusion</h3> 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.