Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation

Abstract:

Background

Left ventricular non-compaction (LVNC) is associated with genetic and phenotypic variability that influences outcomes. We aimed to identify risk factors for death or heart transplant (HTX) in a paediatric LVNC cohort.

Methods

We reviewed patients < 18 years of age (2001 – 2018) with LVNC, either isolated (I-LVNC) or with dilated phenotype (D-LVNC) and at least mildly reduced ejection fraction (EF). Patients with dilated cardiomyopathy (DCM) were included as controls. Descriptive statistics, multivariate analysis, and time-to-event analysis were used.

Results

We included 188 patients, 34 (18%) with I-LVNC, 37 (20%) with D-LVNC, and 117 (62%) with DCM. Overall median age at diagnosis=1.08 years (IQR = 0.22 - 10.65) and median follow-up = 1.4 (IQR = 0.2 - 5.2) years. I-LVNC patients' median baseline LV ejection fraction (LVEF) was 47%, compared with D-LVNC 33%, and DCM 21% (p<0.0001). 62% of I-LVNC patients developed moderate to severe LV dysfunction during follow-up. The incidence of death or transplantation was 43.6% in the overall cohort. Freedom from death or transplantation at 10 years after diagnosis was 88.6% (95% CI, 76–100) for I-LVNC, 47% (95% CI, 29–65) for D-LVNC and 42.3% (95% CI, 33-52) for DCM. On multivariable analysis, baseline LVEF and LVEDD z-score were associated with death or transplantation. Patients with a baseline LVEDD z-score > 4 and moderate to severe LV dysfunction had a transplant free survival of 38%.

Conclusions

Baseline LV dilation and systolic dysfunction were independently associated with progression to death or HTX in LVNC patients.