Long term outcomes in left ventricular noncompaction

Abstract

Type of funding sources: None. Left ventricular noncompaction (LVNC) is a heterogeneous entity with a wide phenotypic expression. Risk factors have not been well established and prognostic stratification remains challenging. Describe long term outcomes of LVNC patients and determine predictors of cardiovascular events.    Prospective multicentric study of consecutive patients fulfilling imaging diangostic criteria for LVNC (Jenni echo criteria and Petersen CMR criteria). Demographic, ECG, imaging and genetic variables were collected. End points were heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause death. Major adverse cardiovascular events (MACE) was the combination of the four previous end points. 585 patients from 12 referral centres were included from 2000 to 2018. Age at diagnosis was 45 ± 20 years, 334 (57%) were male, baseline LVEF was 48 ± 17% and 18% presented late gadolinium enhancement (LGE). During a median follow-up of 5.1 years (IQR 2.3-8.1), 110 (19%) patients presented HF, 87 (15%) VA, 18 (3%) SE and 34 (6%) died. MACE occurred in 223 (38%) patients. LVEF was independently associated with HF, VA, SE and MACE: HR were 1.08, 1.02, 1.04 and 1.02 respectively (all p < 0.05). LGE was more frequent in patients with reduced LVEF (39 Vs 53%, p < 0.001) and was associated with higher HF and VA risk in patients with LVEF > 35% (HR 2.69 and 2.48 respectively, p < 0.05) (Figure 1). Patients with a normal ECG, LVEF≥50%, no LGE and no family aggregation presented no MACE (0%) at long term follow-up. Among patients who underwent genetic testing (354, 61%), TTN variants and complex genotype (more than one variant) presented lower LVEF and higher HF risk. ACTC1 variants were associated with VA. LVNC carries a high long term risk of heart faliure and ventricular arrhythmias. LVEF is the most important predictor and myocardial fibrosis is associated with increased risk in patients without severe systolic dysfunction. Genotype is a modifier of outcomes. These factors might be used to risk stratify LVNC patients. Abstract Figure. Kaplan Meier survival curves