Long-Term Survival of Patients With Left Ventricular Noncompaction

Abstract

Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end-systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end-diastolic trough of trabeculation-to-epicardium (X):peak of trabeculation-to-epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end-diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow-up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02-1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17-4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21-3.68) were associated with all-cause mortality. Compared with the expected survival for age- and sex-matched US population, patients with LVNC had reduced overall survival (P<0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age- and sex-matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population.

Keywords: ejection fraction; mortality; noncompaction; prognosis; survival.

Figure 1. Derivation of the cohort.

EMR indicates electronic medical record; and MRI, magnetic resonance imaging.

Figure 2. Distribution of noncompaction segments; NC:C (noncompacted:compacted) ratios and X:Y ratios by echocardiography.

NC:C indicates noncompacted to compacted; and X:Y, trough of trabeculation‐to‐epicardium (X):peak of trabeculation‐to‐epicardium (Y).

Figure 3. Overall survival of patients with noncompaction, stratified by left ventricular ejection fraction and extent of left ventricular noncompaction.

P values for comparison between each group: LVEF >50%, apex only vs LVEF >50%, mid or basal P=0.06; LVEF >50%, apex only vs LVEF <50%, apex only P=0.001; LVEF >50%, apex only vs LVEF <50%, mid or basal P<0.001; LVEF >50%, mid or basal vs LVEF <50%, apex only P=0.20; LVEF >50%, mid or basal vs LVEF <50%, mid or basal P=0.01; LVEF <50%, apex only vs LVEF <50%, mid or basal P=0.18. EF indicates ejection fraction.

Figure 4. Comparison of overall mortality between left ventricular noncompaction and expected US age‐ and sex‐matched population rates.

Shaded region indicates 95% CIs. LVNC indicates left ventricular noncompaction.

Figure 5. Comparison of overall mortality between left ventricular noncompaction and expected US age‐ and sex‐matched population rates, stratified by left ventricular ejection fraction (A) and noncompaction extent (B).

Shaded regions indicate 95% CIs. EF indicates ejection fraction.

Figure 6. Summarizing illustration.

A, Imaging characteristics of patients with isolated apical vs midbasal noncompaction. In patients with isolated apical noncompaction the noncompacted layer (yellow arrows) is limited to the ventricular apex. In patients with midbasal noncompaction, the noncompacted myocardium extends up to or beyond the midventricular level, identified by presence of papillary muscles distinct from the noncompacted myocardium (*). B, Prognostic factors in adult patients with left ventricular noncompaction.