Original Investigation
Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction

https://doi.org/10.1016/j.jacc.2021.06.016Get rights and content
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Abstract

Background

Left ventricular noncompaction (LVNC) is a heterogeneous entity with uncertain prognosis.

Objectives

This study sought to develop and validate a prediction model of major adverse cardiovascular events (MACE) and to identify LVNC cases without events during long-term follow-up.

Methods

This is a retrospective longitudinal multicenter cohort study of consecutive patients fulfilling LVNC criteria by echocardiography or cardiovascular magnetic resonance. MACE were defined as heart failure (HF), ventricular arrhythmias (VAs), systemic embolisms, or all-cause mortality.

Results

A total of 585 patients were included (45 ± 20 years of age, 57% male). LV ejection fraction (LVEF) was 48% ± 17%, and 18% presented late gadolinium enhancement (LGE). After a median follow-up of 5.1 years, MACE occurred in 223 (38%) patients: HF in 110 (19%), VAs in 87 (15%), systemic embolisms in 18 (3%), and 34 (6%) died. LVEF was the main variable independently associated with MACE (P < 0.05). LGE was associated with HF and VAs in patients with LVEF >35% (P < 0.05). A prediction model of MACE was developed using Cox regression, composed by age, sex, electrocardiography, cardiovascular risk factors, LVEF, and family aggregation. C-index was 0.72 (95% confidence interval: 0.67-0.75) in the derivation cohort and 0.72 (95% confidence interval: 0.71-0.73) in an external validation cohort. Patients with no electrocardiogram abnormalities, LVEF ≥50%, no LGE, and negative family screening presented no MACE at follow-up.

Conclusions

LVNC is associated with an increased risk of heart failure and ventricular arrhythmias. LVEF is the variable most strongly associated with MACE; however, LGE confers additional risk in patients without severe systolic dysfunction. A risk prediction model is developed and validated to guide management.

Key Words

genotype
late gadolinium enhancement
left ventricular ejection fraction
major adverse cardiovascular events
noncompaction cardiomyopathy
physiologic hypertrabeculation

Abbreviations and Acronyms

CI
confidence interval
CMR
cardiovascular magnetic resonance
DCM
dilated cardiomyopathy
ECG
electrocardiogram
HF
heart failure
HR
hazard ratio
ICD
implantable cardioverter-defibrillator
LGE
late gadolinium enhancement
LV
left ventricular
LVEF
left ventricular ejection fraction
LVNC
left ventricular noncompaction
MACE
major adverse cardiovascular events
NCCM
noncompaction cardiomyopathy
SCD
sudden cardiac death
SE
systemic embolism
TTE
transthoracic echocardiography
VA
ventricular arrhythmia
VF
ventricular fibrillation
VT
ventricular tachycardia

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Javed Butler, MD, MPH, MBA, served as Guest Editor-in-Chief for this paper.

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