Journal
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
Volume 78, Issue 7, Pages 643-662Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.jacc.2021.06.016
Keywords
late gadolinium enhancement; left ventricular ejection fraction; major adverse cardiovascular events; noncompaction cardiomyopathy; physiologic hypertrabeculation; genotype
Categories
Funding
- Catalan Society of Cardiology (Barcelona, Spain)
- Foundation Marato TV3 (Barcelona, Spain) [218/C/2015]
- Fondo Europeo de Desarrollo Regional (Union Europea, Una forma de hacer Europa) (Madrid, Spain)
- Instituto de Salud Carlos III (La Fe Biobank) (Madrid, Spain) [PT17/0015/0043]
- Spanish Ministry of Science, Innovation and Universities (Madrid, Spain) [IJC2018-037349-I]
- Cardiopath PhD program (Naples, Italy)
- Spanish Ministry of Science, Innovation and Universities [PID2019-104776RB-I00, CB16/11/00399]
- CIBER Cardiovascular (Madrid, Spain) [CB16/11/00403, 16/11/00420]
- AdvanceCat 2014-2020 (Barcelona, Spain)
- GE Healthcare
- Bracco
- Boehringer Ingelheim
- HeartFlow
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This study confirmed the association of LVNC with an increased risk of heart failure and ventricular arrhythmias, with left ventricular ejection fraction being the variable most strongly associated with MACE. Late gadolinium enhancement (LGE) confers additional risk in patients without severe systolic dysfunction. A risk prediction model was developed and validated in order to guide management.
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.
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