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00925njm 22002777a 4500 dc Casas, Guillem author Limeres, Javier author Oristrell, Gerard author Gutierrez-Garcia, Laura author Andreini, Daniele author Borregan, Mar author Larrañaga-Moreira, Jose M author Lopez-Sainz, Angela author Codina-Solà, Marta author Teixido-Tura, Gisela author Sorolla-Romero, José Antonio author Fernández-Álvarez, Paula author González-Carrillo, Josefa author Guala, Andrea author La Mura, Lucia author Soler-Fernández, Rafaela author Sao Avilés, Augusto author Santos-Mateo, Juan José author Marsal, Josep Ramon author Ribera, Aida author de la Pompa, Jose Luis author Villacorta, Eduardo author Jiménez-Jáimez, Juan author Ripoll-Vera, Tomás author Bayes-Genis, Antoni author Garcia-Pinilla, José Manuel author Palomino-Doza, Julián author Tiron, Coloma author Pontone, Gianluca author Bogaert, Jan author Aquaro, Giovanni D author Gimeno-Blanes, Juan Ramon author Zorio, Esther author Garcia-Pavia, Pablo author Barriales-Villa, Roberto author Evangelista, Artur author Masci, Pier Giorgio author Ferreira-González, Ignacio author Rodríguez-Palomares, José F author 2021-08-17 Left ventricular noncompaction (LVNC) is a heterogeneous entity with uncertain prognosis. 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. 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. 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. 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. J Am Coll Cardiol. 2021 Aug 17;78(7):643-662 http://hdl.handle.net/20.500.12105/15364 34384546 10.1016/j.jacc.2021.06.016 1558-3597 Journal of the American College of Cardiology Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction.