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Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction

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Guillem Casas, Javier Limeres, Gerard Oristrell, Laura Gutierrez-Garcia, Daniele Andreini, Mar Borregan, Jose M. Larrañaga-Moreira, Angela Lopez-Sainz, Marta Codina-Solà, Gisela Teixido-Tura, José Antonio Sorolla-Romero, Paula Fernández-Álvarez, Josefa González-Carrillo, Andrea Guala, Lucia La Mura, Rafaela Soler-Fernández, Augusto Sao Avilés, Juan José Santos-Mateo, Josep Ramon Marsal, Aida Ribera & 19 more José Luis de la Pompa, Eduardo Villacorta, Juan Jiménez-Jáimez, Tomás Ripoll-Vera, Antoni Bayes-Genis, José Manuel Garcia-Pinilla, Julián Palomino-Doza, Coloma Tiron, Gianluca Pontone, Jan Bogaert, Giovanni D. Aquaro, Juan Ramon Gimeno-Blanes, Esther Zorio, Pablo Garcia-Pavia, Roberto Barriales-Villa, Artur Evangelista, Pier Giorgio Masci, Ignacio Ferreira-González, José F. Rodríguez-Palomares

Original languageEnglish
Pages (from-to)643-662
Number of pages20
JournalJournal of the American College of Cardiology
Volume78
Issue number7
DOIs
Published17 Aug 2021

Bibliographical note

Funding Information: The project was partially funded by a grant from the Catalan Society of Cardiology (Barcelona, Spain). Hospital Universitario Virgen de la Arrixaca (Murcia, Spain) was supported by a grant from the Foundation Marató TV3 (218/C/2015) (Barcelona, Spain). Hospital Universitario y Politécnico La Fe (Valencia, Spain) was partially supported by Fondo Europeo de Desarrollo Regional (“Unión Europea, Una forma de hacer Europa”) (Madrid, Spain) and the Instituto de Salud Carlos III (La Fe Biobank PT17/0015/0043) (Madrid, Spain). Dr Guala was supported by funding from the Spanish Ministry of Science, Innovation and Universities (IJC2018-037349-I) (Madrid, Spain). Dr La Mura was supported by a research grant from the Cardiopath PhD program (Naples, Italy). Prof de la Pompa was supported by grants PID2019-104776RB-I00 and CB16/11/00399 (CIBER CV) from the Spanish Ministry of Science, Innovation and Universities. Dr Bayes-Genis was supported by grants from CIBER Cardiovascular (CB16/11/00403 and 16/11/00420) (Madrid, Spain) and AdvanceCat 2014-2020 (Barcelona, Spain); and has received advisory board and lecture fees from Novartis, Boehringer Ingelheim, Vifor, Roche Diagnostics, and Critical Diagnostics. Dr Pontone has received speaker honorarium and/or institutional research grants from GE Healthcare, Bracco, Boehringer Ingelheim, and HeartFlow. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Publisher Copyright: © 2021 American College of Cardiology Foundation

King's Authors

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.

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