Guillem Casas1, Javier Limeres2, Gerard Oristrell1, Laura Gutierrez-Garcia2, Daniele Andreini3, Mar Borregan4, Jose M Larrañaga-Moreira5, Angela Lopez-Sainz6, Marta Codina-Solà7, Gisela Teixido-Tura2, José Antonio Sorolla-Romero8, Paula Fernández-Álvarez7, Josefa González-Carrillo9, Andrea Guala10, Lucia La Mura11, Rafaela Soler-Fernández12, Augusto Sao Avilés13, Juan José Santos-Mateo14, Josep Ramon Marsal15, Aida Ribera15, José Luis de la Pompa16, Eduardo Villacorta17, Juan Jiménez-Jáimez18, Tomás Ripoll-Vera19, Antoni Bayes-Genis20, José Manuel Garcia-Pinilla21, Julián Palomino-Doza22, Coloma Tiron23, Gianluca Pontone24, Jan Bogaert25, Giovanni D Aquaro26, Juan Ramon Gimeno-Blanes27, Esther Zorio28, Pablo Garcia-Pavia29, Roberto Barriales-Villa30, Artur Evangelista2, Pier Giorgio Masci31, Ignacio Ferreira-González32, José F Rodríguez-Palomares33. 1. Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain. 2. Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Cardiovascular Disease Unit and Cardiovascular Genetics. Hospital Universitari Vall d'Hebron, Barcelona, Spain. 3. Centro Cardiologico Monzino, IRCCS, Milan, Italy; Department of Clinical Sciences and Community Health, Cardiovascular Section, University of Milan, Milan, Italy. 4. Genetics and Molecular Medicine Department and Pediatric Institute of Rare Diseases, Hospital Sant Joan de Déu, Barcelona, Spain. 5. Inherited Cardiovascular Diseases Unit, Cardiology Department, Instituto de Investigación Biomédica de A Coruña, Complexo Hospitalario Universitario de A Coruña, Servizo Galego de Saúde, Universidade da Coruña, A Coruña, Spain. 6. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Heart Failure and Inherited Cardiac Diseases Unit, Cardiology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN-GUARD-Heart), Madrid, Spain. 7. Inherited Cardiovascular Disease Unit and Cardiovascular Genetics. Hospital Universitari Vall d'Hebron, Barcelona, Spain. 8. Cardiology Department, Hospital Universitario y Politécnico La Fe de Valencia, Valencia, Spain. 9. Cardiology Department, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain. 10. Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain. 11. Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy. 12. Radiology Department, Instituto de Investigación Biomédica de A Coruña, Complexo Hospitalario Universitario de A Coruña, Servizo Galego de Saúde, Universidade da Coruña, A Coruña, Spain. 13. Cardiology Department, Vall d'Hebron Hospital, Barcelona, Spain; Neurology/Neuroimmunology Department, Vall d'Hebron Hospital, Barcelona, Spain; Statistics and Bioinformatics Unit (UEB-VHIR), Vall d'Hebron Hospital, Barcelona, Spain. 14. Cardiology Department, Hospital Rafael Méndez de Lorca, Murcia, Spain. 15. Epidemiology Unit of the Cardiology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain; Centro de Investigación Biomédica en Red en Epidemiología y Salud Pública, Madrid, Spain. 16. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Intercellular Signalling in Cardiovascular Development and Disease Laboratory, Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain. 17. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Cardiology Department, Hospital Universitario de Salamanca, Salamanca, Spain. 18. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Cardiology Department, Hospital Universitario Virgen de las Nieves, Granada, Spain. 19. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Cardiology Department, Hospital Universitario Son Llatzer and Institut d'Investigaciò Sanitària Illes Balears, Mallorca, Spain. 20. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Heart Failure Unit, Hospital Universitari Germans Trias i Pujol, Badalona, Spain; Cardiology Department, Hospital Universitari Germans Trias i Pujol, Badalona, Spain; Medicine Department, Universitat Autònoma de Barcelona, Barcelona, Spain. 21. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Heart Failure and Familial Cardiomyopathies Unit, Cardiology Department, Hospital Universitario Virgen de la Victoria, Instituto de Investigación Biomédica de Málaga, Málaga, Spain. 22. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Cardiology Department, Hospital Universitario 12 de Octubre, Madrid, Spain. 23. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitari Dr Josep Trueta, Girona, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain. 24. Centro Cardiologico Monzino, IRCCS, Milan, Italy. 25. Gasthuisberg University Hospital, Leuven, Belgium. 26. Fondazione CNR-Regione Toscana G. Monasterio, Pisa, Italy. 27. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Cardiology Department, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain. 28. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Heart Diseases Unit, Cardiology Department, Hospital Universitario y Politécnico La Fe de Valencia, Valencia, Spain; CAFAMUSME Research Group, Instituto de Investigación Sanitaria La Fe, Valencia, Spain. 29. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Heart Failure and Inherited Cardiac Diseases Unit, Cardiology Department, Hospital Universitario Puerta de Hierro, Madrid, Spain; European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart (ERN-GUARD-Heart), Madrid, Spain; Universidad Francisco de Vitoria, Pozuelo de Alarcón, Spain. 30. Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Cardiovascular Diseases Unit, Cardiology Department, Instituto de Investigación Biomédica de A Coruña, Complexo Hospitalario Universitario de A Coruña, Servizo Galego de Saúde, Universidade da Coruña, A Coruña, Spain. 31. School of Biomedical Engineering and Imaging Sciences, King's College London, London, United Kingdom. 32. Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain; Epidemiology Unit of the Cardiology Department, Hospital Universitari Vall d'Hebron, Barcelona, Spain; Centro de Investigación Biomédica en Red en Epidemiología y Salud Pública, Madrid, Spain. Electronic address: iferreir@vhebron.net. 33. Cardiology Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca, Departament de Medicina, Universitat Autónoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Cardiovasculares, Madrid, Spain; Inherited Cardiovascular Disease Unit and Cardiovascular Genetics. Hospital Universitari Vall d'Hebron, Barcelona, Spain. Electronic address: jfrodriguez@vhebron.net.
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.
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.
Authors: George Bazoukis; Konstantinos Tyrovolas; Konstantinos P Letsas; Konstantinos Vlachos; Danny Radford; Cheuk To Chung; Tong Liu; Michael Efremidis; Gary Tse; Adrian Baranchuk Journal: Heart Fail Rev Date: 2022-07-01 Impact factor: 4.654