Clinical Risk Prediction in Patients With Left Ventricular Myocardial Noncompaction

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 RiberaJosé 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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

38 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)643-662
Number of pages20
JournalJournal of the American College of Cardiology
Issue number7
Publication statusPublished - 17 Aug 2021


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


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