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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., Competing Interests: Funding Support and Author Disclosures 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., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Syncope in patients with mid-range left ventricular ejection fraction (LVEF) can be due to potentially serious arrhythmic causes. However, there is no clear consensus on the best way to manage these patients., Objectives: The objectives of this study were to determine the causes of syncope and assess the diagnostic yield and safety of a stepwise workup protocol in this population., Methods: This was a prospective observational study. A stepwise workup protocol was applied to patients with LVEF 35%-50% and unexplained syncope after the initial assessment (step 1)., Results: One hundred four patients were included {median age 75.6 years; (interquartile range [IQR] 67.6-81.2 years); median LVEF 45% (IQR 40%-48%); median follow-up 2.0 years (IQR 0.7-3.3 years). In 71 patients (68.3%), a diagnosis was reached: 55 (77.5%) in step 2 (hospital admission and electrophysiology study) and 16 (36.5%) in step 3 (implantable cardiac monitor). Arrhythmic causes were the most common etiology (45.2% auriculoventricular block and 9.6% ventricular tachycardia). Sixty patients (57.7%) required the implantation of a cardiac device and 11 had a defibrillation function. Patients diagnosed in step 3 had a higher global risk of recurrence of syncope (hazard ratio 6.5; 95% confidence interval 2.3-18.0). The mortality rate was 8.1 per 100 person-years, and the sudden or unknown death rate was 0.9 per 100 person-years., Conclusion: In patients with mid-range left ventricular dysfunction and syncope of unknown cause, a systematic diagnostic strategy based on electrophysiology study and/or implantable cardiac monitor implantation allows a diagnosis to be reached in a high proportion of cases and guides the treatment. Arrhythmia is the most common cause of syncope in this population, particularly auriculoventricular block., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Unlabelled: The current cardiovascular risk tables are based on a 10-year period and therefore, do not allow for predictions in the short or medium term. Thus, we are unable to take more aggressive therapeutic decisions when this risk is very high.To develop and validate a predictive model of cardiovascular disease (CVD), to enable calculation of risk in the short, medium and long term in the general population.Cohort study with 14 years of follow-up (1992-2006) was obtained through random sampling of 342,667 inhabitants in a Spanish region., Main Outcome: time-to-CVD. The sample was randomly divided into 2 parts [823 (80%), construction; 227 (20%), validation]. A stepwise Cox model was constructed to determine which variables at baseline (age, sex, blood pressure, etc) were associated with CVD. The model was adapted to a points system and risk groups based on epidemiological criteria (sensitivity and specificity) were established. The risk associated with each score was calculated every 2 years up to a maximum of 14. The estimated model was validated by calculating the C-statistic and comparison between observed and expected events.In the construction sample, 76 patients experienced a CVD during the follow-up (82 cases per 10,000 person-years). Factors in the model included sex, diabetes, left ventricular hypertrophy, occupational physical activity, age, systolic blood pressure × heart rate, number of cigarettes, and total cholesterol. Validation yielded a C-statistic of 0.886 and the comparison between expected and observed events was not significant (P: 0.49-0.75).We constructed and validated a scoring system able to determine, with a very high discriminating power, which patients will develop a CVD in the short, medium, and long term (maximum 14 years). Validation studies are needed for the model constructed., Competing Interests: The authors have no conflicts of interest to disclose.