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Sickle Cell anemia (SCA) patients carry an increased risk of stroke and an important left atrial (LA) remodeling. Some healthcare data base analysis revealed an increased risk of arrhythmia in SCA but the reality of atrial arrhythmia (AA) has never been prospectively. The aim of this study was to identify predictors of atrial arrhythmia in SCA patients. From January 2019 to November 2022, consecutive adult patients with SCA referred to ambulatory cardiology department for possible cardiac involvement were prospectively included (Drepacoeur cohort). All patients that had 24-hours ECG monitoring (24h-holter), transthoracic echocardiography (TTE) and biology workup on the same day were analyzed in this study. The primary end point was the occurrence of AA, defined by the presence of excessive supraventricular ectopic activity (ESVEA) on 24h-holter (i.e. > 720 premature atrial contractions [PACs] or any run ≥ 20 PACs), or recent history of paroxysmal atrial fibrillation (AF). Patients in persistent AF were excluded from the analysis. Overall, 130 patients were included and 125 were analyzed (5 exclusions for persistent AF). Mean age was 44 ± 12 years (range 18–69), 48% were male. Heart function was mainly preserved with a mean left ventricular ejection fraction (LVEF) of 57.5 ± 5.9% and LA was dilated (56 ± 19 mL/m2). Atrial arrhythmia was observed in 28 (22%) patients (17 with more than 720 PACs/day, 7 with at least a run ≥ 20 PACs and 10 with recent history of paroxysmal AF). In univariate analysis, AA was associated with a multitude of clinical, echocardiographic and biological parameters (Figure 1). In multivariate analysis, age and LA volume were independently associated with AA (OR = 1,07 CI95% [1,01 ; 1,14], P = 0,02 and OR = 1,04 CI95% [1,01 ; 1,07], P = 0,03 respectively). They both were correlated with PAC load on 24h-holter (R = 0,55, P < 0,001 and R = 0,31, P < 0.001 respectively). Interestingly, an age over 46 years old or a LA volume > 53 mL/m2 could predict AA with a PPV of 32% and a NPV of 98%. Atrial arrythmia is common in adult SCA patients and is associated with ageing and LA remodeling. This study provides tools for early detection of AA in this highly vulnerable population. [ABSTRACT FROM AUTHOR]

Unexplained sudden death remains a leading cause of death in sickle cell disease (SCD) adults. Ventricular arrhythmia is a well-known risk factor for sudden death but its prevalence and determinants in SCD remain understudied. The aim of this study was to identify echocardiographic predictors of ventricular arrhythmia in SCD. From January 2019 to March 2021, SCD adults referred to ambulatory cardiology department for possible cardiac involvement were prospectively included (Drepacoeur cohort). Patients that had 24-hours ECG monitoring (24 h-Holter) and thransthoracic echocardiography (TTE) on the same day were analysed. The primary end point was the occurrence of ventricular arrhythmia (sustained or non-sustained ventricular tachycardia (VT), more than 500 premature ventricular contractions (PVC) on 24 h-Holter, or history of VT ablation). Overall, 90 patients were included and 54 (60%) analysed. Mean age was 47.6 ± 11.6 years (range 21–69), 53% were male. Heart function was mainly preserved with a mean left ventricular ejection fraction (LVEF) of 57.9 ± 4.9% and a mean global longitudinal strain (GLS) of −18 ± 2.8%. Mean tricuspid regurgitation velocity was 2.6 ± 0.4 m/s. Ventricular arrhythmia was observed in 13 (24.1%) patients (4 non-sustained VT, 9 with more than 500 PVC and 1 history of VT ablation). Ventricular arrhythmia was associated with lower GLS (−15.8 ± 1.8% vs. −19 ± 2.7%, P < 0.001), lower tricuspid annular plane systolic excursion (TAPSE 23 ± 5.7 mm vs. 26.9 ± 4.6 mm, P = 0.02) and more dilated right ventricle assessed by tricuspid annulus diameter (38.2 ± 6.1 mm vs. 34.2 ± 4.5 mm, P = 0.02). In multivariate analysis, GLS was independently associated with ventricular arrhythmia (OR = 2.1, 95% CI [1,3; 3,3], P = 0.004) with a moderate exponential correlation with PVC load on 24 h-Holter (R = 0.5, P < 0.001). In SCD adults with preserved LVEF, GLS was the only independent echocardiographic predictor of ventricular arrhythmia. [ABSTRACT FROM AUTHOR]

Unexplained sudden death remains one of the leading causes of death in sickle cell disease (SCD) adults. Ventricular arrhythmia is a well-known risk factor for sudden death but its prevalence and determinants in the context of SCD remain understudied. From January 2019 to March 2021, consecutive adult patients with SCD referred to ambulatory cardiology department for possible cardiac involvement were prospectively included (Drepacoeur cohort). All patients that had 24-hour ECG monitoring (24h-holter) and thransthoracic echocardiography (TTE) on the same day were analysed. The primary end point was the occurrence of ventricular arrhythmia, defined as sustained or non-sustained ventricular tachycardia (VT), more than 500 premature ventricular contractions (PVC) on 24h-holter, or history of VT ablation. Overall, 90 patients were included and 54 (60%) were analysed. Mean age was 47.6 ± 11.6 years (range 21-69), 53% were male. Heart function was mainly preserved with a mean left ventricular ejection fraction (LVEF) of 57.9 ± 4.9% and a mean global longitudinal strain (GLS) of -18 ± 2.8%. Mean tricuspid regurgitation velocity was 2.6 ± 0.4 m/s. Ventricular arrhythmia was observed in 13 (24.1%) patients (4 non-sustained VT, 9 with more than 500 PVC and 1 history of VT ablation). Ventricular arrhythmia was associated with lower GLS (−15.8 ± 1.8% vs. −19 ± 2.7%, P < 0,001), lower tricuspid annular plane systolic excursion (TAPSE, 23 ± 5.7 mm vs. 26.9 ± 4.6 mm, P = 0,02) and more dilated right ventricle assessed by tricuspid annulus diameter (38.2 ± 6.1 mm vs. 34.2 ± 4.5 mm, P = 0,02). In multivariate analysis, GLS was independently associated with ventricular arrhythmia (OR = 2,1 CI95% [1,3; 3,3], P = 0,004) with a moderate exponential correlation with PVC load on 24h-holter (R = 0,5, P < 0,001). In SCD adults with preserved LVEF, GLS was the only independent echocardiographic predictor of ventricular arrhythmia. [ABSTRACT FROM AUTHOR]