Atrial Arrhythmia in Sickle Cell Anemia: A Missing Piece to Prevent Cerebrovascular Complications?

Abstract

Background: Although Sickle Cell anemia (SCA) patients carries an increased risk of stroke and an important left atrial (LA) remodeling, the reality of atrial arrythmia (AA) has never been prospectively assessed. Purpose: The aim of this study was to identify the prevalence and predictors of atrial arrhythmia in SCA patients among Drepacoeur registry. Methods: From January 2019 to November 2022, consecutive adult patients with SCA were referred to the physiology department to specifically analyze cardiac function and were prospectively included. They underwent a 24-hour electrocardiogram monitoring (ECG-Holter), transthoracic echocardiography, and laboratory tests on the same day. The primary end point was the occurrence of AA, defined by the presence of excessive supraventricular ectopic activity (ESVEA) on ECG-holter (i.e. >720 premature atrial contractions [PACs] or any run ≥ 20 PACs), recent history of paroxysmal atrial fibrillation (AFib), of persistant AFib. Results: Overall, 130 patients were included. Mean age was 45±12 years, 48% were male. AA was observed in 34 (26%) patients (17 with more than 720 PACs/day, 6 with at least a run ≥ 20 PACs, 11 with recent history of paroxysmal AFib and 5 with persistant AFib). Ageing (52±9 vs. 42±12 years, P=0,004), LA dilation (LAV i, 71±24 vs. 52±14 ml/m², P<0.001) and history of stroke without underlying cerebral vasculopathy (23% vs. 7%, P=0.04) were independently associated with AA. Age and LAV i correlated with PAC load on ECG-holter (R=0,56, P<0,001 and R=0,33, P<0.001 respectively). Interestingly n age over 47 years or a LAV i >55ml/m² could predict AA with a positive prective value of 33% and a negative one of 92%. Conclusion: Atrial arrythmia is common in adult SCA patients and is associated with ageing, LA remodeling and considerably increases the risk of ischemic stroke. This study provides tools for early detection of AA and prevent cardiac and cerebrovascular dramatic complications in this highly vulnerable population.