{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T11:20:13Z","timestamp":1781263213552,"version":"3.54.1"},"reference-count":66,"publisher":"American Society of Hematology","issue":"5","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,8,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Sudden death is 1 of the leading causes of death in adults with sickle cell anemia (SCA) but its etiology remains mostly unknown. Ventricular arrhythmia (VA) carries an increased risk of sudden death; however, its prevalence and determinants in SCA are poorly studied. This study aimed to identify the prevalence and predictors of VA in patients with SCA. From 2019 to 2022, 100 patients with SCA were referred to the physiology department to specifically analyze cardiac function and prospectively included in the DREPACOEUR registry. They underwent a 24-hour electrocardiogram monitoring (24h-Holter), transthoracic echocardiography, and laboratory tests on the same day. The primary end point was the occurrence of VA, defined as sustained or nonsustained ventricular tachycardia (VT), &amp;gt;500 premature ventricular contractions (PVCs) on 24h-Holter, or a recent history of VT ablation. The mean patient age was 46\u00a0\u00b1 13 years, and 48% of the patients were male. Overall, VA was observed in 22 (22%) patients. Male sex (81% vs 34%; P\u00a0= .02), impaired global longitudinal strain (GLS): \u201316%\u00a0\u00b1 1.9% vs \u201318.3%\u00a0\u00b1 2.7%; P\u00a0= .02), and decreased platelet count (226\u00a0\u00b1 96 giga per liter [G\/L] vs 316\u00a0\u00b1 130 G\/L) were independently associated with VA. GLS correlated with PVC load every 24 hours (r\u00a0= 0.39; P\u00a0&amp;lt;\u00a0.001) and a cutoff of \u201317.5% could predict VA with a sensitivity of 82% and a specificity of 63%. VAs are common in patients with SCA, especially in men. This pilot study uncovered GLS as a valuable parameter for improving rhythmic risk stratification.<\/jats:p>","DOI":"10.1182\/blood.2022019571","type":"journal-article","created":{"date-parts":[[2023,5,22]],"date-time":"2023-05-22T21:34:00Z","timestamp":1684791240000},"page":"409-420","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":14,"title":["Determinants of ventricular arrhythmias in sickle cell anemia: toward better prevention of sudden cardiac death"],"prefix":"10.1182","volume":"142","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0414-0962","authenticated-orcid":false,"given":"Thomas","family":"d\u2019Humi\u00e8res","sequence":"first","affiliation":[{"name":"1Department of Physiology, FHU SENEC, Henri Mondor Hospital, Assistance Publique H\u00f4pitaux de Paris, INSERM IMRB U955, Universit\u00e9 Paris Est, Cr\u00e9teil, France"},{"name":"2Sickle Cell Referral Center \u2013 UMGGR, Plateforme d\u2019expertise Maladies Rares Grand Paris Est, Universit\u00e9 Paris Est, FHU SENEC, CHU Henri Mondor, Assistance Publique H\u00f4pitaux de Paris, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Saba","sequence":"additional","affiliation":[{"name":"1Department of Physiology, FHU SENEC, Henri Mondor Hospital, Assistance Publique H\u00f4pitaux de Paris, INSERM IMRB U955, Universit\u00e9 Paris Est, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6862-8975","authenticated-orcid":false,"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[{"name":"3Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, H\u00f4pital Bic\u00eatre, Assistance Publique H\u00f4pitaux de Paris, Universit\u00e9 Paris-Saclay, School of Medicine, Le Kremlin-Bic\u00eatre, France"},{"name":"4INSERM UMR_S 999 Pulmonary Hypertension, Pathophysiology 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Assistance Publique H\u00f4pitaux de Paris, Cr\u00e9teil, France"},{"name":"5Department of Internal Medicine, Henri-Mondor University Hospital-UPEC, Assistance Publique-H\u00f4pitaux de Paris, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Alassaad","sequence":"additional","affiliation":[{"name":"1Department of Physiology, FHU SENEC, Henri Mondor Hospital, Assistance Publique H\u00f4pitaux de Paris, INSERM IMRB U955, Universit\u00e9 Paris Est, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gonzalo","family":"de Luna","sequence":"additional","affiliation":[{"name":"2Sickle Cell Referral Center \u2013 UMGGR, Plateforme d\u2019expertise Maladies Rares Grand Paris Est, Universit\u00e9 Paris Est, FHU SENEC, CHU Henri Mondor, Assistance Publique H\u00f4pitaux de Paris, Cr\u00e9teil, France"},{"name":"5Department of Internal Medicine, Henri-Mondor University Hospital-UPEC, Assistance 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