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• W2992504037 abstract "The Cooperative Study of Sickle Cell Disease (CSSCD) accrued patients until 1996, with approx. 30–40% of mortality in CSSCD being sudden death at home or rapid deterioration within 24 hours of presentation to hospital (Platt et al., 1994; Manci et al., 2003). With overall improved survival in sickle cell disease (SCD), we sought to identify the rates of and factors associated with sudden death in the years following 1998 FDA approval of hydroxyurea for SCD. We conducted a retrospective study of deaths in SCD patients as documented by the Partners HealthCare system, using the Partners Research Patient Data Registry (RPDR). RPDR is a centralised data registry of clinical information from hospitals within the largest healthcare system in the Northeastern US. The RPDR was queried from 1997 to 2017, excluding patients who died before 1998, to identify all SCD patients who were deceased. Patients’ charts were reviewed to confirm SCD, identify cause of death, and extract SCD-related clinical data between 2 years and 2 weeks prior to death. Sudden death was defined as unexpected death occurring in a relatively healthy SCD patient who died at home or within 24 hours of initial hospitalisation, with or without a vaso-occlusive crisis. Cause of death was categorised into five groups: sudden death at home (Group I), death of known cause related to SCD (Group II), death of known cause unrelated to SCD (Group III), unexpected death within 24 hours of hospitalisation (Group IV) and death of unknown cause (Group V). Deaths attributable to a cause were excluded from Group IV. Patients in Group I and Group IV were classified as having died of ‘sudden unexpected death’ (SUD). Patient variables, listed in Table 1, were compared between classes: SUD (Group I + IV) versus other deaths (Groups II, III, V). Categorical variables were summarised as percentages and continuous variables by a median. The study was approved by our Institutional Review Board. We identified a total of 623 SCD patients. Sixty-one patients were deceased. See the demographics in Table 1. Nineteen patients (31%) suffered SUD: seven (11%) from sudden death at home (Group I) and 12 (20%) deaths within 24 hours of hospitalisation (Group IV). Ten (16%) died of known causes related to SCD (Group II), 22 (36%) died of known causes unrelated to SCD (Group III) and 10 (16%) died of unknown causes (Group V). Median age at death was 39 years, with no significant difference between SUD (41 years) and other causes of death (38 years). Of the 19 patients who suffered SUD, 14 (74%) had a history of Acute Chest Syndrome (ACS), compared to only 15 (36%) of those who suffered other deaths. Almost all patients with SUD (17/19 or 89%) had exposure to hydroxyurea (HU), while less than half of those who died of other deaths (18/42 or 43%) had exposure to HU. A history of priapism was found in four (40%) men who suffered SUD and none (0%) who died of other causes. Other measured variables were not different between the groups (Table 1). We found a high prevalence of non-specific electrocardiogram abnormalities – in 95% of patients with SUD and 79% of other deaths. From the available echocardiogram data, on 45 patients only, ejection fractions were normal, with no difference in median tricuspid regurgitation jet velocity (TRJV) – 2·8 m/s in the patients with SUD and 3·1 m/s in other deaths. With patients with SCD surviving to adulthood, the consequences of chronic endothelial inflammation and repeated vaso-occlusive episodes become more prevalent. Furthermore, as mortality rates from paediatric causes in SCD improve (Telfer et al., 2007; Quinn et al., 2010; Couque et al., 2016), the impact of sudden death on overall mortality rates has become more evident (Darbari et al., 2006; Graham et al., 2007). Our retrospective study of deaths after 1998 documents 31% of deaths as being sudden and unexpected or within 24 hours of presentation, not appreciably different from rates before use of HU. Cardiopulmonary factors, such as pulmonary hypertension and strokes which are associated with early mortality (Castro et al., 2003; Fitzhugh et al., 2010), did not have significant associations with SUD in our study. However, a history of ACS and priapism (in males) was more frequent in patients who suffered sudden death, whether or not these were active at the time of death. ACS is a known major cause of mortality in SCD. Its implication in SUD raises the question of whether patients with a predominantly vaso-occlusive phenotype of SCD, as opposed to the endothelial dysfunction phenotype, are more likely to suffer sudden death. Priapism (in males) was also associated with an increased risk of SUD in our study. Of the patients that did not suffer sudden death, none (0%) of the males had a reported history of priapism. The overall event rate for priapism was low, but the complete absence in the groups not suffering sudden death is noteworthy. A history of HU exposure at any time was more likely in patients who suffered SUD. It is unlikely that the relationship between HU and SUD was causal. Instead, HU use may indicate a more severe phenotype of SCD, suggesting that such patients are at increased risk of SUD. These findings are aligned with a recent meta-analysis of 3257 SCD patients, which was unable to confirm a mortality benefit with HU use in SCD (Maitra et al., 2017). Our study has some limitations – it is a retrospective study and a causal relationship can therefore not be inferred. Nevertheless, our study suggests that there is an association between sudden death and a history of ACS that may not be influenced by exposure to HU. Our study was small (61 deaths), and a larger study is required to identify other differences between patients who die of SUD and those who die of other deaths. Sudden unexpected death in SCD remains a significant problem in the HU era. Measures to influence modifiable factors that have been confirmed as foretelling SUD in SCD could have a major impact on current health maintenance practices and the rate of SUD in SCD. A prospective study of a larger cohort of patients may allow the realisation of this goal." @default.
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• W2992504037 date "2019-12-05" @default.
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• W2992504037 title "Sudden death in sickle cell disease: current experience" @default.
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