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Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled. Forty-three patients (21 males and 22 females) died during the study period. The median age of survival was 39 years for females (95% CI: 34-56), 40 years for males (95% CI: 34-48), and 40 years overall (95% CI: 35-48). Cardiac causes of death accounted for 25.6% (11/43 patients); pulmonary, 14.0% (six patients); other SCD related, 32.6% (14 patients); unknown, 14.0% (six patients); and others, 14.0% (six patients). Pulseless electrical activity arrest, pulmonary emboli, multiorgan failure, and stroke were the most frequent causes of death. Among the deceased patients, the most common premorbid conditions were cardiopulmonary: acute chest syndrome/pneumonia (58.1%), Pulmonary hypertension (pHTN; 41.9%), systemic HTN (25.6%), congestive heart failure (25.6%), myocardial infarction (20.9%), and arrhythmias (14.0%). Tricuspid regurgitant jet velocity was significantly higher (3.1 m/sec vs. 2.6 m/sec, P < 0.001) and hemoglobin significantly lower (8.3 g/dL vs. 9.2 g/dL, P < 0.05) in deceased patients when compared with patients who lived, respectively. With improved preventive and therapeutic advances, including hydroxyurea therapy, acute complications such as infection are no longer the leading cause of death; instead, causes of death and premorbid conditions are shifting to chronic cardiopulmonary complications. Further, arrhythmia leading to premature death is under-recognized in SCD and warrants further investigation.

Sickle cell disease (SCD) is associated with extensive morbidity and early mortality. Although the most common known causes of death for adults with SCD are acute chest syndrome, stroke, pulmonary hypertension, and infection, the direct cause of death is frequently undefined, and patients often die suddenly. In one series of 306 autopsies of patients with SCD, death was sudden and unexpected in 41% of cases (Manci et al 2003). The incidence of sudden cardiac death and associated risk factors in patients with SCD are currently unknown. We sought to identify risk factors for mortality in adult subjects with SCD and to evaluate the frequency, risk factors and co-morbidities of sudden death in this population. We identified 43 adult patients (21 males and 22 females) who had been followed in the SCD clinic at Duke University Medical Center (DUMC) and who had died between January 2000 and April 2005. Clinical characteristics and laboratory data were evaluated by retrospective chart review. Findings were compared with data from patients who were actively followed during the same time period and were still living (n=197). The average age at death was 44.3 years (range 21–83). The most frequently listed causes of death were liver failure, multiorgan failure, stroke, and pulseless electrical activity (PEA) arrest. The etiology of death in 29 of the 43 patients was unknown. Recognized risk factors for sudden cardiac death, including ejection fraction (52% vs. 54%), left ventricular size (LVIDd 5.0cm vs. 5.2cm), and fractional shortening (0.30 ±0.01 vs. 0.33± 0.01) as measured by echocardiogram, were not significantly different between deceased and living patients, respectively. Left ventricular hypertrophy (LVH), defined as a left ventricular mass index ≥134 and ≥110 g/m2 for men and women, was reported in 41% of the deceased patients but in only 31% of living subjects. Of the 12 deceased patients with LVH, 7 had mild LVH and 5 had moderate-severe LVH. The average tricuspid regurgitant jet velocity measured by Doppler echocardiogram was higher in patients who died compared to those who were still living (3.72 vs. 2.17 m/s). The most frequently documented cardiopulmonary complications among deceased patients were acute chest syndrome/pneumonia, pulmonary hypertension, systemic hypertension, and stroke. Identified risk factors associated with premature death were pulmonary hypertension (p