Background Cardiovascular disease is an important cause of morbidity and mortality in sickle cell disease (SCD). We sought to characterize sickle cell cardiomyopathy using multi-modality non-invasive cardiovascular testing and identify potential causative mechanisms. Methods and Results Stable adults with SCD (n=38) and healthy controls (n=13) prospectively underwent same day multi-parametric cardiovascular magnetic resonance (cine, T2* iron, vasodilator first pass myocardial perfusion, and late gadolinium enhancement (LGE) imaging), transthoracic echocardiography, and applanation tonometry. Compared to controls, patients with SCD had severe dilation of the left ventricle (124±27 vs 79±12 ml/m2), right ventricle (127±28 vs 83±14 ml/m2), left atrium (65±16 vs 41±9 ml/m2), and right atrium (78±17 vs 56±17 ml/m2), p<0.01 for all. SCD patients also had a 21% lower myocardial perfusion reserve index than control subjects (1.47±0.34 vs 1.87±0.37, p=0.034). A significant subset of SCD patients (25%) had evidence of LGE while only one patient had evidence of myocardial iron overload. Diastolic dysfunction was present in 26% of SCD patients compared to 8% in controls. Estimated filling pressures (E/e’, 9.3±2.7 vs 7.3±2.0, p=0.0288) was higher in SCD patients. Left ventricular dilation and the presence of LGE were inversely correlated to hepatic T2* times (i.e. hepatic iron overload due to frequent blood transfusions, p<0.05 for both); whereas, diastolic dysfunction and increased filling pressures were correlated to aortic stiffness (augmentation pressure and index, p<0.05 for all). Conclusions Sickle cell cardiomyopathy is characterized by 4-chamber dilation and in some patients myocardial fibrosis, abnormal perfusion reserve, diastolic dysfunction, and only very rarely myocardial iron overload. Left ventricular dilation and myocardial fibrosis are associated with increased blood transfusion requirements while left ventricular diastolic dysfunction is predominantly correlated with increased aortic stiffness.

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Objectives: The objectives were to report the baseline (prior to quality improvement interventions) patient and visit characteristics and analgesic management practices for each site participating in an emergency department (ED) sickle cell learning collaborative.Methods: A prospective, multisite longitudinal cohort study in the context of a learning-collaborative model was performed in three midwestern EDs. Each site formed a multidisciplinary team charged with improving analgesic management for patients with sickle cell disease (SCD). Each team developed a nurse-initiated analgesic protocol for SCD patients (implemented after a baseline data collection period of 3.5 months at one site and 10 months at the other two sites). All sites prospectively enrolled adults with an acute pain crisis and SCD. All medical records for patients meeting study criteria were reviewed. Demographic, health services, and analgesic management data were abstracted, including ED visit frequency data, ED disposition, arrival and discharge pain score, and name and route of initial analgesic administered. Ten interviews per quarter per site were conducted with patients within 14 days of their ED discharge, and subjects were queried about the highest level of pain acceptable at discharge. The primary outcome variable was the time to initial analgesic administration. Variable data were described as means and standard deviations (SDs) or medians and interquartile ranges (IQR) for nonnormal data.Results: A total of 155 patients met study criteria (median age = 32 years, IQR = 24-40 years) with a total of 701 ED visits. Eighty-six interviews were conducted. Most patients (71.6%) had between one and three visits to the ED during the study period. However, after removing Site 3 from the analysis because of the short data enrollment period (3.5 months), which influenced the mean number of visits for the entire cohort, 52% of patients had between one and three ED visits over 10 months, 21% had four to nine visits, and 27% had between 10 and 67 visits. Fifty-nine percent of patients were discharged home. The median time to initial analgesic for the cohort was 74 minutes . Differences between choice of analgesic agent and route selected were evident between sites. For the cohort, 680 initial analgesic doses were given (morphine sulfate, 42%; hydromorphone, 46%; meperidine, 4%; morphine sulfate and ibuprofen or ketorolac, 7%) using the following routes: oral (2%), intravenous (67%), subcutaneous (3%), and intramuscular (28%). Patients reported a significantly lower targeted discharge pain score (mean ± SD = 4.19 ± 1.18) compared to the actual documented discharge pain score within 45 minutes of discharge (mean ± SD = 5.77 ± 2.45; mean difference = 1.58, 95% confidence interval = .723 to 2.44, n = 43).