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      In-hospital Outcomes and Characteristics of Heart Failure in Sickle Cell Disease

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          Abstract

          Sickle cell disease (SCD) predominantly affects African-Americans (AAs) in the United States (US). Due to increasing life expectancy in developed countries, SCD-associated cardiomyopathy is typically seen in adults. The aim of this study was to distinguish hospitalization for this phenotype from traditional heart failure (HF) in AAs. We used the National Inpatient Sample (NIS) database to identify HF hospitalizations in AAs between 2005 and 2014 and stratified them according to SCD status. We compared the characteristics and outcomes before and after matching in a 1:3 ratio for age, gender, insurance, smoking status and admission year. Amongst the 1,195,718 HF admissions in AAs, SCD accounted for 7835. The age (mean ± SD) in the SCD cohort was significantly younger (45.66 ± 13.2) vs non-SCD (64.8 ± 15.2), p<0.001. SCD adults had significantly higher rates of pulmonary hypertension (PH), deep vein thrombosis, and pulmonary embolism while non-SCD adults had higher rates of cardiogenic shock and respiratory failure requiring intubation. The national hospitalization rate for HF in AAs increased from 151 to 257 per million between 2005 and 2011 before declining to 241 per million in 2014. There was a decrease in in-hospital mortality in AAs from 4.8% in 2005 to 3.6% in 2014. We also identified independent predictors of in-hospital mortality in SCD with HF. In conclusion, we described hospitalizations for an emerging heart failure phenotype in AAs. Although there is a national decreasing rate of HF hospitalizations in the US, this may not be reflective of the AA population.

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          Pulmonary hypertension as a risk factor for death in patients with sickle cell disease.

          The prevalence of pulmonary hypertension in adults with sickle cell disease, the mechanism of its development, and its prospective prognostic significance are unknown. We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 195 consecutive patients (82 men and 113 women; mean [+/-SD] age, 36+/-12 years). Pulmonary hypertension was prospectively defined as a tricuspid regurgitant jet velocity of at least 2.5 m per second. Patients were followed for a mean of 18 months, and data were censored at the time of death or loss to follow-up. Doppler-defined pulmonary hypertension occurred in 32 percent of patients. Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified a self-reported history of cardiovascular or renal complications, increased systolic blood pressure, high lactate dehydrogenase levels (a marker of hemolysis), high levels of alkaline phosphatase, and low transferrin levels as significant independent correlates of pulmonary hypertension. The fetal hemoglobin level, white-cell count, and platelet count and the use of hydroxyurea therapy were unrelated to pulmonary hypertension. A tricuspid regurgitant jet velocity of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was strongly associated with an increased risk of death (rate ratio, 10.1; 95 percent confidence interval, 2.2 to 47.0; P<0.001) and remained so after adjustment for other possible risk factors in a proportional-hazards regression model. Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Therapeutic trials targeting this population of patients are indicated. Copyright 2004 Massachusetts Medical Society
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            A hemodynamic study of pulmonary hypertension in sickle cell disease.

            The prevalence and characteristics of pulmonary hypertension in adults with sickle cell disease have not been clearly established. In this prospective study, we evaluated 398 outpatients with sickle cell disease (mean age, 34 years) at referral centers in France. All patients underwent Doppler echocardiography, with measurement of tricuspid-valve regurgitant jet velocity. Right heart catheterization was performed in 96 patients in whom pulmonary hypertension was suspected on the basis of a tricuspid regurgitant jet velocity of at least 2.5 m per second. Pulmonary hypertension was defined as a mean pulmonary arterial pressure of at least 25 mm Hg. The prevalence of a tricuspid regurgitant jet velocity of at least 2.5 m per second was 27%. In contrast, the prevalence of pulmonary hypertension as confirmed on catheterization was 6%. The positive predictive value of echocardiography for the detection of pulmonary hypertension was 25%. Among the 24 patients with confirmed pulmonary hypertension, the pulmonary-capillary wedge pressure was 15 mm Hg or less (indicating precapillary pulmonary hypertension) in 11 patients. Patients with confirmed pulmonary hypertension were older and had poorer functional capacity and higher levels of N-terminal pro-brain natriuretic peptide than other patients. In contrast, patients who had a tricuspid regurgitant jet velocity of at least 2.5 m per second without pulmonary hypertension and patients with a tricuspid regurgitant jet velocity of less than 2.5 m per second had similar clinical characteristics. In this study of adults with sickle cell disease, the prevalence of pulmonary hypertension as confirmed on right heart catheterization was 6%. Echocardiographic evaluation alone had a low positive predictive value for pulmonary hypertension. (Funded by the French Ministry of Health and Assistance Publique-Hôpitaux de Paris; ClinicalTrials.gov number, NCT00434902.).
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              National Differences in Trends for Heart Failure Hospitalizations by Sex and Race/Ethnicity

              Background National heart failure (HF) hospitalization rates have not been appropriately age-standardized by sex or race/ethnicity. Reporting hospital utilization trends by subgroup is important for monitoring population health and developing interventions to eliminate disparities. Methods and Results The National Inpatient Sample (NIS) was used to estimate the crude and age-standardized rates of HF hospitalization between 2002 and 2013 by sex and race/ethnicity. Direct standardization was used to age-standardize rates to the 2000 U.S. standard population. Relative differences between subgroups were reported. The national age-adjusted HF hospitalization rate decreased 30.8% from 526.86 to 364.66 per 100,000 between 2002 and 2013. While hospitalizations decreased for all subgroups, the ratio of the age-standardized rate for males compared to females increased from 20% greater to 39% (p-for-trend=0.002) between 2002 and 2013. Black males had a rate that was 229% (p-for-trend=0.141) and black females 240% (p-for-trend=0.725) with reference to whites in 2013 with no significant change between 2002 and 2013. Hispanic males had a rate that was 32% greater in 2002 and the difference narrowed to 4% (p-for-trend=0.047) greater in 2013 relative to whites. For Hispanic females the rate was 55% greater in 2002 and narrowed to 8% greater (p-for-trend=0.004) in 2013 relative to whites. Asian/Pacific Islander (PI) males had a 27% lower rate in 2002 that improved to 43% (p-for-trend=0.040) lower in 2013 relative to whites. For Asian/PI females the hospitalization rate was 24% lower in 2002 and improved to 43% (p-for-trend=0.021) lower in 2013 relative to whites. Conclusions National HF hospitalization rates have decreased steadily over the recent decade. Disparities in HF burden and hospital utilization by sex and race/ethnicity persist. Significant population health interventions are needed to reduce the HF hospitalization burden among blacks. The evaluation of factors explaining the improvements in the HF hospitalization rates among Hispanics and Asian/PI are needed.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                15 September 2019
                September 2019
                : 11
                : 9
                : e5660
                Affiliations
                [1 ] Internal Medicine, Howard University Hospital, Washington, USA
                [2 ] Internal Medicine, Wake Forest Baptist Medical Center, Winston-Salem, USA
                [3 ] Cardiovascular Disease, George Washington University Hospital, Washington, USA
                [4 ] Cardiovascular Disease, Howard University Hospital, Washington, USA
                Author notes
                Article
                10.7759/cureus.5660
                6823030
                31720136
                a9a7d5dc-63c3-4794-8382-f99be3a5b42c
                Copyright © 2019, Fadiran et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 23 August 2019
                : 15 September 2019
                Categories
                Miscellaneous
                Other
                Cardiology

                heart failure,hospitalizations,african americans,sickle cell disease,outcomes,race/ethnicity

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