Impact of Pulmonary Hypertension on in-hospital outcomes amongst Sickle Cell Disease hospitalizations

Introduction: Pulmonary hypertension (PH) is a common and severe complication of Sickle Cell Disease (SCD), and an independent risk factor for mortality. While there is a clear association between SCD and PH, the predictors of PH in SCD and the impact of PH on in-hospital outcomes of SCD hospitalizations remains unknown. In our study, we sought to assess the in-hospital prevalence, predictors, and the impact of PH in SCD hospitalizations. Methods: We used the 2016 and 2017 National Inpatient Sample (NIS) to identify all adult hospitalizations with a primary discharge diagnosis of SCD. The sample was then stratified based on the presence or absence of PH. We used the Pearson chi-square test and weighted Student's t-test to analyze categorical and continuous variables, respectively. Multivariate logistic regression analysis was performed to calculate the adjusted odds ratio for various clinical outcomes. SAS was used for the analysis, and the p-value was defined as <0.05. Results: We identified n=191,080 weighted hospitalizations for SCD, of which, 5.54% (n=10590) had concomitant PH. Female gender and comorbidities including hypertension, obesity, illicit drug use, hepatic cirrhosis, renal failure, prior venous-thromboembolism, valvular, and congenital heart disease were identified as significant predictors of PH in SCD. PH was associated with increased in-hospital mortality (1.04% vs 0.22%, AOR=2.14, 95% CI 1.15-3.98, p=0.0158). PH in SCD hospitalizations also increased the odds of - acute kidney injury (AKI), need for dialysis, acute respiratory failure (ARF), and need for mechanical ventilation for > 96 hours. The adjusted odds ratio for venous thromboembolism, shock-state, and the need for cardiac catheterization (both right and bilateral) were also higher in patients with PH. Overall hospitalization cost and length of stay increased (7.06±0.16 vs 1.82±0.02 days) in patients with SCD and PH (see table 1). Conclusion: In sickle cell disease hospitalizations, PH is independently associated with increased in-hospital morbidity and mortality, with an increased need for in-hospital catheterizations thereby, prolonging the length of stay and overall health care costs. Identifying and treating PH in the SCD population would improve in-hospital outcomes. Disclosures No relevant conflicts of interest to declare.

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Sickle cell trait and the risk of venous thromboembolism among blacks

Blood ◽

10.1182/blood-2006-11-057604 ◽

2007 ◽

Vol 110 (3) ◽

pp. 908-912 ◽

Cited By ~ 142

Author(s):

Harland Austin ◽

Nigel S. Key ◽

Jane M. Benson ◽

Cathy Lally ◽

Nicole F. Dowling ◽

...

Keyword(s):

Venous Thromboembolism ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Odds Ratio ◽

Sickle Cell Trait ◽

Coagulation System ◽

Cell Disease ◽

Hemoglobin C ◽

Increased Risk ◽

S Allele

Abstract People with sickle cell disease have a chronically activated coagulation system and display hemostatic perturbations, but it is unknown whether they experience an increased risk of venous thromboembolism. We conducted a case–control study of venous thromboembolism that included 515 hospitalized black patients and 555 black controls obtained from medical clinics. All subjects were assayed for hemoglobin S and hemoglobin C genotypes. The prevalence of the S allele was 0.070 and 0.032 for case patients and controls, respectively (P < .001). The odds that a patient had sickle cell trait were approximately twice that of a control, indicating that the risk of venous thromboembolism is increased approximately 2-fold among blacks with sickle cell trait compared with those with the wild-type genotype (odds ratio = 1.8 with 95% confidence interval, 1.2-2.9). The odds ratio for pulmonary embolism and sickle cell trait was higher, 3.9 (2.2-6.9). The prevalence of sickle cell disease was also increased among case patients compared with controls. We conclude that sickle cell trait is a risk factor for venous thromboembolism and that the proportion of venous thromboembolism among blacks attributable to the mutation is approximately 7%.

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Venous Thromboembolism In Pregnant Women With Sickle Cell Disease

Blood ◽

10.1182/blood.v122.21.2217.2217 ◽

2013 ◽

Vol 122 (21) ◽

pp. 2217-2217

Author(s):

Craig D. Seaman ◽

Margaret V. Ragni ◽

Charity G. Moore ◽

Jie Li ◽

Jonathan Yabes

Keyword(s):

Venous Thromboembolism ◽

Sickle Cell Disease ◽

Length Of Stay ◽

Pregnant Women ◽

Sickle Cell ◽

Pregnancy Complications ◽

Categorical Variables ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Discharge Data

Abstract Background The risk of venous thromboembolism (VTE) is increased in pregnancy and sickle cell disease (SCD), yet the rates of pregnancy-related VTE are not firmly established in SCD, nor are other SCD-related complications, including pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS), which may be clinically indistinguishable from VTE. Moreover, the American College of Chest Physicians has made no recommendations regarding thromboprophylaxis in pregnant women with SCD. Methods Inpatient hospital discharge data for PE for the most recent 5-year period available, 2007-2011, were obtained from the Pennsylvania Health Care Cost Containment Council (PHC4). We compared VTE, pregnancy complications, medical co-morbidities, and mortality among pregnant women with and without SCD. Among pregnant SCD women with and without VTE, we also compared rates of pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS). All patient identifiers were removed. Diagnostic categories and co-morbidities were obtained using ICD-9 codes. Data from categorical variables were analyzed by chi-square or Fisher's exact test; and from continuous variables by two-sample Student t-test. Results The prevalence of VTE was 2.8% among pregnant women with SCD. Among pregnant women with SCD and VTE, the rate of pneumonia, 28.6% vs. 4.4%, p=0.043, was significantly higher than in those without VTE. While somewhat higher, the rates of VOC, 57.1% vs. 24.7%, p=0.073, and ACS, 14.3% vs. 2.4%, p=0.177, were not significantly different between pregnant SCD VTE and non-VTE groups. Comparing VTE and non-VTE pregnant SCD women, the rate of pregnancy complications did not differ, p=0.999; nor did the rates of medical co-morbidity, other than diabetes, 28.6% vs. 3.6%, p=0.031. Among the subset of pregnant SCD with pneumonia, the prevalence of VOC, 80.0% vs. 36.1%, p=0.001; ACS, 35.0% vs. 2.9%, p<0.001; and length of stay, 12.5 vs. 4.6 days, p=0.030, were significantly greater than in those without pneumonia. Among the subset of SCD pregnancies with VOC, the prevalence of preeclampsia, 28.4% vs. 13.5%, p=0.003; pneumonia, 15.7% vs. 2.6%, p=0.001; ACS, 12.8% vs. 0.6%, p<0.001; and length of stay, 7.7 vs. 3.6 days, p<0.001, were significantly more common than in those without VOC. Among the subset of SCD pregnancies with ACS, the rates of intrauterine fetal death, 14.3% vs. 1.6%, p=0.036; postpartum infection, 28.6% vs. 6.6%, p=0.016; pneumonia, 50.0% vs. 5.3%, p<0.001; and VOC, 92.9% vs. 36.5%, p<0.001, were significantly higher than in those without ACS. Overall, the rate of VTE, among SCD women with pneumonia, VOC, and/or ACS, 6.6%, was significantly higher than among those without these conditions, 2.2%, p<0.001. Discussion The prevalence of pregnancy-related VTE in women with SCD, while low, 2.8%, appears to be at least 10-fold greater than the general 0.2% incidence of pregnancy-related VTE in the unaffected population. Further, the higher rates of VTE we observed among pregnant women with SCD-related complications, including pneumonia, VOC, or ACS, and the well-recognized potential clinical overlap with VTE, suggest that VTE may be missed in such women, and that VTE rates in pregnant women with SCD may be higher than herein reported or previously recognized. The presence of pneumonia, VOC, or ACS appears to be associated with VTE and may be a potential marker(s) of its occurrence. Prospective studies, however, are needed to determine the incidence of VTE in pregnant women with SCD with and without complicating pneumonia, VOC, and/or ACS. We conclude that pregnancy-related VTE may be more common than previously recognized in pregnant women with SCD and, if confirmed, such women might be candidates for thromboprophylaxis. Disclosures: No relevant conflicts of interest to declare.

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Sickle Cell Disease and Venous Thromboembolism: Hospital Mortality, Length of Stay and Cost

Blood ◽

10.1182/blood-2018-99-119778 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 2378-2378

Author(s):

Michael Rainone ◽

Stuthi Pavani Perimbeti ◽

Rishi Shrivastav ◽

Jeffrey A. Glassberg ◽

Lawrence Cytryn

Keyword(s):

Venous Thromboembolism ◽

Sickle Cell Disease ◽

Length Of Stay ◽

Sickle Cell ◽

Treatment Guidelines ◽

Conflicts Of Interest ◽

Primary Diagnosis ◽

Cell Disease ◽

Chi Square ◽

Blood Institute

Abstract Introduction: It is estimated that there are 100,000 Americans living with Sickle Cell Disease (SCD). Patients with SCD experience a number of complications that frequently require hospitalization. SCD is a prothrombotic state that is commonly complicated by venous thromboembolism (VTE) and recurrent VTE. The National Heart, Lung and Blood Institute do not include VTE as one of the complications of SCD in their latest guidelines, and the topics of prophylaxis and treatment of VTE in SCD are not discussed. There are no guidelines specifically designed for the prophylaxis or treatment of VTE in the SCD population, and traditionally management guidelines for VTE in the general population are followed. Recent information on national prevalence, mortality, length of stay, and cost for hospitalized patients with with SCD complicated by VTE is limited. Methods: We used data from the Healthcare Cost and Utilization Project's National Inpatient Sample (NIS) from 1999-2014 to examine these variables. The data on SCD from 1999-2014 was analyzed using ICD-9-CM codes for SCD (ICD-9-CM: 282.41, 282.42, 282.6, 282.60, 282.62, 282.63, 282.64, 282.68, 282.69) in the primary diagnosis field, and VTE (ICD-9-CM: 453.40, 453.41, 453.42, 453.82, 453.83, 415.11, 415.19) in the secondary diagnosis field which includes codes for venous thrombosis and pulmonary embolism. Univariate and bivariate statistical analysis was performed using the chi-square test. Multivariate analysis was performed using cox proportional hazard regression. The alpha was set at 0.05. Results: Over a 15 year period, from 1999-2014, a total of 217,791 (weighted N = 1,073,215) admissions with SCD were identified. A total of 7,898 admissions were associated with VTE. Mean age at admission of those with VTE was 27.42 (+/- 0.05) years and those without VTE was 34.00 (+/- 0.51) years. In patients with SCD and VTE, the average inpatient mortality was 3.08% (p < 0.0001) versus mortality of 0.27% in patients that did not have VTE. The hazard ratio for mortality was 4.18 (CI: 2.95-5.93) (p < 0.0001). Length of stay in the SCD with VTE group was 10.45 days (+/- 0.43) versus 5.09 days (+/- 0.02) (p < 0.0001) in SCD without VTE. Overall hospital cost was higher in those with VTE at $60,055 (+/- $1,940) versus $28,729 (+/- 232.97) (p < 0.0001) in those without VTE. Conclusions: Patients with SCD and VTE experience significant morbidity, mortality, prolonged hospitalization and increased cost associated with this complication of the disease as was observed in this study. Furthermore, patients who experience VTE are significantly younger than those who do not, with mean age of 27 versus 34. After controlling for multiple confounders like age, race, sex, income, comorbidities, the presence of VTE is associated with a significantly higher risk of mortality in SCD. Currently, there are no prophylaxis or treatment guidelines designed specifically for patients with SCD and VTE. We recommend the use of antithrombotic prophylaxis or therapy in patients with SCD be evaluated in prospective studies. Disclosures No relevant conflicts of interest to declare.

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