Morbidity and Associated Sudden Death in Sickle Cell Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2348-2348 ◽  
Author(s):  
Courtney Fitzhugh ◽  
Naudia Lauder ◽  
Jude Jonassaint ◽  
F. Roosevelt Gilliam ◽  
Marilyn J. Telen ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Sudden Cardiac Death ◽  
Sickle Cell Disease ◽  
Sudden Death ◽  
Cardiac Death ◽  
Causes Of Death ◽  
Left Ventricular ◽  
Acute Chest Syndrome ◽  
Cell Disease

Abstract 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<0.0001) and severe anemia (p=0.002). Baseline WBC count and oxygen saturation were not significantly different between deceased and living patients. We conclude that despite improved medical care and therapeutic advances, adult patients with SCD continue to experience a high rate of premature mortality, and a significant number of patients die suddenly. The etiology of death is frequently multifactorial and poorly defined. Identifying the variables contributing to sudden death in SCD patients may enable clinicians to successfully intervene and prevent early demise.

Progression of Pulmonary Hypertension in Patients with Sickle Cell Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3187-3187 ◽  
Author(s):  
Kenneth I. Ataga ◽  
Charity Moore ◽  
Susan Jones ◽  
Oludamilola Olajide ◽  
Dell Strayhorn ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Left Ventricular ◽  
Right Atrial ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Original Cohort ◽  
The Mean

Abstract Introduction: The prevalence of pulmonary hypertension (PHT) is high in patients with sickle cell disease (SCD). Although most patients have only mild increases in their pulmonary artery systolic pressure (PASP), the presence of PHT is strongly associated with an increased risk of death. While PHT seen in SCD is thought to progress over time, both the rate of development of PHT and the factors that affect disease progression remain unknown. Methods: The 41 subjects in this study were drawn from an original cohort of 60 patients followed in the Sickle Cell Clinic at UNC-Chapel Hill. All patients were previously evaluated for PHT (defined using an age-, sex-, and BMI-adjusted reference range). Of the 60 patients in the original cohort, six are now deceased and 13 others were not available for repeat evaluation. The PASP was determined using Doppler echocardiography and then applying the modified Bernoulli equation (PASP = 4V2 + right atrial pressure). Individuals were not studied if they: 1) showed clinical evidence of left ventricular failure; 2) had a recent acute illness (e.g., vaso-occlusive crisis); or 3) had experienced an episode of acute chest syndrome within the preceding 4 weeks. Means and standard deviations were calculated for all measures at the time of initial evaluation and at the time of follow-up. Results: Of the 41 subjects in our study, PHT was originally present in 12, while no evidence of PHT was present in 29. Of the 29 subjects who initially had no evidence of PHT, 4 (or 14%) have now developed PHT (mean follow-up period of 3.3 ± 0.4 years). In these 4 subjects, the mean PASP at the time of initial and follow-up evaluations respectively were: 37.0 ± 2.0 mm Hg vs. 55.8 ± 11.0 mm Hg. The patients who developed PHT during the course of the study had lower systolic BP (143 ± 12 mm Hg vs. 128 ± 12 mm Hg), lower fetal hemoglobin levels (6.2 ± 5.7 % vs. 4.2 ± 3.7 %), and higher platelet counts (276 ± 119 X 103/μL vs. 426 ± 96 X 103/μL) at the time of their follow-up analyses. By contrast, 3 of the 12 subjects (or 25%) who were thought to have PHT at the time of their original evaluations were found to have normal PASP determinations at the time of their repeat echocardiograms (mean follow-up period of 3.2 ± 0.6 years). In these latter 3 subjects, the mean PASP values at the time of the initial and follow-up evaluations respectively were: 40.0 ± 4.6 mm Hg vs. 33.7 ± 4.7 mm Hg. Conclusion: In this small group of patients with SCD, we found that PHT developed in 14% of subjects who had no evidence of PHT 3 years earlier. Based on this observation, it seems that periodic echocardiograms to screen for the development of PHT would be appropriate. On the other hand, our observation that some patients initially classified as having PHT failed to have elevated PASP measurements at the time of follow-up illustrates the limitation of a single echocardiographic evaluation in establishing this diagnosis. Because of the increase in PASP that occurs during acute vaso-occlusive episodes, and the difficulty usually encountered in distinguishing steady state from crisis, the initial elevation of the PASP in these patients could have resulted from sub-clinical crisis states. For these reasons, a patient found to have an elevated PASP at the time of a screening echocardiogram should have a repeat study, and perhaps a right heart catheterization, before the diagnosis of PHT is firmly established.

Prolonged QTc Interval Is Common In Patients with Sickle Cell Disease and Has An Inverse Relationship to Hemoglobin and Hematocrit: Results From CSSCD

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2675-2675
Author(s):  
Ruchika Goel ◽  
Sudha Rajderkar ◽  
Rema Padman ◽  
Lakshmanan Krishnamurti
Keyword(s):  
Sudden Cardiac Death ◽  
Sickle Cell Disease ◽  
Pericardial Effusion ◽  
Sickle Cell ◽  
Odds Ratio ◽  
Qt Interval ◽  
Cardiac Death ◽  
Left Ventricular ◽  
Qtc Interval ◽  
Cell Disease

Abstract Abstract 2675 Background: Sudden unexplained premature cardiac death is common among patients with sickle cell disease (SCD). Prolonged QTc (Corrected QT interval) on electrocardiogram (EKG) is an established marker for higher risk of sudden cardiac death. There are limited data on the prevalence of prolonged QTc in SCD patients. Aim: We studied the prevalence, EKG and echocardiographic characteristics correlated with prolonged QTc in SCD patients and assessed for any predictors of prolonged QTc interval in the study subjects. Methods: Data from the National Institutes of Health sponsored Cooperative Study of Sickle Cell Disease (CSSCD), a multi-center, prospective study of the clinical course of SCD were analyzed. HbSS patients > 5 years of age from four clinical centers participated in the study. Each patient underwent a standard 12 lead EKG and echocardiogram and laboratory testing at a steady state. Bazett's square root formula was used for rate correction to calculate QTc. QTc> 440msec was considered as prolonged. Patients with a known diagnosis of congenital prolonged QTc syndrome or those on any medications with a known association with prolonged QTc were excluded from the analysis. Multivariate logistic regression was used for statistical analysis. P value of <0.05 was considered statistically significant. Results: Data from 240 patients (62% females, 100% African American with mean age 22.3 +−10.3 years were analyzed. Of these, 33.1% were women. Prolonged QTc, per the above specified definition was seen in 31.3% of the study patients. Mean serum hemoglobin (7.9 vs. 8.6; p<0.001) and hematocrit value (23.4 vs. 25.1; p = 0.002) were lower in those with prolonged QTc interval. Subjects with prolonged Qtc had slightly higher ventricular septal wall thickness (end diastole 1.23 vs. 1.12 cm, p = 0.03; end systole 1.54 vs. 1.45 cm, p = 0.09). Pericardial effusion (seen in 8.8%) was associated with higher odds of prolonged QTc (odds ratio (95% CI): 3.24 (1.24, 8.46). In a multivariable model with age, gender, venticular septal thickness, pericardial effusion and hemoglobin%, only hemoglobin% was associated with prolonged QT interval: odds ratio (SD) per unit increase in Hb% = 0.65 (0.49, 0.87). Age, gender, echocardiography determined left ventricular hypertrophy, X-ray determined cardiomegaly, PR interval, ventricular or atrial thickness or dimensions were not related to prolonged QTc interval. Conclusions: These data suggest that prolonged QTc is common among patients with sickle cell disease and that hemoglobin and hematocrit are independent and inverse predictors of prolonged QTc interval. The mechanism of prolonged QTc interval and its role if any, in sudden cardiac deaths in patients with sickle cell disease is unknown. We speculate that chronic anemia and resulting sub-acute cardiac ischemia may be associated with ventricular repolarization defects leading to prolonged QTc interval. These data provide the rationale for mechanistic studies of prolonged QTc interval and its potential relationship to sudden cardiac death in patients with sickle cell disease. Disclosures: No relevant conflicts of interest to declare.

Low‐risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease

2019 ◽  
Vol 66 (6) ◽  
pp. e27667 ◽  
Author(s):  
Elena María Rincón‐López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández‐Sampelayo Matos ◽  
Jesús Saavedra‐Lozano ◽  
Yurena Aguilar de la Red ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Low Risk ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Severe Bacterial Infection

Severity of Sickle Cell Disease: Modeling Interrelationships among Hemolysis, Pulmonary Hypertension and Risk of Death.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 786-786
Author(s):  
Paola Sebastiani ◽  
Vikki G. Nolan ◽  
Clinton T. Baldwin ◽  
Maria M. Abad-Grau ◽  
Ling Wang ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Clinical Severity ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Risk Of Death

Abstract A single point mutation in the β hemoglobin gene causes sickle cell disease (SCD), but patients have extremely variable phenotypes. Hemolysis-related complications include pulmonary hypertension (PHT), priapism, stroke and leg ulceration; blood viscosity and sickle vasoocclusion are associated with painful episodes, acute chest syndrome and osteonecrosis. Predicting who is at highest risk of death would be useful therapeutically and prognostically. Applying Bayesian network modeling that describes complex interactions among many variables by factorizing their joint probability distribution into modules, to data from 3380 SCD patients, we constructed a disease severity score (DSS: 0, least severe; 1, most severe), defining severity as risk of death within 5 years. A network of 24 variables described complex associations among clinical and laboratory complications of SCD. The analysis was validated in 140 patients whose SCD severity was assessed by expert clinicians and 210 adults where severity was also assessed by the echocardiographic diagnosis of PHT and death. Information about PHT allowed a comparison of the DSS with the tricuspid regurgitant jet velocity (TRJV), an objective marker of PHT and an independent risk factor for death. DSS and three indices of clinical severity (severity ranking of individuals by expert clinicians; objective measurement of the presence and severity of PHT; risk of prospective death) were correlated. Among living subjects, the median score was 0.57 in 135 patients without PHT, 0.64 in 40 patients with mild PHT and 0.86 in 15 patients with severe PHT. The difference in average score between living patients with and without PHT is significant. The same increasing trend was noticeable in the subjects who died during follow-up: 0.60 in subjects without PHT; 0.68 in subjects with mild PHT; 0.79 in subjects with severe PHT. The utility of the DSS is also supported by the ability to assign a score to subjects for whom the TRJV cannot be measured. Surprisingly, besides known risk factors like renal insufficiency and leukocytosis, we identified the intensity of hemolytic anemia and clinical events associated with hemolytic anemia as contributing to risk for death. Priapism, an excellent reflection of the hemolytic anemia-related complications of SCD, is associated with PHT and its association with death was unexpected. Laboratory variables predictive of disease severity included LDH and reticulocytes that reflect the intensity of hemolytic anemia. Elevated systolic blood pressure increased the odds of death by 3.4, consistent with hypertension as a marker of early death in SCD. Subjects with sickle cell anemia are at greatest risk compared with subjects with sickle cell anemia-α thalassemia and with subjects with HbSC disease. Our model suggests that the intensity of hemolytic anemia, estimated by LDH, reticulocyte count and AST, and shown previously to be associated with PHT, priapism, leg ulceration and possibly stroke, is an important contributor to death. This model can be used to compute a personalized measure of disease severity that might be useful for guiding therapeutic decisions and designing clinical trials.

Plasma Level of NT-Pro-BNP In Children with Sickle Cell Disease Is Associated with Degree of Anemia and Left Ventricular Measures: The PUSH Study

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 948-948 ◽  
Author(s):  
Gregory J. Kato ◽  
Craig Sable ◽  
Gregory Ensing ◽  
Niti Dham ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Logistic Regression ◽  
Sickle Cell Disease ◽  
Regression Model ◽  
Sickle Cell ◽  
Logistic Regression Model ◽  
Clinical Laboratory ◽  
Left Ventricular ◽  
Filling Pressure ◽  
Cell Disease

Abstract Abstract 948 Background: Amino-terminal pro-brain type natriuretic peptide (NT-proBNP) is a widely used clinical laboratory marker of left ventricular stress, although it is also known to be elevated in adults with right ventricular stress due to pulmonary hypertension associated with sickle cell disease (SCD) and other disorders. NT-proBNP is associated with early mortality in adults with SCD. Methods: Using a standard clinical laboratory assay, we measured NT-proBNP in 346 children (median age 12; IQR 7–16 years) with SCD enrolled in the Pulmonary Hypertension and the Hypoxic Response in SCD (PUSH) study, an ongoing, longitudinal and observational multicenter study of children with sickle cell disease. In order to adjust for age, a known confounding factor of NT-proBNP in young children, we compared the characteristics of children with the top quartile of NT-proBNP for each 3-year increment of age with those of children in the remaining three quartiles for that age increment. We examined several factors alone and as part of a logistic regression model. Results: In univariate analyses, high expression of NT-proBNP was associated with lower hemoglobin levels (median 83 vs. 87 gm/L, p=0.0006). The high NT-proBNP group also had a higher hemolytic component, a principal component-derived index of the common properties of the hemolytic markers reticulocyte count, serum lactate dehydrogenase, aspartate aminotransferase and bilirubin (median 0.5 vs. 0.7 relative units, p=0.021). The high NT-proBNP group had a higher prevalence of high left ventricular filling pressure, as indicated by the mitral inflow E wave to tissue Doppler E wave (E/Etdi) above 9.22 (13.1% vs. 2.9%, p<0.001) and left ventricular dilatation (median internal diastolic diameter z-score 2.1 vs. 1.5, p=0.013). There was a trend toward higher prevalence of elevated estimated pulmonary artery pressures as indicated by tricuspid regurgitant velocity of 2.6 m/s or higher (17.1% vs. 10.8%, p=0.14), and higher ferritin (median 339 vs. 199, p=0.15). Children on hydroxyurea treatment had lower median NTproBNP levels (77 vs. 121 pg/mL, p=0.005). In a logistic regression model, hemoglobin (odds ratio 0.7, 95% confidence interval 0.6–0.9, p=0.001) and E/ETdi>9.22 were independently associated with high NT-proBNP levels. Conclusions: After adjustment for age in children with SCD, upper quartile NTproBNP is independently associated with severity of anemia and echocardiographic markers of left ventricular size and diastolic filling pressure. At this age, they have not yet developed the association observed prominently in SCD adults between NT-proBNP and elevated pulmonary arterial pressures. Our data lends support to childhood being considered as a pre-symptomatic phase of pulmonary hypertension; which could be targeted for clinical trials of preventative strategies to prevent adult onset of pulmonary vascular changes associated with higher NT-proBNP and higher TRV. Hydroxyurea therapy is associated in our results from children with SCD with lower NT-proBNP, making it an attractive candidate for a trial in children with SCD to prevent pulmonary hypertension in adulthood. NT-proBNP in childhood SCD remains a marker of left ventricular measures, identifying this additional feature of cardiopulmonary risk. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Sudden cardiac death in athletes

2009 ◽  
Vol 62 (1-2) ◽  
pp. 37-41
Author(s):  
Zdravko Mijailovic ◽  
Zoran Stajic ◽  
Dragan Tavciovski ◽  
Radomir Matunovic
Keyword(s):  
Sudden Cardiac Death ◽  
Sudden Death ◽  
Cardiac Death ◽  
Ventricular Hypertrophy ◽  
Ventricular Tachyarrhythmia ◽  
Left Ventricular ◽  
Young Athletes ◽  
Physical Effort ◽  
Social Community ◽  
Esc Guidelines

The entity of sudden cardiac death in young athletes has been known since the year 490 B.C. when young Greek soldier Phidipides had run the distance from Marathon to Athens and suddenly fell down dead. In the last twenty years, sudden death of famous athletes have attracted huge attention of medical and social community; afterwards both American and European Cardiology Societies started to publish periodically guidelines for preparticipation screening. These guidelines have focused on both identifying athletes with potential cardiovascular risk for sudden death and eligibility conditions for athletes participating in competitive sports. Structural and functional abnormalities causing sudden cardiac death in young athletes have been identified by autopsy-based studies. Unrecognized congenital cardiovascular abnormalities associated with excessive physical effort create background for electrophysiological instability and occurrence of malignant ventricular tachyarrhythmia and consequent death. The most frequent causes of sudden cardiac death in young athletes include hypertrophic cardiomyopathy, anomalies of the coronary arteries and idiopathic left ventricular hypertrophy. Current ACC/AHA & ESC guidelines should be widely used in order to reduce potential sudden cardiac death in young athletes.

scholarly journals Acute Chest Syndrome in Children with Sickle Cell Disease in Italy: Results of a National Survey from the Italian Association of Pediatric Hematology Oncology (AIEOP)

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2301-2301
Author(s):  
Vania Munaretto ◽  
Raffaella Colombatti ◽  
Serena Ilaria Tripodi ◽  
Corti Paola ◽  
Simone Cesaro ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Italian Association ◽  
Pediatric Hematology ◽  
Frequent Event ◽  
Regional Hospitals

Background: Acute Chest Syndrome (ACS) is the second cause of hospitalization in Sickle Cell Disease (SCD), burdened by significant morbidity and mortality. The guidelines regarding management of ACS are sometimes difficult to follow in the real world and the prevention and treatment strategies of ACS are often applied in an uneven manner in the various settings (community care, regional hospitals, reference university centers). Moreover, epidemiology, clinical phenotype and outcomes as well as risk factors could vary in different populations according to ethnicity, genotype or health care system organization. Aims and Methods: A retrospective multicenter observational study was conducted to investigate the epidemiology of ACS and to the evaluate the diagnostic and therapeutic pathways of ACS in children with SCD (age 0-18 years) in the 2013-2018 period, after the publication of the Italian Association of Pediatric Hematology Oncology (AIEOP) Guidelines for the Management of SCD in Childhood in Italy in 2012. Results: 126 children were recruited and 122 included in the analysis, with 208 evaluable episodes of ACS (range: 1-6 episodes /patient) from 11 AIEOP Centers. 73 M, 49 F. Mean age was 10.9 years. 85% patients were of African origin, 92% were HbSS/SB°; mean age at diagnosis of SCD of the entire cohort was 25,3 months (range 0-16,8). 44.2% of patients had more than one episode of ACS during the study period; 37% had had a previous episode before 2013. 58% had comorbidities, mostly respiratory (asthma or allergy). 75% of the patients underwent disease modifying treatment during study period (73% hydroxyurea, 2% chronic transfusion). The seasonality of ACS episodes was important in our country: 75% of episodes occured between October and March. 95% of ACS episodes were secondary to a Vaso-Occlusive Crisis. 76% of the admissions occurred in SCD reference centers, 24% in regional hospitals, but 30% later required transfer to reference centers for worsening of clinical conditions or need of exchange transfusion. The mean length of hospitalization was 9.6 days (range 1-46); one patient died of pneumococcal sepsis; 6 episodes required transfer to the Intensive Care Unit, mechanical ventilation was required in one episode. A good adherence to the AIEOP Guidelines was documented for some aspects: 99% of the patients were hospitalized, 98% performed chest X-ray for the diagnosis of ACS and in 99% antibiotic therapy was started. Others aspects were less satisfactory and in need of improvement: incentive spirometry was only performed in 19% of admissions; oxygen therapy was performed only in 75% of patients even if SatO2 was<95%; transfer to reference centers was not always timely. During 75% of ACS episodes a simple red cell transfusion was required for Hb>8g/dl, while in 16% an exchange transfusion was performed for severe respiratory distress (of these 71% were performed in patients transfered from regional hospitals); 38% required inhaled bronchodilators, 6% steroids. A preliminay evaluation of risk factors for recurrent ACS showed that in our cohort allergy to inhaled allergens (p 0.02) and enuresis (p 0.01) were associated with increased prevalence of recurrent ACS; patients with asthma/wheezing also presented more recurrent ACS compared to patients wihout them (23% vs 13%) but this data did not reach statistical significance. Conclusion: This study represents the first analysis in Italy of ACS, which is confirmed as a frequent event in our cohort, with a significant proportion of patients who experience recurrent ACS. Steps need to be undertaken to improve management of ACS and adherence to the AIEOP guidelines at a national level: stimulate the application of early preventive measures that are still under-utilized, increase the appropriateness of multidisciplinary specialist approach (transfusion specialist, acute care physicians, pneumologists, hematologists) strengthen the dissemination of information through training events for all the Hospitals of the network. Disclosures Colombatti: AddMedica: Consultancy; Global Blood Therapeutics: Consultancy; Novartis: Consultancy.

Association of Pulmonary Hypertension with Sickle Cell Disease Subtypes

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4824-4824
Author(s):  
Alice J. Cohen ◽  
Chaim Tuckman-Vernon
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Hemolytic Anemia ◽  
Sickle Cell ◽  
Doppler Echocardiography ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Hemoglobin C ◽  
Baseline Hemoglobin

Abstract Pulmonary hypertension (PH) is a common complication of sickle cell disease (SD) and a significant cause of morbidity and mortality. PH, measured by Doppler echocardiography and defined as a tricuspid regurgitant jet velocity (TRV) &gt; 2.5 m per second (m/s), is hypothesized to be related to the chronic hemolytic anemia of SD, but causality is unproven. If so, the presence of hemoglobin C, which reduces hemolysis, would be expected to have a reduced likelihood of PH. This study reviewed the prevalence of PH in 3 categories of patients with SD: homozygous S (SS), sickle-beta thalassemia (SB), and SC. Methods: Sickle cell disease patients registered at a state funded community comprehensive care adult sickle cell center were routinely screened for PH by Doppler echocardiography. The presence of PH, the incidence of a related complication, acute chest syndrome (ACS), and baseline hemoglobin (hgb) were reviewed. Results: 16 patients with SC type, 30 with SS and 39 with SB disease underwent screening. The prevalence of PH, ACS and hgb are listed in the table below. Conclusion: SC patients have PH and ACS similar to patients with SS and SB patients. These patients have higher baseline hemoglobin and may have hyperviscosity as a cause of PH and ACS as opposed to hemolytic anemia. Further study of PH and ACS in SC patients is warranted. SC SS SB p value PH 6/16 (38%) 12/40 (40%) 11/39 (28%) p= NS ACS 7/16 (44%) 10/30 (33%) 19/39 (49%) p=NS PH + ACS 4/16 (25%) 5/30 (17%) 4/39 (10%) p=NS ACS in PH patients 4/6 (67%) 5/12 (42%) 4/11 (36%) p-=NS Hgb 10.8 7.89 8.57 p=0.000

Sign in / Sign up

Export Citation Format

Share Document