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  • 1
    In: Journal of the American College of Cardiology, Elsevier BV, Vol. 76, No. 25 ( 2020-12), p. 2982-3021
    Type of Medium: Online Resource
    ISSN: 0735-1097
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    Language: English
    Publisher: Elsevier BV
    Publication Date: 2020
    detail.hit.zdb_id: 1468327-1
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  • 2
    In: Journal of the American Heart Association, Ovid Technologies (Wolters Kluwer Health), Vol. 10, No. 18 ( 2021-09-21)
    Abstract: The natural history of latent rheumatic heart disease (RHD) detected by echocardiography remains unclear. We aimed to assess the accuracy of a simplified score based on the 2012 World Heart Federation criteria in predicting mid‐term RHD echocardiography outcomes in children from 4 different countries. Methods and Results Patient‐level baseline and follow‐up data of children with latent RHD from 4 countries (Australia, n=62; Brazil, n=197; Malawi, n=40; New Zealand, n=94) were combined. A simplified echocardiographic scoring system previously developed from Brazilian and Ugandan cohorts, consisting of 5 point‐based variables with respective weights, was applied: mitral valveanterior leaflet thickening (weight=3), excessive leaflet
tip motion (3), regurgitation jet length ≥2 cm (6), aortic valve
focal thickening (4), and any regurgitation (5). Unfavorable outcome was defined as worsening diagnostic category, persistent definite RHD or development/worsening of valve regurgitation/stenosis. The score model was updated using methods for recalibration. 393 patients (314 borderline, 79 definite RHD) with median follow‐up of 36 (interquartile range, 25–48) months were included. Median age was 14 (interquartile range, 11–16) years and secondary prophylaxis was prescribed to 16%. The echocardiographic score model applied to this external population showed significant association with unfavorable outcome (hazard ratio, 1.10; 95% CI, 1.04–1.16; P =0.001). Unfavorable outcome rates in low (≤5 points), intermediate (6–9), and high‐risk (≥10) children at 3‐year follow‐up were 14.3%, 20.8%, and 38.5% respectively ( P 〈 0.001). The updated score model showed good performance in predicting unfavorable outcome. Conclusions The echocardiographic score model for predicting RHD outcome was updated and validated for different latent RHD populations. It has potential utility in the clinical and screening setting for risk stratification of latent RHD.
    Type of Medium: Online Resource
    ISSN: 2047-9980
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2021
    detail.hit.zdb_id: 2653953-6
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  • 3
    In: The American Journal of Cardiology, Elsevier BV, Vol. 105, No. 10 ( 2010-05), p. 1465-1469
    Type of Medium: Online Resource
    ISSN: 0002-9149
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    Language: English
    Publisher: Elsevier BV
    Publication Date: 2010
    detail.hit.zdb_id: 2019595-3
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  • 4
    In: Circulation, Ovid Technologies (Wolters Kluwer Health), Vol. 115, No. 23 ( 2007-06-12), p. 3015-3038
    Abstract: The intent of this review is to provide the clinician with a summary of what is currently known about the contribution of genetics to the origin of congenital heart disease. Techniques are discussed to evaluate children with heart disease for genetic alterations. Many of these techniques are now available on a clinical basis. Information on the genetic and clinical evaluation of children with cardiac disease is presented, and several tables have been constructed to aid the clinician in the assessment of children with different types of heart disease. Genetic algorithms for cardiac defects have been constructed and are available in an appendix. It is anticipated that this summary will update a wide range of medical personnel, including pediatric cardiologists and pediatricians, adult cardiologists, internists, obstetricians, nurses, and thoracic surgeons, about the genetic aspects of congenital heart disease and will encourage an interdisciplinary approach to the child and adult with congenital heart disease.
    Type of Medium: Online Resource
    ISSN: 0009-7322 , 1524-4539
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2007
    detail.hit.zdb_id: 1466401-X
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  • 5
    In: Open Heart, BMJ, Vol. 9, No. 2 ( 2022-12), p. e002160-
    Abstract: Screening programmes using echocardiography offer opportunity for intervention through identification and treatment of early (latent) rheumatic heart disease (RHD). We aimed to compare two methods for classifying progression or regression of latent RHD: serial review method and blinded, side-by-side review. Methods A four-member expert panel reviewed 799 enrolment (in 2018) and completion (in 2020) echocardiograms from the GOAL Trial of latent RHD in Uganda to make consensus determination of normal, borderline RHD or definite RHD. Serial interpretations (enrolment and completion echocardiograms read at two different time points, 2 years apart, not beside one another) were compared with blinded side-by-side comparisons (enrolment and completion echocardiograms displayed beside one another in random order on same screen) to determine outcomes according to prespecified definitions of disease progression (worsening), regression (improving) or no change. We calculated inter-rater agreement using Cohen’s kappa. Results There were 799 pairs of echocardiogram assessments included. A higher number, 54 vs 38 (6.8% vs 4.5%), were deemed as progression by serial interpretation compared with side-by-side comparison. There was good inter-rater agreement between the serial interpretation and side-by-side comparison methods (kappa 0.89). Disagreement was most often a result of the difference in classification between borderline RHD and mild definite RHD. Most discrepancies between interpretation methods (46 of 47, 98%) resulted from differences in valvular morphological evaluation, with valves judged to be morphologically similar between enrolment and final echocardiograms when compared side by side but classified differently on serial interpretation. Conclusions There was good agreement between the methods of serial and side-by-side interpretation of echocardiograms for change over time, using the World Heart Federation criteria. Side-by-side interpretation has higher specificity for change, with fewer differences in the interpretation of valvular morphology, as compared with serial interpretation.
    Type of Medium: Online Resource
    ISSN: 2053-3624
    Language: English
    Publisher: BMJ
    Publication Date: 2022
    detail.hit.zdb_id: 2747269-3
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  • 6
    In: Journal of the American Heart Association, Ovid Technologies (Wolters Kluwer Health), Vol. 5, No. 11 ( 2016-10-26)
    Type of Medium: Online Resource
    ISSN: 2047-9980
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2016
    detail.hit.zdb_id: 2653953-6
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  • 7
    In: Blood, American Society of Hematology, Vol. 118, No. 21 ( 2011-11-18), p. 515-515
    Abstract: Abstract 515 Background: Recent studies indicate that the disease-specific mortality In sickle cell anemia is about 6% in children up to 18 years and 15% in the 18–30 year age group, yielding a cumulative mortality of 21% by age 30 years. It is important to identify children at high risk so that early interventions can be developed to reduce this high mortality. Methods: We prospectively enrolled 505 children and adolescents with sickle cell disease in 2005–2010, 380 with hemoglobin SS and 130 with other genotypes. The median age at enrollment was 12 years with a range of 3 to 20 years. Baseline clinical features, echocardiography, six-minute walk test and pulmonary function testing were performed at steady-state. Follow-up for mortality has been performed in 470 of the participants at a median of 37 months after enrollment, range of 1 to 59 months. Results: Six of 470 patients (1.3%) died during the follow-up period, five with hemoglobin SS and one with hemoglobin SC. The median age at the time of death in these six participants was 20 years, range of 15 to 23 years. Death occurred during the follow-up period in 2.7% of participants over 12 years of age at enrollment and 3.7% of those over 15 years of age. The causes of death were stroke in 4, multiorgan failure in 1 and unknown in 1. Death occurred in 5.9% of 51 participants with a history of stroke versus 0.7% of 416 without stroke history; in 3.5% of 113 participants with a history of asthma versus 0.6% of 354 without asthma history; in 4.9% of 103 participants with 10 or more blood transfusions lifetime versus 0.3% of 359 with less than 10 blood transfusions; in 3.3% of 90 participants with two or more severe pain episodes in the past year versus 0.8% of 380 participants with less than two severe pain episodes in the past year. In age-adjusted analyses, the hazards ratio (95% CI) of death was 6.1 (1.2-30.5) for history of stroke (P=0.029), 10.2 (1.2-89.5) for history of frequent blood transfusions (P=0.036), 5.8 (1.1-31.8) for history of asthma (P=0.044) and 1.07 (1.00-1.14) for frequent severe pain episodes (P=0.047). Clinical findings associated with these risk factors included higher concentrations of markers of hemolysis for history of stroke and history of frequent blood transfusions, decreased FEV1/FVC and increased total lung capacity for history of asthma, and lower concentrations of markers of hemolysis and high ECHO-determined tricuspid regurgitation velocity for history of frequent severe pain episodes. Conclusions: Over a median of three years of observation of this cohort, no deaths occurred among 248 sickle cell disease children 12 years of age or younger at enrollment but there were 6 deaths among 222 participants 13–20 years of age at enrollment. In bi-variate age-adjusted analyses, histories of stroke, asthma, frequent blood transfusions and frequent pain episodes were associated with an increased risk of death. Strikingly, four of the five deaths in which the cause was known were due to stroke. The present data on mortality in the PUSH study suggest that prevention of stroke is critical in improving the survival in adolescents and young adults with sickle cell disease. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2011
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 8
    In: Blood, American Society of Hematology, Vol. 114, No. 21 ( 2009-11-19), p. 4639-4644
    Abstract: Hydroxyurea and higher hemoglobin F improve the clinical course and survival in sickle cell disease, but their roles in protecting from pulmonary hypertension are not clear. We studied 399 children and adolescents with sickle cell disease at steady state; 38% were being treated with hydroxyurea. Patients on hydroxyurea had higher hemoglobin concentration and lower values for a hemolytic component derived from 4 markers of hemolysis (P ≤ .002) but no difference in tricuspid regurgitation velocity compared with those not receiving hydroxyurea; they also had higher hemoglobin F (P 〈 .001) and erythropoietin (P = .012) levels. Hemoglobin F correlated positively with erythropoietin even after adjustment for hemoglobin concentration (P 〈 .001). Greater hemoglobin F and erythropoietin each independently predicted higher regurgitation velocity in addition to the hemolytic component (P ≤ .023). In conclusion, increase in hemoglobin F in sickle cell disease may be associated with relatively lower tissue oxygen delivery as reflected in higher erythropoietin concentration. Greater levels of erythropoietin or hemoglobin F were independently associated with higher tricuspid regurgitation velocity after adjustment for degree of hemolysis, suggesting an independent relationship of hypoxia with higher systolic pulmonary artery pressure. The hemolysis-lowering and hemoglobin F–augmenting effects of hydroxyurea may exert countervailing influences on pulmonary blood pressure in sickle cell disease.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2009
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 9
    Online Resource
    Online Resource
    American Society of Hematology ; 2006
    In:  Blood Vol. 108, No. 11 ( 2006-11-16), p. 1210-1210
    In: Blood, American Society of Hematology, Vol. 108, No. 11 ( 2006-11-16), p. 1210-1210
    Abstract: Background Studies in adults with sickle cell disease (SCD) suggest an increased prevalence of pulmonary artery hypertension (PAHTN) with associated increased morbidity and mortality. These findings have not been validated in children. Methods The digital echocardiography (echo) database at Children’s National Medical Center was searched for echos performed on patients with SCD from 1999 to 2006. Patients on chronic transfusions undergo regularly scheduled studies; other patients are referred for a variety of indications including murmurs, cardiomegaly, hypoxia, chest pain, and respiratory distress. Echo reports and digital images were reviewed and the following information was recorded: Chronic transfusion, date of study, blood pressure, cardiac structure, ventricular function, presence of tricuspid regurgitation (TR), and velocity of TR. The estimated right ventricular (RV) pressure was calculated using modified Bernoulli equation (adding 5 mm Hg for estimated right atrial pressure if the inferior vena cava was not dilated). The ratio of RV systolic pressure to systemic systolic blood pressure was calculated. All studies with TR velocities of & lt; 3 m/sec or a RV to systemic ratio of less than one third were considered to be normal. Results: The SCD database has 1060 patients ages 0 to 21 years; 166 echos were performed in 104 routine patients and 72 echos in 47 children while on chronic transfusions. TR could be measured in 56 routine and 20 transfusion studies. Comorbidities were present in 11 studies (7 patients) in the routine group: structural heart disease (4 ), constrictive pericarditis (1 ), significant obstructive airway disease (1 ), and pneumonia requiring mechanical ventilation (1 ). There were no comorbidities in the transfusion group. The mean TR velocity, estimated RV pressure, and RV to systemic pressure ratios were higher in the routine group; however when patients with comorbidities were removed, this difference was no longer statistically significant. Routine (n=55) Routine/no comorbidities (n=44) Transfused (n=20) 1p = 0.01 transfused vs. routine; p = 0.11 transfused vs. routine/no comorbidities 2p & lt; 0.01 transfused vs. routine; p = 0.09 transfused vs. routine no comorbidities 3p = 0.01 transfused vs. routine; p = 0.13 transfused vs. routine/no comorbidities 1TR velocity (m/sec) 2.54 ± 0.48 2.30 ± 0.30 2.30 ± 0.30 Range 1.67–3.93 Range 1.67–3.19 Range 1.70–2.76 2RV pressure (mm Hg) 31.8 ±10.5 29.2 ± 6.6 26.5 ± 5.6 Range 16.2–67.8 Range 16.2–45.7 Range 16.6–36.4 3RV/systemic pressure ratio 0.29 ± 0.11 0.26 ± 0.064 0.23 ± 0.065 Range 0.16–0.78 Range 0.16–0.41 Range 0.14–0.38 There were 6 routine studies (2 when patients with comorbidities were removed) and no transfusion studies with a TR velocity above 3.0 m/sec. There were 23 routine studies (18 when patients with comorbidities removed) and 6 transfusion studies with a TR velocity between 2.5 and 3.0 m/sec. The ratio of RV to systemic pressure was greater than one third in 11 routine patients (5 when patients with comorbidities were removed) and 1 transfusion patient. Conclusions PAHTN is present in a small number of children with SCD and may be exacerbated by other medical problems. Chronic transfusion may be protective against PAHTN. Prospective analysis of this population is warranted.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2006
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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  • 10
    In: Blood, American Society of Hematology, Vol. 112, No. 11 ( 2008-11-16), p. 1423-1423
    Abstract: Background. As a part of a multicenter, observational study in determining the prevalence and risk factors of elevated tricuspid regurgitant jet velocity (TRV) in children, oxygen desaturation correlated with TRV. We further investigated the risk factors and clinical associations of oxygen desaturation at rest and after execrcise in children at steady state. Methods. 310 children and adolescents with sickle cell disease were studied under basal conditions. Pulse oximetry was determined at rest and after a six minute walk test. The relationships of oxygen saturation at rest and desaturation during exercise to the available clinical and laboratory variables were investigated. Results. Among 300 patients with available baseline oxygen saturations, 30 (10%) had saturation & lt;95 percent, 129 (43%) had saturation of 95–98 percent, and 141 (47%) had saturation & gt;98 percent. Twenty-three (9%) of 244 patients had & gt;3 percentage point reduction in oxygen saturation during a six minute walk; the median (interquartile range) baseline saturation was 96 (95– 99) percent among these patients versus 98 (97–100) percent among those with less or no reduction in saturation during the walk (P = 0.0009). Hemoglobin (p & lt;0.0001), creatinine (p=0.014) and total lung capacity (p=0.042) were also lower in patients with declines in saturations & gt;3 percentage points during the walk while a hemolytic index (p & lt;0.0001), tricuspid regurgitant jet velocity (p=0.030), pulmonary insufficiency end diastolic velocity (PIEDV) (p=0.019), left ventricular mass index (LVMI) (p=0.0006) and left ventricular internal diameter z score (LVIDD z score) (p=0.0001) were higher. In 3 separate logistic regression models (clinical variables, echocardiographic parameters, and pulmonary function testing), lower hemoglobin, hemolytic index, PIEDV, LVIDD z score, and low TLC were independent predictors of six-minute-walk desaturation of & gt;3 percentage points. Conclusion. Markers of hemolysis, low hemoglobin, PIEDV, LVMI, LVIDD z score, lower TLC, and elevated TRV velocities are associated with ≥3% reduction in oxygen desaturation during six minute walk in children and adolescents with sickle cell disease. A high degree of oxygen desaturation during the six minute walk in sickle cell disease patients might serve as an early biomarker for pulmonary hypertension. Exercise induced changes in oxygen saturation in sickle cell disease children may provide insight into the development of pulmonary hypertension as adults.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
    RVK:
    RVK:
    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2008
    detail.hit.zdb_id: 1468538-3
    detail.hit.zdb_id: 80069-7
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