Echocardiographic Screening of Cardiovascular Status in Pediatric Sickle Cell Disease

Allen, Kiona Y.; Jones, Shannon; Jackson, Tannoa; DeCost, Grace; Stephens, Paul; Hanna, Brian D.; Cohen, Meryl S.; Smith‐Whitley, Kim; Mercer‐Rosa, Laura; Natarajan, Shobha

doi:10.1007/s00246-019-02202-3

Cited by 9 publications

(18 citation statements)

References 27 publications

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“…Some studies have found that a higher TRV cut‐off of 3.0 m/second is more strongly associated with mortality than the 2.5 m/second cut‐off 38,39 . The definition of elevated TRV of ≥2.5 m/second has been adopted for studying children with SCD, 25‐29 although it is not clear that the same definition of elevated TRV would apply to both adults and children. The PUSH study enrolled a non‐SCD control group contemporaneous with the SCD patients, and we utilized this control group to define an elevated TRV to be ≥2.7 m/second, or more than 2 SD above the mean in non‐SCD controls.…”

Section: Discussionmentioning

confidence: 99%

“…30,[35][36][37] Some studies have found that a higher TRV cut-off of 3.0 m/second is more strongly associated with mortality than the 2.5 m/second cut-off. 38,39 The definition of elevated TRV of ≥2.5 m/second has been adopted for studying children with SCD, [25][26][27][28][29] although it is not clear that the same definition of elevated TRV would apply to both adults and children. [41][42][43] Serum ferritin also increases with inflammation or with hepatocellular damage in the absence of increased iron stores.…”

Section: Discussionmentioning

confidence: 99%

“…Most studies of children and adolescents with SCD have defined an elevated TRV to be ≥2.5 m/second, [25][26][27][28][29] but this cut-off represents 2 SD above the mean value in adult rather than pediatric non-SCD controls. 30 For the present study, we performed echocardiograpy in 75 control children without SCD, 67 of whom had a measurable TRV.…”

Section: Definition Of Elevated Trv In Scd Patientsmentioning

confidence: 99%

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Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

et al. 2020

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In the US, mortality in sickle cell disease (SCD) increases after age 18‐20 years. Biomarkers of mortality risk can identify patients who need intensive follow‐up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3‐20 years into an observational study in 2006‐2010 and followed 497 patients for a median of 88 months (range 1‐105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18 years was 99% and to 25 years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ≥2.7 m/second (>2 SD above the mean in age‐matched and gender‐matched non‐SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P = .012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ≥2000 μg/L (observed in 60% of patients who died vs 7.8% of survivors, P < .001), forced expiratory volume in 1 minute to forced vital capacity ratio (FEV1/FVC) <0.80 (71.4% of patients who died vs 18.8% of survivors, P < .001), and neutrophil count ≥10x109/L (30.0% of patients who died vs 7.9% of survivors, P = .018). In SCD children, adolescents and young adults, steady‐state elevations of TRV, ferritin and neutrophils and a low FEV1/FVC ratio may be biomarkers associated with increased risk of death.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Definition Of Elevated Trv In Scd Patientsmentioning

confidence: 99%

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Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

et al. 2020

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“…As seen in our study, mitral Doppler indices were no different between SCD patients and controls. A recent study of routine pediatric SCD echocardiogram found that mildly elevated RV pressure and LV dilation were associated with some laboratory markers such as lactate dehydrogenase, Hb, retic count, and bilirubin 35 . In this study, RV pressure was estimated by tricuspid regurgitant jet velocity, which was only reliably obtained in 60% of the patients.…”

Section: Discussionmentioning

confidence: 80%

Assessing the atria in pediatric sickle cell disease: Beyond the dilation

et al. 2020

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Background Diastolic dysfunction (DD) and pulmonary hypertension (PH) are common causes of mortality for sickle cell disease (SCD) patients in developed countries. We hypothesized that left and right atrial strain (LAS‐Ɛ, RAS‐rƐ) are decreased in SCD adolescents, and that worsening values correlate with laboratory markers of disease severity. Methods Prospective cohort study of patients with HbSS genotype of SCD was compared with healthy controls. LAS and RAS were measured from 4‐ and 2‐chamber views by a blinded reader. Peak strain and strain rate values were obtained for atrial contraction (ac), reservoir (res), and conduit (con) phases. Mitral/tricuspid Doppler velocities, left atrial volume, right atrial area were obtained. Laboratory variables were obtained from the electronic record with the three prior values being averaged. Differences in variables were assessed with Wilcoxon rank sum test, and correlations assessed with Spearman's coefficient. Results There were 33 SCD patients compared to 35 healthy controls of similar age, gender, and size. SCD patients had increased left atrial volume and right atrial area. For LAS, Ɛres was significantly lower in SCD patients. For RAS, RƐcon was significantly lower. Neither measurement correlated with clinical markers. The majority of SCD patients had relatively normal atrial strain values. Those with markedly lower values had similar atrial size. Conclusions A sub‐set of SCD patients have markedly low Ɛres and rƐcon. No correlation with clinical markers was identified. Larger, longitudinal studies may determine utility of atrial strain as a screening tool in this at‐risk population.

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“…Of CXRs within the study, 12·8% showed signs of cardiomegaly or pulmonary congestion, potentially indicating the presence of functional heart disease, despite the young mean age of 12·8 years. Multiple studies 23,24 highlight the need for further workup in these children; Dasgupta et al 23 . have shown a positive predictive value of 31% for cardiomegaly on CXR, predicting subsequent heart disease in children.…”

Section: Discussionmentioning

confidence: 99%

What is the role of chest X‐ray imaging in the acute management of children with sickle cell disease?

et al. 2021

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Children with sickle cell disease (SCD) frequently present to hospital acutely unwell and are often exposed to diagnostic chest X-rays (CXRs). Little evidence exists to determine when CXRs are clinically useful. Using electronic hospital records, we audited CXR use in children aged 0-18 who presented to hospital over the past 10 years in both an inpatient and emergency department setting. From a total of 915 first CXRs, only 28Á2% of CXRs (n = 258) had clinically significant findings that altered management or final diagnosis. Of these abnormalities, consolidation represented 52Á3%, effusion 8Á9%, cardiomegaly 8Á4% and sickle cell-related bone changes 6Á3%. Indications for CXR of respiratory distress (OR = 3Á74, 95% CI 2Á28-6Á13), hypoxia (OR = 1Á86, 95% CI 1Á50-2Á31) and cough (OR = 1Á64, 95% CI 1Á33-2Á02), were more likely to have significant CXR findings. Patients who had higher peak fever (38Á4°C vs. 37Á4°C, P = 0Á001), higher peak CRP (156Á4 vs. 46Á1, P < 0Á001) and higher WCC (20Á2 vs. 13Á6, P < 0Á001) were more likely to have clinically significant abnormalities on CXR. We found a decision tool using either hypoxia, cough, respiratory distress, T > 38°C, CRP > 50 or WCC > 15 × 10 9 /l as indications for CXR, to have a sensitivity of 88% (with 95% CI 0Á78-0Á95) and specificity of 46% (95% CI 0Á43-0Á50) for clinically significant findings.

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Echocardiographic Screening of Cardiovascular Status in Pediatric Sickle Cell Disease

Cited by 9 publications

References 27 publications

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study

Assessing the atria in pediatric sickle cell disease: Beyond the dilation

What is the role of chest X‐ray imaging in the acute management of children with sickle cell disease?

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