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Elevated tricuspid regurgitant velocity (TRV) ≥2.5 m/s is a predictor of disease severity in adults and children with sickle cell anemia (SCA), but how disease-modifying therapies (DMTs) affect this biomarker is incompletely understood. We investigated the effect of DMTs on TRV elevation in children. In a prospective single-center study, 204 subjects with HbSS or HbSβ0 thalassemia (mean age, 10.6 years; range, 5-18) had echocardiograms with assessment of TRV, with repeat evaluations after 2 years of observation. One-hundred and twelve participants received DMTs (hydroxyurea, n = 72; monthly erythrocyte transfusions, n = 40), 58 did not receive any DMT, and 34 were begun on hydroxyurea during this observation period. In the entire cohort, an increase in hemoglobin of 1.0 g/dL was associated with a 0.03-m/s decrease in TRV (P = .024), and a decrease in absolute reticulocyte count of 1.0 × 106/mL was associated with a 0.34-m/s decrease in TRV (P = .034). Compared with baseline, hydroxyurea exposure (continuous or newly started) was associated with an average 5% decline in mean TRV at the 2-year evaluation. Among participants newly started on hydroxyurea (mean treatment duration 1.2 ± 0.6 years), an increase in hemoglobin of 1.0 g/dL was associated with a 0.06-m/s decrease in TRV (P = .05). We conclude that hydroxyurea therapy may mitigate TRV elevation in children with SCA, possibly as a result of a reduction in hemolysis and improvement in anemia.

Elevated tricuspid regurgitant velocity (TRV) ≥2.5 m/s is a predictor of disease severity in adults and children with sickle cell anemia (SCA), but how disease-modifying therapies (DMTs) affect this biomarker is incompletely understood. We investigated the effect of DMTs on TRV elevation in children. In a prospective single-center study, 204 subjects with HbSS or HbSβ0thalassemia (mean age, 10.6 years; range, 5-18) had echocardiograms with assessment of TRV, with repeat evaluations after 2 years of observation. One-hundred and twelve participants received DMTs (hydroxyurea, n = 72; monthly erythrocyte transfusions, n = 40), 58 did not receive any DMT, and 34 were begun on hydroxyurea during this observation period. In the entire cohort, an increase in hemoglobin of 1.0 g/dL was associated with a 0.03-m/s decrease in TRV (P= .024), and a decrease in absolute reticulocyte count of 1.0 × 106/mL was associated with a 0.34-m/s decrease in TRV (P= .034). Compared with baseline, hydroxyurea exposure (continuous or newly started) was associated with an average 5% decline in mean TRV at the 2-year evaluation. Among participants newly started on hydroxyurea (mean treatment duration 1.2 ± 0.6 years), an increase in hemoglobin of 1.0 g/dL was associated with a 0.06-m/s decrease in TRV (P= .05). We conclude that hydroxyurea therapy may mitigate TRV elevation in children with SCA, possibly as a result of a reduction in hemolysis and improvement in anemia.

Cancer survivors who have undergone hematopoietic cell transplantation (HCT) are at risk for myocardial dysfunction. Children who receive allogenic HCT encounter systemic inflammation resulting in tachycardia and hypertension. The effect of these abnormalities on myocardial function is not known. The aim of this study was to determine whether cardiac dysfunction early after HCT can be predicted by tachycardia or hypertension, within a retrospective single-center sample of pediatric HCT recipients. Early tachycardia or hypertension was defined as a majority of values taken from infusion date to 90 days post-infusion being abnormal. Ejection fraction <53% determined systolic dysfunction. A composite score of accepted pediatric diastolic abnormalities determined diastolic dysfunction. Among 80 subjects (median age 8 years), early tachycardia, systolic dysfunction, and diastolic dysfunction were present in 64%, 25%, and 48% of the sample, respectively. In multivariable models, early tachycardia was an independent predictor of early systolic dysfunction (OR = 12.6 [1.4–112.8], p= 0.024) and diastolic dysfunction (OR = 3.9 [1.3–11.5], p= 0.013). Tachycardia and cardiac dysfunction are common and associated with one another in the early period after pediatric HCT. Future studies may elucidate the role of tachycardia and myocardial dysfunction early after HCT as important predictors of future cardiovascular dysfunction.

Despite increasing continuous-flow ventricular assist device (CF-VAD) use in children, minimal data exist regarding the functional recovery and rehabilitation potential after device placement. We hypothesized that after CF-VAD implantation, children would demonstrate a time-limited improvement in 6 minute walk distance (6MWD) and brain-type natriuretic peptide (BNP). A retrospective cohort study of 27 patients was conducted, those <18 years of age at a tertiary-care center during the study period. Seventy-four percent were male; median age was 12.7 years. Six minute walk distance and BNP were evaluated within 365 days of implantation. Associations were examined before and after 90 days postimplantation because a plateau in both values was seen after 90 days. Data included 92 6MWD and 341 BNP values. In the first 90 days, 6MWD increased by 12 percent predicted (%P) per 30 days (P< 0.01); with no significant change thereafter, increasing 0.6 %P per 30 days (P= 0.482). In the first 90 days, BNP decreased by 59% per 30 days (P< 0.01); with no significant change thereafter, increasing 1.2% per 30 days (P= 0.561). Six minute walk distance and BNP improved after CF-VAD implantation, with a significant improvement only in the first 90 days. Routine use of 6MWD and BNP can help in assessment of functional recovery in children after CF-VAD placement.

We describe the long‐term follow‐up of a child with recurrent hemoptysis due to severe pulmonary vein stenosis decompressing via collaterals to esophageal varices. Case report Tertiary children's hospital Single child through ages 2‐ to 11‐year old The child underwent cutting balloon angioplasty, bare metal stenting, and implantation of a PTFE‐covered stent, all of which failed rapidly. Only after placement of a paclitaxel drug eluting stent did he have prolonged relief from hemoptysis and long‐term patency of the treated vein. The stents were serially dilated to keep up with somatic growth of the child, eventually culminating in the need to induce intentional stent fracture. We highlight novel transcatheter techniques to treat this vexing condition, discuss mechanisms of disease treatment and progression, and present the only patient with this rare combination of lesions to have achieved both longstanding pulmonary vein patency and resolution of esophageal varices.

Anemia is common among adult heart failure patients and is associated with adverse outcomes, but data are lacking in children with heart failure. The purpose of this study was to determine the prevalence of anemia in children hospitalized with acute heart failure and to evaluate the association between anemia and adverse outcomes. Review of the medical records of 172 hospitalizations for acute heart failure. Single, tertiary children's hospital. All acute heart failure admissions to our institution from 2007 to 2012. None. Composite endpoint of death, mechanical circulatory support deployment, or cardiac transplantation. Patients ages ranged in age from 4 months to 23 years, with a median of 7.5 years, IQR 1.2, 15.9. Etiologies of heart failure included: dilated cardiomyopathy (n= 125), restrictive cardiomyopathy (n= 16), transplant coronary artery disease (n = 18), ischemic cardiomyopathy (n= 7), and heart failure after history of congenital heart disease (n = 6). Mean hemoglobin concentration at admission was 11.8 g/dL (±2.0 mg/dL). Mean lowest hemoglobin prior to outcome was 10.8 g/dL (±2.2 g/dL). Anemia (hemoglobin <10 g/dL) was present in 18% of hospitalizations at admission and in 38% before outcome. Anemia was associated with increased risk of death, transplant, or mechanical circulatory support deployment (adjusted odds ratio 1.79, 95% confidence interval = 1.12–2.88, P= .011). For every 1 g/dL increase in the patients' lowest hemoglobin during admission, the odds of death, transplant, or mechanical circulatory support deployment decreased by 18% (adjusted odds ratio = 0.82, 95% confidence interval = 0.74–0.93, P = 0.002). Anemia occurs commonly in children hospitalized for acute heart failure and is associated with increased risk of transplant, mechanical circulatory support, and inhospital mortality.

Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of “simple” lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long‐term complications after these repairs, prompting the need for continued follow‐up. This review describes the current understanding of diagnosis, treatment, and long‐term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long‐term.

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