Pediatric Heart Failure Patients Research Articles

Sacubitril-valsartan vs ACE/ARB in pediatric heart failure: A retrospective cohort study

The first angiotensin receptor/neprilysin inhibitor on the market, sacubitril-valsartan, has shown marked improvements in death and hospitalization for heart failure among adults, and is now approved for use in pediatric heart failure. While the ongoing PANORAMA-HF trial is evaluating the effectiveness of sacubitril-valsartan for pediatric patients with a failing systemic left ventricle, the enrollment criteria do not include the majority of pediatric heart failure patients. Additional studies are needed. Using the TriNetX database, we performed a propensity score matched, retrospective cohort study to assess the incidence of a composite of all-cause mortality or heart transplant within 1year. The 519 patients who received sacubitril-valsartan were compared to 519 matched controls who received an angiotensin converting enzyme inhibitor (ACE) or angiotensin II receptor blocker (ARB). There was no significant difference in the incidence of the composite outcome with sacubitril-valsartan over an ACE/ARB (13.3% vs 13.2%, p=0.95), or among the components of mortality (5.0% vs 5.8%, p=0.58) or heart transplantation (8.7% vs 7.5%, p=0.50). Patients who were receiving full goal-directed medical therapy (14.4% vs 16.0%, p=0.55) also showed no difference in the composite outcome. We observed a significantly increased incidence of hypotension (10% vs 5.2%, p=0.006) and a trend toward reduced number of hospitalizations per year (mean (SD) 1.3 (4.4) vs 2.0 (9.1), p=0.09). Sacubitril-valsartan is not associated with a decrease in the composite of all-cause mortality or heart transplantation within 1year. Future studies should evaluate the possible reduction in hospitalizations and optimal dosing to minimize hypotension.

Development of the PSU Child Pump.

The Pennsylvania State University (PSU) Child Pump, a centrifugal continuous-flow ventricular assist device (cf-VAD), is being developed as a suitable long-term implantable device for pediatric heart failure patients between 10 and 35 kg, body surface area (BSA) of 0.5-1.2 m2, 1-11 years of age, and requiring a mean cardiac output of 1.0-3.5 L/min. In-vitro hydraulic and hemodynamic performances were evaluated on a custom mock circulatory loop with ovine blood. Normalized index of hemolysis (NIH) was evaluated under four conditions: 1) 8,300 rpm, 3.5 L/min, ΔP = 60 mm Hg, 2) 8,150 rpm, 5.1 L/min, ΔP = 20 mm Hg, 3) 8,400 rpm, 3.2 L/min, ΔP = 70 mm Hg, and 4) 9,850 rpm, 5.0 L/min, ΔP = 80 mm Hg, resulting in normalized index of hemolysis = 0.027 ± 0.013, 0.015 ± 0.006, 0.016 ± 0.008, and 0.026 ± 0.011 mg/dl, respectively. A mock fit study was conducted using a three-dimensional printed model of a 19 kg patient's thoracic cavity to compare the size of the PSU Child Pump to the HeartMate3 and the HVAD. Results indicate the PSU Child Pump will be a safer, appropriately sized device capable of providing the given patient cohort proper support while minimizing the risks of blood trauma as they wait for a transplant.

Direct comparison of the diagnostic performance of growth differentiation factor 8 in pediatric versus adult heart failure

IntroductionGrowth differentiation factor 8 (GDF-8, myostatin) has been proposed for the management of adult heart failure (HF). Its potential role in pediatric HF patients is unknown. We sought to investigate its diagnostic performance in adult versus pediatric HF. MethodsGDF-8 was measured prospectively in pediatric and adult HF patients and in matching controls. HF was defined as the combination of typical symptoms and impaired left ventricular systolic function. Diagnostic performance for the detection of HF was evaluated by receiver operating characteristic (ROC) analysis. ResultsWe enrolled 137 patients with HF (85 pediatric) and 67 healthy controls (47 pediatric).Neither pediatric nor adult HF patients had significantly different GDF-8 levels compared to the reference groups (3.53 vs 3.46 ng/mL, p = 0.334, and 6.87 vs 8.15 ng/mL, p = 0.063, respectively), but pediatric HF patients had significantly lower GDF-8 levels compared to adult patients (p < 0.001). ROC analysis showed no significant improvement adding GDF-8 to NT-proBNP, age and sex (area under the curve (AUC): 0.870 vs 0.868, p = 0.614) in children and neither in addition to age nor sex in adult HF patients (AUC: 0.74 vs 0.62, p = 0.110). ConclusionGDF-8 did not accurately differentiate between HF patients and normal comparators in neither adults nor in children.

IMPACT OF MALNUTRITION ON PEDIATRIC HEART FAILURE ANALYZING Z-SCORES AT PRESENTATION

Pediatric heart failure represents a critical health concern characterized by the heart's inability to effectively pump blood, often resulting from congenital heart defects, myocarditis, and cardiomyopathies. Malnutrition has emerged as a recognized comorbidity in pediatric heart failure, significantly impacting patient outcomes. This study aimed to investigate the relationship between malnutrition and pediatric heart failure by analyzing Z-scores at presentation to guide targeted interventions and improve patient management. The study employed a retrospective observational design and was conducted at the Department of Pediatric Cardiology, Lady Reading Hospital, Peshawar, from January 2023 to January 2024. A total of 764 pediatric patients with heart failure were included in the analysis. Nutritional status was assessed using weight and height measurements, and Z-scores were calculated based on WHO Child Growth Standards. Data analysis was performed using IBM SPSS version 22. Results revealed varying degrees of malnutrition among pediatric heart failure patients, with 62.8% classified as severely malnourished, 28.4% as moderately malnourished, and 8.8% as mildly malnourished. The calculated Z-score of -3.2 indicated significant malnutrition among the study population. Furthermore, a negative correlation (r = -0.32, p &lt; 0.001) between Z-score and left ventricular ejection fraction was observed, suggesting a link between malnutrition severity and cardiac dysfunction. These findings underscore the high prevalence of malnutrition among pediatric heart failure patients and its substantial impact on outcomes. Early nutritional assessment and intervention are crucial for optimizing patient care and enhancing outcomes in children with heart failure. Collaborative, multidisciplinary approaches that integrate cardiac and nutritional management strategies can comprehensively address the diverse needs of this vulnerable population.

First Pediatric HeartMate 3 Ventricular Assist Device Implantation in Romania – a Case Report

Abstract The use of ventricular assist devices as bridge-to-transplantation therapy has a critical role in the management of end-stage heart failure in pediatric patients without available compatible organ donors. The HeartMate 3 is an intracorporeal centrifugal flow pump with a fully magnetically levitated motor currently approved for the management of advanced refractory left ventricular failure in pediatric patients, with positive early outcomes. We report the case of a 17-year-old adolescent girl with end-stage heart failure secondary to dilated cardiomyopathy of idiopathic etiology, with multiple failed attempts of weaning from inotropic support (PEDIMACS 3 profile), who successfully received a HeartMate 3 left ventricular assist device as bridge-to-transplantation therapy with no significant adverse events during the early follow-up period. This paper presents the first case of pediatric ventricular assist device implantation in Romania.

Outcomes of extracorporeal membrane oxygenation support in pediatric hemato-oncology patients.

In this study, we reviewed the outcomes of pediatric patients with malignancies who underwent hematopoietic stem cell transplantation (HSCT) and extracorporeal membrane oxygenation (ECMO). We retrospectively analyzed the records of pediatric hemato-oncology patients treated with chemotherapy or HSCT and who received ECMO in the pediatric intensive care unit (PICU) at Seoul National University Children's Hospital from January 2012 to December 2020. Over a 9-year period, 21 patients (14 males and 7 females) received ECMO at a single pediatric institute; 10 patients (48%) received veno-arterial (VA) ECMO for septic shock (n=5), acute respiratory distress syndrome (ARDS) (n=3), stress-induced myopathy (n=1), or hepatopulmonary syndrome (n=1); and 11 patients (52%) received veno-venous (VV) ECMO for ARDS due to pneumocystis pneumonia (n=1), air leak (n=3), influenza (n=1), pulmonary hemorrhage (n=1), or unknown etiology (n=5). All patients received chemotherapy; 9 received anthracycline drugs and 14 (67%) underwent HSCT. Thirteen patients (62%) were diagnosed with malignancies and 8 (38%) were diagnosed with non-malignant disease. Among the 21 patients, 6 (29%) survived ECMO in the PICU and 5 (24%) survived to hospital discharge. Among patients treated for septic shock, 3 of 5 patients (60%) who underwent ECMO and 5 of 10 patients (50%) who underwent VA ECMO survived. However, all the patients who underwent VA ECMO or VV ECMO for ARDS died. ECMO is a feasible treatment option for respiratory or heart failure in pediatric patients receiving chemotherapy or undergoing HSCT.

Pediatric heart failure classification based on left ventricular ejection fraction

AbstractLeft ventricle ejection fraction (LVEF) is still not well acknowledged in classification of pediatric heart failure (PHF). We categorized PHF according to LVEF and aimed to determine the role of LVEF in PHF classification. Patients who were diagnosed with HF were divided into three groups according to their LVEF values: HF with reduced ejection fraction (HFrEF), HF with mildly reduced ejection fraction (HFmrEF), and HF with preserved ejection fraction (HFpEF). The clinical information of PHF patients was compared among those three groups. Factors associated with HF with improved EF (HFimpEF) and risk factors for in‐hospital death in PHF patients were analyzed. A total number of 1228 cases were collected. The proportion of HF patients with preserved LVEF (66.3%) was significantly higher than those with mildly reduced LVEF (21.7%) and reduced LVEF (12%). Clinical features such as age, B‐type natriuretic peptide (BNP) level, Ross classification, and E/A abnormal proportion in HF children with different LVEF value were statistically different. HF patients with younger age, lower BNP levels, minor cardiac dysfunction and less E/A abnormality could be found with higher LVEF value. The proportion of primary disease in PHF was largely different in HFpEF, HFmrEF and HFrEF groups. Medication treatment was more aggressive in patients with lower LVEF, except for vasoactive drugs. Children with congenital heart disease in HFrEF group were more prone to develop into HFimpEF. Sepsis, renal insufficiency, and an abnormal E/A ratio are risk factors for in‐hospital death of HF children. Clinical features of PHF could be well classified by LVEF, which is an essential and helpful indicator for PHF classification and management.

Does sacubitril/valsartan work in children with heart failure?—a pilot study

BackgroundSacubitril/valsartan is an angiotensin receptor neprilysin antagonist (ARNI) approved for adult heart failure (HF). Its safety and efficacy in pediatric HF patients with cardiomyopathy or congenital heart disease are poorly understood. A pilot study was conducted to assess the clinical response, efficacy and safety of sacubitril/valsartan in this population at a tertiary care hospital in China.MethodsClinical parameters of patients who received sacubitril/valsartan from January 2019 to March 2023 were retrospectively collected and analyzed. Children over 1 month with a left ventricular ejection fraction (LVEF) &amp;lt;45% were included. Clinical efficacy was evaluated by echocardiographic LVEF, N-terminal pro-brain natriuretic peptide (NT-proBNP), New York Heart Association (NYHA) HF classification, HF re-admission, and death or transplantation. The initial dose was either 0.2 mg/kg bid or 0.4 mg/kg bid, with a target dose of 2.3 mg/kg bid or 3.1 mg/kg bid.ResultsForty-five patients (60% male) with a median age of 7.86 years were enrolled. Among them, 23 had congenital heart disease and 22 had cardiomyopathies. The median maintenance dose was 0.76 mg/kg. The primary endpoint of LVEF up to 45% was reached by 24 patients (53.3%). The median NT-proBNP was significantly decreased from 5,501.5 pg/ml to 2,241.5 pg/ml (P &amp;lt; 0.001), more in congenital heart disease than in cardiomyopathies (P = 0.032). The NYHA HF class was improved or remained stable in 42 cases (93.3%). During a median follow-up of 1.23 years, 13 patients (28.9%) were re-hospitalized due to HF, and 9 patients (20%) died or underwent transplantation. Hypotension was the main adverse event, occurring in 8 patients.ConclusionsSacubitril/valsartan may be effective in children with HF, but its safety and outcomes may differ depending on the etiology and anatomy of HF. Early post-operative congenital heart disease patients had less tolerance, more hypotension but better recovery and outcomes, while mid- and late- post-operative congenital heart disease patients and cardiomyopathy patients had less side effects but poorer clinical outcomes.

30- and 60-Day Readmission Rates for Children With Heart Failure in the United States

BackgroundStudies on readmission for pediatric heart failure (HF) patients is sparse. ObjectivesThis study evaluated 30- and 60-day readmission rates in pediatric HF patients from 2010 to 2019. MethodsThe authors used data from the Nationwide Readmission Database to evaluate trends in 30- and 60-day hospital readmissions among pediatric patients with HF and compare them with adults with HF. Readmissions were also stratified by sex, diagnosis, neighborhood income, and hospital volume. ResultsThere were 84,731 hospital admissions for HF. Compared with children without HF, those with HF were older, had Medicare/Medicaid insurance, and resided in micropolitan areas and low-income neighborhoods. The 30- (19.5% vs 3.1%) and 60-day (27.5% vs 4.3%) all-cause readmission rates were higher for children with HF compared with those without HF. Compared with children without HF, lengths of stay, deaths, and costs related to their readmission were higher for children readmitted with HF (P < 0.05 for all). There was no significant decline in pediatric HF-related 30- or 60- day readmissions during the study period overall, or for those with congenital heart disease (P > 0.05), unlike adult HF readmissions (P < 0.01). Infants were at highest risk, and readmission rates for teenagers are rising. ConclusionsThe 30- and 60-day readmission rates for pediatric patients with HF in the current era is high (∼20% and 30%, respectively). Unlike adult HF, pediatric HF readmission rates have not declined. Pediatric HF patients readmitted to the hospital have higher death rates and greater resource utilization than patients without HF. National measures to decrease readmissions for pediatric patients with HF is warranted.

Abstract 11359: Association Between PROMIS Scores and Heart Failure Severity in Children

Introduction: Patient reported outcome (PRO) measures correlate with heart failure (HF) severity among adults and are widely used as adjunct tools in the assessment of adult HF. However, limited data exist in pediatric HF. PROs may be valuable in monitoring heart failure (HF) status in children. Hypothesis: Patient Reported Outcome Measurement Information System (PROMIS) fatigue score correlates with HF severity in children. Methods: In this prospective cohort study, English and Spanish speaking patients were recruited from 4 pediatric HF centers 09/2019 - 02/2023. Children 8.0 to 18.0 years old with systolic and/or diastolic HF were enrolled while receiving inpatient HF treatment. Pediatric self-report PROMIS measures were administered during inpatient treatment and at additional outpatient follow-up time point(s) within 6 months post-discharge. Measures included mobility, fatigue, anxiety, depressive symptoms, and peer relationships. NYU HF Index scores were calculated at each time point. Pearson’s correlation with a 95% confidence interval (CI) compared PROMIS Fatigue T-score and NYU HF Index score at the inpatient time point, and a linear mixed effects model determined their association over time. Results: We enrolled 41 patients with median age of 13.5 ±4.2 years. Patient diagnoses included cardiomyopathy (48.8%), congenital heart disease (41.5%) and other (9.7%). Twenty patients (48.8%) were discharged from hospital alive and without a VAD or heart transplant, of which 18 completed both inpatient and outpatient follow-up assessment. Median PROMIS Fatigue T-scores and NYU HF Index scores improved from inpatient to outpatient assessment. NYU Index scores improved from 12.0 (IQR 11.2, 15.0) to 7.5 (IQR 5.8,10.2); p&lt;0.05. PROMIS fatigue scores improved from 58.5 (IQR 54.6, 64.9) to 50.2 (IQR 33.5, 59.2); p&lt;0.05. The correlation between PROMIS Fatigue T-score and NYU HF Index score at the inpatient time point was 0.5 (95% CI 0.2, 0.7), and their correlation over time was 0.5 (95% CI 0.3, 0.6). Conclusions: PROMIS Fatigue T-score moderately correlates with HF severity in children. Larger scale application of PROMIS could aid in longitudinal symptom management of pediatric HF patients and should be further tested in routine clinical care.

Long term results of pediatric heart transplantations - single center experiences.

Heart failure (HF) is characterized by significant mortality in both adults and children. Characteristic of paediatric HF are feeding problems, poor weight gain, exercise intolerance or dyspnoea. These changes are often accompanied by endocrine disorders. The main causes of HF are congenital heart defects (CHD) cardiomyopathies, arrhythmias, myocarditis or heart failure secondary to oncological treatment. Heart transplantation (HTx) is the method of choice for treatment of end-stage HF in paediatric patients. The aim is to summarize single center experience in heart transplantation in children. Between 1988 and 2021 in the Silesian Center for Heart Diseases in Zabrze 122 pediatric cardiac transplantations were performed. In the group of recipients with falling Fontan circulation HTx was performed in 5 children. The study group was evaluated for postoperative course: rejection episodes depending on the medical treatment scheme, coinfections and mortality. 1-, 5- and 10-year survival rate between 1988 and 2001 were 53%, 53% and 50%, respectively. 1-, 5- and 10-year survival rate between 2002 and 2011 was 97%, 90% and 87%; between 2012 and 2021 1-year observation with survival rate 92%. The main cause of mortality both in early and in late period after transplantation was graft failure. Cardiac transplantation in children remains the main method of treatment of end-stage heart failure. Our results at both early and long-term posttransplant period, are comparable to those obtained in the most experienced foreign centers.

Diagnosis and Management of Pediatric Heart Failure.

Heart failure is a highly morbid syndrome, recognized as a major cause of adult mortality. Heart failure in pediatric patients, whether in the setting of congenital or acquired heart disease, is similarly associated with high mortality and resource utilization. Understanding the clinical presentation, diagnosis, and initial stabilization of pediatric heart failure is paramount for any acute care clinician as it may mimic common childhood ailments like viral respiratory or gastrointestinal illnesses. Pediatric heart failure occurs in patients with palliated or unpalliated congenital heart disease, familial or acquired cardiomyopathy, acquired valve disease, and myocarditis. This review will focus on heart failure in pediatric patients with structurally normal hearts and will summarize what is known about patterns of presentation, etiologies, diagnostic evaluation, and the acute and chronic management of this highly morbid syndrome.

Successful Use of Angiotensin Receptor–Neprilysin Inhibitor along with Intravenous Inotropes, in a Postcardiac Surgery Child with an Anomalous Left Coronary Artery from the Pulmonary Artery Repair

Abstract Treating heart failure (HF) in postoperative congenital cardiac repair in children with an anomalous left coronary artery from the pulmonary artery (ALCAPA) is a daunting task. Regular anti-failure measures with angiotensin-converting enzyme (ACE) inhibitors may become inadequate or resistant in certain situations. Angiotensin receptor–neprilysin inhibitor (ARNI) in place of ACE inhibitors can be an alternative to help these postcardiac surgical children convalesce better. Sacubitril/valsartan is an ARNI-approved treatment for HF in adults, and in pediatric HF patients, it is showing promising benefits. However, in postcardiac surgical children with HF, its effects are not known. We used ARNI successfully in one of our postcardiac surgical infants with ALCAPA repair in HF, and we describe this case here.

Arrhythmogenic cardiomyopathy is under-recognized in end-stage pediatric heart failure: A 36-year single-center experience.

Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies. We performed a single-center retrospective cohort study reviewing 457 consecutive pediatric (≤18 years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings. Demographic and clinical variables were compared between AC versus non-HCM cardiomyopathy cases. Forty-five percent (n= 205/457) had non-HCM cardiomyopathies as the underlying primary diagnosis. Ten cases (10/205=4.9%) were diagnosed with AC. All 10 had biventricular disease. In 8/10 patients (80%), AC diagnosis was unrecognized pre-HT. Compared with non-AC cardiomyopathies, the AC group was older at diagnosis (9.3 years vs. 4.3 years, p= .012) and transplant (11.1 years vs. 6.5 years, p= .010), had more ventricular arrhythmias (80.0% vs 32.8%, p= .003), and required more anti-arrhythmic use (80.0% vs 32.3%, p= .001). Genetic testing yielded causative pathogenic variants in all tested individuals (n= 5/5, 100%). AC is often an unrecognized cardiomyopathy pretransplant in children who undergo HT. Pediatric non-HCM phenotypes with heart failure who have a significant ventricular arrhythmia burden should be investigated for AC.

Early Clinical Experience with Dapagliflozin in Children with Heart Failure.

Pediatric heart failure (HF) is associated with significant morbidity and mortality. Medical treatment for pediatric HF is largely derived from adult studies. Previously, there has been no described use of dapagliflozin in pediatric HF patients. We describe our single-center experience using dapagliflozin in addition to standard HF medical therapy in 38 pediatric HF patients since January 2020. Median age was 12.2years (interquartile range 6.2-17.5). Majority of patients had dilated cardiomyopathy (68.4%) and reduced left ventricular ejection fraction (LVEF) of 40% or less (65.8%). HF regimens commonly included sacubitril/valsartan, beta-blocker, mineralocorticoid receptor antagonist, and loop diuretic. Median follow-up from dapagliflozin initiation for the whole cohort was 130days (IQR 76-332). Median B-type natriuretic peptide decreased significantly from 222 to 166pg/mL at latest clinical follow-up (P = .04). Estimated glomerular filtration rate trended lower at latest follow-up but was not significant from baseline. There were no clinically significant changes in blood chemistries or vital signs after initiation of dapagliflozin. No patients experienced symptomatic hypoglycemia or hypovolemia. Six patients (15.8%) experienced a symptomatic urinary tract infection necessitating antibiotic treatment. In a separate analysis of 16 patients with dilated cardiomyopathy who received dapagliflozin for a median of 313days (IQR 191-414), median LVEF increased significantly from 32 to 37.2% (P = .006). Dapagliflozin, when added to a background of guideline-directed medical therapy, appears well tolerated in children with HF. Larger studies are needed to evaluate safety and efficacy of dapagliflozin in this population.

Characterization of decellularized left and right ventricular myocardial matrix hydrogels and their effects on cardiac progenitor cells

Congenital heart defects are the leading cause of right heart failure in pediatric patients. Implantation of c-kit+ cardiac-derived progenitor cells (CPCs) is being clinically evaluated to treat the failing right ventricle (RV), but faces limitations due to reduced transplant cell survival, low engraftment rates, and low retention. These limitations have been exacerbated due to the nature of cell delivery (narrow needles) and the non-optimal recipient microenvironment (reactive oxygen species (ROS)). Extracellular matrix (ECM) hydrogels derived from porcine left ventricular (LV) myocardium have emerged as a potential therapy to treat the ischemic LV and have shown promise as a vehicle to deliver cells to injured myocardium. However, no studies have evaluated the combination of an injectable biomaterial, such as an ECM hydrogel, in combination with cell therapy for treating RV failure. In this study we characterized LV and RV myocardial matrix (MM) hydrogels and performed in vitro evaluations of their potential to enhance CPC delivery, including resistance to forces experienced during injection and exposure to ROS, as well as their potential to enhance angiogenic paracrine signaling. While physical properties of the two hydrogels are similar, the decellularized LV and RV have distinct protein signatures. Both materials were equally effective in protecting CPCs against needle forces and ROS. CPCs encapsulated in either the LV MM or RV MM exhibited similar enhanced potential for angiogenic paracrine signaling when compared to CPCs in collagen. The RV MM without cells, however, likewise improved tube formation, suggesting it should also be evaluated as a potential standalone treatment.

Relationship of NT-proBNP to All-Cause Death and Heart Transplant in Children with Ventricular Dysfunction and Compared to Adults with Heart Failure

<h3>Purpose</h3> Plasma NT-proBNP concentration is a widely used diagnostic biomarker for heart failure (HF) in adults and children. We sought to determine whether NT-proBNP concentrations predicted the risk of heart transplantation or death in pediatric HF patients. <h3>Methods</h3> We studied medical records from 109 children with HF in the IBM Watson Explorys database and from 150 children in the Cardiac Biomarkers in Pediatric Cardiomyopathy (PCMR) study. Nonlinear regression with an Emax function was used to model the relationship between NT-proBNP concentrations and the number of events in the two cohorts and with adult patients from Explorys. <h3>Results</h3> All children in the PCMR cohort had dilated cardiomyopathy, whereas the Explorys cohort also included children with congenital heart diseases (Table). Mean age at diagnosis was 3 years in the Explorys cohort and 1.4 years in the PCMR cohort; median NT-proBNP concentrations were 1250 pg/mL and 183.5 pg/mL, respectively. The percentage of deaths and transplantations was higher in the Explorys cohort (7% and 22%, respectively, over 2 years) than in the PCMR cohort (3% and 16%, respectively, over 5 years). Mean estimates of NT-proBNP concentration indicative of half-maximum risk for events (EC50 values) at 2 and 5 years were 3730 pg/mL and 4199 pg/mL, values both close to the mean of 3880 pg/mL established for adults with HF (Figure). <h3>Conclusion</h3> NT-proBNP concentration is suitable for estimating the risk of mortality and morbidity in children with HF, independent of age, and is a candidate surrogate marker for clinical outcome both in adults and children with HF.

Ivabradine Use in a Pediatric Heart Transplant Recipient with Refractory Atrial Tachycardia

<h3>Introduction</h3> Ivabradine, an oral I_f channel antagonist, has been used in pediatric and adult heart failure patients for heart rate control, and in adult heart transplant (HT) recipients with sinus tachycardia secondary to cardiac graft denervation. There are little data describing the use of ivabradine in pediatric HT patients. We describe the use of ivabradine in a pediatric HT recipient with refractory ectopic atrial tachycardia (EAT) causing hemodynamic instability. <h3>Case Report</h3> A 3 year old male underwent HT for restrictive cardiomyopathy. Post-transplant course was complicated by pulmonary hypertensive crisis and cardiac arrest, followed by the development of EAT at a rate of >200 bpm and periods of atrial flutter 2 weeks post-transplant. Steroids were started for suspected clinical rejection, but endomyocardial biopsy was reassuring (ACR grade 1R/1A, pAMR0). Hypotension was noted when EAT rate was >170 bpm. Patient was refractory to esmolol, sotalol, and amiodarone. He underwent EP study with ablation of a posterolateral atrial focus, with recurrence of EAT several hours later. Given ongoing hemodynamic instability with EAT, repeat ablation was performed 1 week later with termination of EAT. However, periodic EAT recurred 3 weeks later and he was again symptomatic with recurrent emesis with rates >170 bpm. Ivabradine 0.05 mg/kg twice daily was initiated. After a single dose, the duration and frequency of EAT periods decreased. Rhythm was mostly sinus (SR) with a rate of 90-100 bpm with premature atrial contractions (PACs), few atrial couplets and blocked PACs. No hypotension, bradycardia, or hemodynamic instability was noted. He was discharged home and dose was up-titrated as an outpatient to 0.2 mg/kg twice daily. Six weeks later, a 72 hour Holter monitor showed predominately SR with short bursts of EAT with variable AV conduction, atrial ectopy overall making up <10% of beats, and rare premature ventricular contractions (PVCs). All subsequent clinic ECGs with follow up to 3 months demonstrated SR. Repeat Holter 3 months after initiation of ivabradine showed SR with rare, isolated PACs and PVCs with no EAT. <h3>Summary</h3> Ivabradine can be considered for EAT treatment in pediatric patients post-HT, with no identified side effects.

Development of Inspired Therapeutics Pediatric VAD: Computational Analysis and Characterization of VAD V3

Pediatric heart failure patients remain in critical need of a dedicated mechanical circulatory support (MCS) solution as development efforts for specific pediatric devices continue to fall behind those for the adult population. The Inspired Pediatric VAD is being developed as a pediatric specific MCS solution to provide up to 30-days of circulatory or respiratory support in a compact modular package that could allow for patient ambulation during treatment. Hydrodynamic performance (flows, pressures), impeller/rotor mechanical properties (torques, forces), and flow shear stress and residence time distributions of the latest design version, Inspired Pediatric VAD V3, were numerically predicted and investigated using computational fluid dynamics (CFD) software (SolidWorks Flow Simulator). Hydrodynamic performance was numerically predicted, indicating no change in flow and pressure head compared to the previous device design (V2), while displaying increased impeller/rotor torques and translation forces enabled by improved geometry. Shear stress and flow residence time volumetric distributions are presented over a range of pump rotational speeds and flow rates. At the lowest pump operating point (3000 RPM, 0.50 L/min, 75 mmHg), 79% of the pump volume was in the shear stress range of 0-10 Pa with < 1% of the volume in the critical range of 150-1000 Pa for blood damage. At higher speed and flow (5000 RPM, 3.50 L/min, 176 mmHg), 65% of the volume resided in the 0-10 Pa range compared to 2.3% at 150-1000 Pa. The initial computational characterization of the Inspired Pediatric VAD V3 is encouraging and future work will include device prototype testing in a mock circulatory loop and acute large animal model.

Abstract 14278: Lessons From COVID-19 Pandemic: Using Telemedicine in Ambulatory Care Delivery for Pediatric Heart Failure and Heart Transplant Patients

Introduction: COVID-19 related restrictions necessitated a rapid transition to telemedicine (TM) ambulatory services for pediatric heart failure (HF) and heart transplant (HT) patients. The impact on patient related outcomes remains unknown. We report the feasibility of incorporating TM in routine ambulatory care delivery to pediatric HF and HT patients at a large academic center during the COVID era (04/2020-04/2021). Methods and Results: A total of 650 HF patients, median age 10 (0-25) years and 200 HT recipients, median age 10 (0-22) years were followed at our center during the COVID era. A center-specific, risk stratified model based on patient characteristics (Table 1) and local COVID restrictions were used to determine patient eligibility for TM only, TM + in-person (IP) or IP care only. There were 2 unexpected deaths in the HT and 0 deaths in the HF patients during the COVID era compared to 1 death in HT and 0 deaths in HF patients in the pre-COVID era (01/2019-12/2019). Both deaths in the COVID era were due to non-cardiac causes and neither patient utilized the TM platform for care. There was a significant decrease in emergency room use and unplanned hospital admissions during the COVID-19 era compared to pre-COVID era (Table 2, Fig 1). Conclusions: TM can safely be incorporated in routine ambulatory care delivery for appropriately selected pediatric patients with HF and HT.