Impact of Age on Emergency Resource Utilization and Outcomes in Pediatric and Young Adult Patients Supported with a Ventricular Assist Device.

2021

2021 Nov 03

1538-943X

<p>There are minimal data describing outcomes in ambulatory pediatric and young adult ventricular assist device (VAD)-supported patient populations. We performed a retrospective analysis of encounter-level data from 2006 to 2017 Nationwide Emergency Department Sample (NEDS) to compare emergency department (ED) resource utilization and outcomes for pediatric (≤18 years, n = 494) to young adult (19-29 years, n = 2,074) VAD-supported patient encounters. Pediatric encounters were more likely to have a history of congenital heart disease (11.3% vs. 4.8%). However, Pediatric encounters had lower admission/transfer rates (37.8% vs. 57.8%) and median charges ($3,334 (IQR $1,473-$19,818) vs. $13,673 ($3,331-$45,884)) (all p &lt; 0.05). Multivariable logistic regression modeling revealed that age itself was not a predictor of admission, instead high acuity primary diagnoses and medical complexity were: (adjusted odds ratio; 95% confidence intervals): cardiac (3.0; 1.6-5.4), infection (3.4; 1.7-6.5), bleeding (3.9; 1.7-8.8), device complication (7.2; 2.7-18.9), and ≥1 chronic comorbidity (4.1; 2.5-6.7). In this largest study to date describing ED resource use and outcomes for pediatric and young adult VAD-supported patients, we found that, rather than age, high acuity presentations and comorbidities were primary drivers of clinical outcomes. Thus, reducing morbidity in this population should target comorbidities and early recognition of VAD-related complications.</p>

10.1097/MAT.0000000000001603

ASAIO J

34743138

Mental Health Disorders and Emergency Resource Use and Outcomes in Ventricular Assist Device Supported Patients.

2021

2021 Jun 02

1097-6744

<p>There are limited data describing the prevalence of mental health disorders (MHDOs) in patients with ventricular assist devices (VADs), or associations between MHDOs and resource use or outcomes. We used the Nationwide Emergency Department Sample administrative database to analyze 44,041 ED encounters for VAD-supported adults from 2010 to 2017, to assess the relationship between MHDOs and outcomes in this population. MHDO diagnoses were present for 23% of encounters, and were associated with higher charges and rates of admission, but lower mortality.</p>

10.1016/j.ahj.2021.05.018

Am Heart J

34089695

Resource utilization in children with paracorporeal continuous-flow ventricular assist devices.

2021

2021 Feb 22

1557-3117

<p><strong>BACKGROUND: </strong>Paracorporeal continuous-flow ventricular assist devices (PCF VAD) are increasingly used in pediatrics, yet PCF VAD resource utilization has not been reported to date.</p>

<p><strong>METHODS: </strong>Pediatric Interagency Registry for Mechanically Assisted Circulatory Support (PediMACS), a national registry of VADs in children, and Pediatric Health Information System (PHIS), an administrative database of children's hospitals, were merged to assess VAD implants from 19 centers between 2012 and 2016. Resource utilization, including hospital and intensive care unit length of stay (LOS), and costs are analyzed for PCF VAD, durable VAD (DVAD), and combined PCF-DVAD support.</p>

<p><strong>RESULTS: </strong>Of 177 children (20% PCF VAD, 14% PCF-DVAD, 66% DVAD), those with PCF VAD or PCF-DVAD are younger (median age 4 [IQR 0-10] years and 3 [IQR 0-9] years, respectively) and more often have congenital heart disease (44%; 28%, respectively) compared to DVAD (11 [IQR 3-17] years; 14% CHD); p &lt; 0.01 for both. Median post-VAD LOS is prolonged ranging from 43 (IQR 15-82) days in PCF VAD to 72 (IQR 55-107) days in PCF-DVAD, with significant hospitalization costs (PCF VAD $450,000 [IQR $210,000-$780,000]; PCF-DVAD $770,000 [IQR $510,000-$1,000,000]). After adjusting for patient-level factors, greater post-VAD hospital costs are associated with LOS, ECMO pre-VAD, greater chronic complex conditions, and major adverse events (p &lt; 0.05 for all). VAD strategy and underlying cardiac disease are not associated with LOS or overall costs, although PCF VAD is associated with higher daily-level costs driven by increased pharmacy, laboratory, imaging, and clinical services costs.</p>

<p><strong>CONCLUSION: </strong>Pediatric PCF VAD resource utilization is staggeringly high with costs primarily driven by pre-implantation patient illness, hospital LOS, and clinical care costs.</p>

10.1016/j.healun.2021.02.011

J Heart Lung Transplant

33744087

Resource Use and Outcomes of Pediatric Congenital Heart Disease Admissions: 2003 to 2016.

2021

e018286

2021 Feb 06

2047-9980

<p><strong>Background</strong> Children with congenital heart disease (CHD) are known to consume a disproportionate share of resources, yet there are limited data concerning trends in resource use and mortality among admitted children with CHD. We hypothesize that charges in CHD-related admissions increased but that mortality improved over time. <strong>Methods and Results</strong> This study, including patients &lt;18&nbsp;years old with CHD, examined inpatient admissions from the nationally representative Kids' Inpatient Database from 2003 to 2016 in order to assess the frequency, medical complexity, and outcomes of CHD hospital admissions. A total of 859&nbsp;843 admissions of children with CHD were identified. CHD admissions increased by 31.8% from 2003 to 2016, whereas overall pediatric admissions decreased by 13.4%. Compared with non-CHD admissions, those with CHD were more likely to be &lt;1&nbsp;year of age (80.5% versus 63.3%), and to have ≥1 complex chronic condition (39.7% versus 9.3%). For CHD admissions, mortality was higher (2.97% versus 0.31%) and adjusted median charges greater ($48&nbsp;426 [interquartile range (IQR), $11.932-$161&nbsp;048] versus $4697 [IQR, $2551-$12&nbsp;301]) (&lt;0.0001 for all). Among CHD admissions, whereas adjusted median charges increased from $35&nbsp;577 (IQR, $9303-$110&nbsp;439) to $61&nbsp;696 (IQR, $15&nbsp;212-$219&nbsp;237), mortality decreased from 3.2% to 2.7% ( &lt;0.0001). CHD admissions accounted for an increased proportion of all inpatient deaths, from 18.0% in 2003 to 24.5% in 2016. <strong>Conclusions</strong> Children admitted with CHD are 10 times more likely to die than those without CHD and have higher charges. Although the rate of mortality in CHD admissions decreased, children with CHD accounted for an increasing proportion of all pediatric inpatient deaths. Effective resource allocation is critical to optimize outcomes in these high-risk patients.</p>

10.1161/JAHA.120.018286

J Am Heart Assoc

33554612

An Increasing Burden of Disease: Emergency Department Visits Among Patients With Ventricular Assist Devices From 2010 to 2017.

2021

e018035

2021 Feb 05

2047-9980

<p>Background With a growing population of patients supported by ventricular assist devices (VADs) and the improvement in survival of this patient population, understanding the healthcare system burden is critical to improving outcomes. Thus, we sought to examine national estimates of VAD-related emergency department (ED) visits and characterize their demographic, clinical, and outcomes profile. Additionally, we tested the hypotheses that resource use increased and mortality improved over time. Methods and Results This retrospective database analysis uses encounter-level data from the 2010 to 2017 Nationwide Emergency Department Sample. The primary outcome was mortality. From 2010 to 2017, &gt;880&nbsp;million ED visits were evaluated, with 44&nbsp;042 VAD-related ED visits identified. The annual mean visits were 5505 (SD 4258), but increased 16-fold from 2010 to 2017 (824 versus 13&nbsp;155). VAD-related ED visits frequently resulted in admission (72%) and/or death (3.0%). Median inflation-adjusted charges were $25&nbsp;679 (interquartile range, $7450, $63&nbsp;119) per encounter. The most common primary diagnoses were cardiac (22%), and almost 30% of encounters were because of bleeding, stroke, or device complications. From 2010 to 2017, admission and mortality decreased from 82% to 71% and 3.4% to 2.4%, respectively ( for trends &lt;0.001, both). Conclusions We present the first study using national-level data to characterize the growing ED resource use and financial burden of patients supported by VAD. During the past decade, admission and mortality rates decreased but remain substantial; in 2017 ≈1 in every 40 VAD ED encounters resulted in death, making it critical that clinical decision-making be optimized for patients with VAD to maximize good outcomes.</p>

10.1161/JAHA.120.018035

J Am Heart Assoc

33543642

Genotype-phenotype association by echocardiography offers incremental value in patients with Noonan Syndrome with Multiple Lentigines.

2020

2020 Dec 14

1530-0447

<p><strong>BACKGROUND: </strong>Noonan Syndrome with Multiple Lentigines (NSML) and Noonan Syndrome (NS) can be difficult to differentiate clinically in early childhood. This study aims to describe characteristics of the ventricular septum that may differentiate NSML from NS. We hypothesize that the shape of the ventricular septum determined by echocardiography correlates with genotype and may distinguish patients with NSML from those with NS.</p>

<p><strong>METHODS: </strong>We analyzed data from 17 NSML and 67 NS patients. Forty normal and 30 sarcomeric hypertrophic cardiomyopathy (HCM) patients were included as controls. Septal morphology was qualitatively evaluated, and septal angle was measured quantitatively at end diastole. We recorded the presence of a ventricular septal bulge (VSB) and reviewed genetic testing results for each patient.</p>

<p><strong>RESULTS: </strong>The most important findings were a sigmoid septum (71%) and VSB (71%) in NSML. NSML septal angle was decreased compared to the normal and sarcomeric HCM control groups, respectively (149 ± 13 vs. 177 ± 3, p &lt; 0.001; 149 ± 13 vs. 172 ± 7, p &lt; 0.001). NS septal angle was similar to the controls (176 ± 6 vs. 177 ± 3, p &gt; 0.5; 176 ± 6 vs. 172 ± 7, p &gt; 0.5). NSML-linked pathogenic variants were associated with sigmoid septum and VSB.</p>

<p><strong>CONCLUSIONS: </strong>These findings provide novel phenotypic evidence to clinicians that may offer incremental diagnostic value in counseling families in ambiguous NSML/NS cases.</p>

<p><strong>IMPACT: </strong>Characteristics of the ventricular septum are linked to specific gene variants that cause NSML and NS. Sigmoid septum and VSB are associated with NSML. This novel echocardiographic association may help clinicians distinguish NSML from NS in ambiguous cases. Early distinction between the two may be important, as syndrome-specific therapies may become available in the near future. This study may encourage further research into genotype-phenotype associations in other forms of HCM.</p>

10.1038/s41390-020-01292-7

Pediatr Res

33318624

Measuring Quality in Pediatric Heart Transplantation-An Important but Challenging Goal.

2020

e2024137

2020 Nov 02

2574-3805

10.1001/jamanetworkopen.2020.24137

JAMA Netw Open

33136130

Genetic variant burden and adverse outcomes in pediatric cardiomyopathy.

2020

2020 Aug 03

1530-0447

<p><strong>BACKGROUND: </strong>Previous genetic research in pediatric cardiomyopathy (CM) has focused on pathogenic variants for diagnostic purposes, with limited data evaluating genotype-outcome correlations. We explored whether greater genetic variant burden (pathogenic or variants of unknown significance, VUS) correlates with worse outcomes.</p>

<p><strong>METHODS: </strong>Children with dilated CM (DCM) and hypertrophic CM (HCM) who underwent multigene testing between 2010 and 2018 were included. Composite endpoint was freedom from major adverse cardiac event (MACE).</p>

<p><strong>RESULTS: </strong>Three hundred and thirty-eight subjects were included [49% DCM, median age 5.7 (interquartile range (IQR) 0.2-13.4) years, 51% HCM, median age 3.0 (IQR 0.1-12.5) years]. Pathogenic variants alone were not associated with MACE in either cohort (DCM p = 0.44; HCM p = 0.46). In DCM, VUS alone [odds ratio (OR) 4.0, 95% confidence interval (CI) 1.9-8.3] and in addition to pathogenic variants (OR 5.2, 95% CI 1.7-15.9) was associated with MACE. The presence of VUS alone or in addition to pathogenic variants were not associated with MACE in HCM (p = 0.22 and p = 0.33, respectively).</p>

<p><strong>CONCLUSION: </strong>Increased genetic variant burden (pathogenic variants and VUS) is associated with worse clinical outcomes in DCM but not HCM. Genomic variants that influence DCM onset may be distinct from those driving disease progression, highlighting the potential value of universal genetic testing to improve risk stratification.</p>

<p><strong>IMPACT: </strong>In pediatric CM, inconsistent findings historically have been shown between genotype and phenotype severity when only pathogenic variants have been considered.Increased genetic variant burden (including both pathogenic variants and VUS) is associated with worse clinical outcomes in DCM but not HCM.Genomic variants that influence CM onset may be distinct from those variants that drive disease progression and influence outcomes in phenotype-positive individuals.Incorporation of both pathogenic variants and VUS may improve risk stratification models in pediatric CM.</p>

10.1038/s41390-020-1101-5

Pediatr. Res.

32746448

Increasing Pump Speed During Exercise Training Improves Exercise Capacity in Children with Ventricular Assist Devices.

2020

2020 Jul 17

1538-943X

<p>Exercise rehabilitation during pediatric ventricular assist device (VAD) support aims to improve musculoskeletal strengthening while awaiting heart transplantation (HT). This study aimed to determine whether increasing VAD pump speed during exercise testing and training improves exercise capacity. A single-center cohort study was performed comparing changes in exercise capacity on serial cardiopulmonary exercise testing (CPET) after exercise training at a fixed VAD pump speed (historical cohort from 2014 to 2017) compared with a prospective cohort (2017-2019) who underwent increasing pump speed during exercise training. All children were supported with intracorporeal continuous-flow VAD. Four subjects (13 ± 2.8 years) were included in the historical cohort, and 6 subjects (14 ± 1.7 years) were enrolled in the prospective cohort. Ninety percent had dilated cardiomyopathy, and one had single ventricle Fontan physiology. Baseline maximal oxygen consumption (VO2) was 19 ± 6.3 ml/kg/min. After exercise training with increased pump speed, there was substantial improvement in aerobic capacity (maximal VO2 increased 42% vs. decreased 3%, respectively) and working capacity (maximal work increased 49% vs. 13%, respectively) compared with fixed pump speed. There were no adverse events reported in either the fixed or increased pump speed cohorts. Increasing VAD pump speed during exercise training results in substantial improvement in both physical working and aerobic capacity compared a fixed pump speed in children on VAD support regardless of single or biventricular ventricle physiology. Further study of a larger cohort is needed to validate these findings to improve the approach to pediatric cardiac rehabilitation in this population.</p>

10.1097/MAT.0000000000001231

ASAIO J.

32701623

The Impact of Syndromic Genetic Disorders on Medical Management and Mortality in Pediatric Hypertrophic Cardiomyopathy Patients.

2020

2020 May 30

1432-1971

<p>Hypertrophic cardiomyopathy (HCM) is a prevalent cardiomyopathy in children, with variable etiologies, phenotypes, and associated syndromic genetic disorders (GD). The spectrum of evaluation in this heterogeneous population has not been well described. We aimed to describe mortality and medical management in the pediatric HCM population, and compare HCM pediatric patients with GD to those without GD. Children (&lt; 18&nbsp;years) with HCM from the claims-based Truven Health Analytics MarketScan Research Database for years 2013-2016 were identified. Outcomes, including patient visits, diagnostic tests, procedures, medications, and mortality, were reported across demographic and clinical characteristics. Multivariable negative binomial, logistic, and survival models were utilized to test the association between those with and without GD by outcomes. 4460 patients were included, with a median age of 11&nbsp;years (IQR 3-16), 61.7% male, 17.7% with GD, and 2.1% who died during the study period. There were 0.36 inpatient admissions per patient-year. Patients with GD were younger [8&nbsp;years (IQR 1-14) vs 12&nbsp;years (IQR 3-16) (p &lt; 0.0001)], had more echocardiograms (1.77 vs 0.93) p &lt; 0.0001; and ambulatory cardiac monitoring per year (0.32 vs 0.24); p = 0.0002. Adjusting for potential confounders including age, other chronic medical conditions, procedures, and heart failure, GD had increased risk of mortality [HR 2.46 (95% CI 1.62, 3.74)], myectomy [HR 1.59 (95% CI 1.08, 2.35)], and more annual admissions [OR 1.36 (CI 1.27, 1.45]. Patients with HCM show higher rates of death, admission, testing, and myectomy when concomitant syndromic genetic disorders are present, suggesting that the disease profile and resource utilization are different from HCM patients without GD.</p>

10.1007/s00246-020-02373-4

Pediatr Cardiol

32474737