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

Interventricular septal hematoma complicating placement of a ventricular assist device in an infant and support with bi-atrial cannulation.

2020

90-92

2020 Mar

2666-2507

<p>Interventricular septal hematoma is a rare complication of pediatric cardiac surgery, usually in the context of ventricular septal defect closure.&nbsp;We present intraoperative echocardiography of a large interventricular septal hematoma secondary to the placement of a left ventricular assist device (VAD) in an infant who required conversion to biventricular support with bi-atrial cannulation. As the patient later died of neurologic complications, we present correlation with pathologic findings.</p>

10.1016/j.xjtc.2019.10.001

JTCVS Tech

33103126

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

Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.

2019

2019 May 07

1432-1971

<p>In complete atrioventricular canal defect (CAVC), there are limited data on preoperative clinical and echocardiographic predictors of operative timing and postoperative outcomes. A retrospective, single-center analysis of all patients who underwent primary biventricular repair of CAVC between 2006 and 2015 was performed. Associated cardiac anomalies (tetralogy of Fallot, double outlet right ventricle) and arch operation were excluded. Echocardiographic findings on first postnatal echocardiogram were correlated with surgical timing and postoperative outcomes using bivariate descriptive statistics and multivariable logistic regression. 153 subjects (40% male, 84% Down syndrome) underwent primary CAVC repair at a median age of 3.3 (IQR 2.5-4.2) months. Median postoperative length of stay (LOS) was 7 (IQR 5-15) days. Eight patients (5%) died postoperatively and 24 (16%) required reoperation within 1&nbsp;year. On multivariable analysis, small aortic isthmus (z score &lt; -&nbsp;2) was associated with early primary repair at &lt; 3&nbsp;months (OR 2.75, 95% CI 1.283-5.91) and need for early reoperation (OR 3.79, 95% CI 1.27-11.34). Preoperative ventricular dysfunction was associated with higher postoperative mortality (OR 7.71, 95% CI 1.76-33.69). Other factors associated with mortality and longer postoperative LOS were prematurity (OR 5.30, 95% CI 1.24-22.47 and OR 5.50, 95% CI 2.07-14.59, respectively) and lower weight at surgery (OR 0.17, 95% CI 0.04-0.75 and OR 0.55, 95% CI 0.35-0.85, respectively). Notably, preoperative atrioventricular valve regurgitation and Down syndrome were not associated with surgical timing, postoperative outcomes or reoperation, and there were no echocardiographic characteristics associated with late reoperation beyond 1 year after repair. Key preoperative echocardiographic parameters helped predict operative timing and postoperative outcomes in infants undergoing primary CAVC repair. Aortic isthmus z score &lt; -&nbsp;2&nbsp; was associated with early surgical repair and need for reoperation, while preoperative ventricular dysfunction was associated with increased mortality. These echocardiographic findings may help risk-stratified patients undergoing CAVC repair and improve preoperative counseling and surgical planning.</p>

10.1007/s00246-019-02116-0

Pediatr Cardiol

31065759

Significant mortality, morbidity and resource utilization associated with advanced heart failure in congenital heart disease in children and young adults.

2018

9-19

2018 Dec 05

1097-6744

<p><strong>BACKGROUND: </strong>Children with congenital heart disease (CHD) are at risk for advanced heart failure (AHF). We sought to define the mortality and resource utilization in CHD-related AHF in children and young adults.</p>

<p><strong>METHODS: </strong>All hospitalizations in the Pediatric Health Information System database involving patients ≤21 years old with a CHD diagnosis and heart failure requiring at least 7 days of continuous inotropic support between 2004 and 2015 were included. Hospitalizations including CHD surgery were excluded.</p>

<p><strong>RESULTS: </strong>Of 465,482 CHD hospitalizations, AHF was present in 2,712 (0.6%) [58% infant, 55% male, 30% single ventricle]. AHF therapies frequently used included extracorporeal membrane oxygenation (ECMO) (15%) and cardiac transplant (16%). Ventricular assist device (VAD) support was rare (3%), although VAD use significantly increased from 2004 to 2015 (P &lt; .0010). Hospital mortality in CHD with AHF was 26%, with higher mortality associated with single ventricle heart disease (OR 1.64, 95% CI 1.23-2.19; P = .0009), infancy (OR 1.71, 95% CI 1.17-2.5; P = .0057), non-white race (OR 1.28, 95% CI 1.04-1.59; p=0.0234), and chronic complex comorbidities (OR 1.76, 95% CI 1.34-2.30; P &lt; .0001). Over the 11-year study period, despite the significant increase in CHD-related AHF hospitalizations (P &lt; .0001), hospital mortality improved (P = .0011). Median hospital costs were $252,000, a 6-fold increase above those without AHF, and was primarily driven by hospital length of stay (P &lt; .0001).</p>

<p><strong>CONCLUSION: </strong>AHF in children with CHD in uncommon but increasing and is associated with significant morbidity, mortality and resource utilization. Approximately 1 in 5 children do not survive to hospital discharge. Many risk factors for mortality may not be modifiable, and further study is needed to identify modifiable risk factors and improve care for this complex population.</p>

10.1016/j.ahj.2018.11.010

Am. Heart J.

30639612

Mortality, Resource Utilization And Inpatient Costs Vary Among Pediatric Heart Transplant Indications: A Merged Data Set Analysis From The United Network For Organ Sharing And The Pediatric Health Information Systems (UNOS-PHIS) Databases.

2018

2018 Nov 25

1532-8414

<p><strong>BACKGROUND: </strong>Merging UNOS and PHIS databases have enabled a more granular analysis of pediatric heart transplant outcomes and resource utilization. We evaluated whether transplant indication at time of transplantation was associated with mortality, resource utilization and inpatient costs during the first year post-transplant.</p>

<p><strong>METHODS: </strong>We analyzed transplant outcomes and resource utilization between 2004 and 2015. Patients were categorized as congenital (CHD), myocarditis or cardiomyopathy based on UNOS-defined primary indication. CHD complexity subgroup analyses (single ventricle, complex, and simple biventricular CHD) were also performed.</p>

<p><strong>RESULTS: </strong>Of 2251 transplants (49% CHD, 5% myocarditis, 46% cardiomyopathy), CHD recipients were younger (2 [IQR 0-10] vs. 6 [IQR 0-12] vs. 7 [IQR 1-14] years, respectively; p&lt;0.001) and less likely to have ventricular assist device (VAD) at transplant (CHD 3%, myocarditis 27%, cardiomyopathy 13%; p&lt;0.001). Patients with single ventricle CHD had the longest waitlist and were least likely to receive a VAD pre-transplant. After adjusting for patient-level factors, transplant recipients with single ventricle CHD had the greatest mortality during transplant-admission and at 1-year (vs. cardiomyopathy, OR 11.8 [95% CI 5.9-23.6] and OR 6.0 [95% CI 3.6-10.2], respectively). Mortality was similar between patients with myocarditis and cardiomyopathy. Post-transplant length of stay (LOS) was longer in transplant recipients with CHD than myocarditis or cardiomyopathy (25 [IQR 15-45] vs. 21 [IQR 12-35] vs. 16 [IQR 12-25] days; p&lt;0.001), partially related to longer duration of ICU-level care (ICU LOS 8 [IQR 4-20] vs. 6 [IQR 4-13] vs. 5 [IQR 3-8] days; p&lt;0.001). Similarly, patients with CHD had higher median post-transplant costs than myocarditis or cardiomyopathy ($415k [IQR $201-503k] vs. $354k [IQR $179-390k] vs. $284k [IQR $145-319k]; p&lt;0.001) that persisted after adjusting for patient-level factors (CHD vs. cardiomyopathy Adjusted Cost Ratio 1.4 [95% CI 1.4-1.5]) and was primarily driven by longer LOS. Over 50% were readmitted during first year post-transplant, although readmission rates were similar across transplant indications (p=0.42).</p>

<p><strong>CONCLUSION: </strong>Children with CHD, particularly single ventricle patients, require substantially greater hospital resource utilization and have significantly worse outcomes during the first year after heart transplant compared to other indications. Further work is aimed at identifying modifiable pre-transplant risk factors, such as pre-transplant conditioning with VAD support and cardiac rehabilitation, to improve post-transplant outcomes and reduce resource utilization in this complex population.</p>

10.1016/j.cardfail.2018.11.014

J. Card. Fail.

30485789