Staged Palliation of Hypoplastic Left Heart Syndrome: Trends in Mortality, Cost, and Length of Stay Using a National Database from 2000 Through 2009

doi:10.1016/j.amjcard.2013.02.039

The American Journal of Cardiology

Volume 111, Issue 12, 15 June 2013, Pages 1792-1799

https://doi.org/10.1016/j.amjcard.2013.02.039 Get rights and content

Staged surgical palliation has revolutionized the care of patients with hypoplastic left heart syndrome (HLHS), although the outcomes of survival and cost at a national level remain unclear. This study sought to evaluate (1) trends in HLHS surgical outcomes including in-hospital mortality, length of stay (LOS), and cost, and (2) patient and hospital risk factors associated with these outcomes. Hospitalizations for patients with HLHS, including stage I, II, and III palliations, were analyzed using the Kids' Inpatient Database from 2000 through 2009. Trends in mortality, LOS, and cost were analyzed and chi-squared tests were used to test association between categorical variables. Patient and hospital characteristics associated with death were analyzed using logistic regression and associations with LOS were analyzed using ordinary least squared regression. There were 16,923 hospital admissions in patients with HLHS of which 5,672 (34%) included surgical intervention. Total (3,201–5,102) and surgery-specific admissions (1,165–1,618) increased from 2000 to 2009. Mortality decreased 14% per year in stage III palliations (odds ratio [OR] 0.86; 95% confidence interval [CI]: 0.79–0.94) and 6% per year for stage I palliations (OR 0.94; 95% CI 0.90–0.99) but not for stage II palliations (OR 1.01; 95% CI; 0.89–1.14). Length of stay increased for stage I and II palliations; however, per-patient hospital cost decreased in 2009. In conclusion, recent decrease in per patient cost for staged surgical palliation for HLHS has correlated temporally with improved mortality.

Section snippets

Methods

This study was a retrospective, observational data analysis over a 10-year period from 2000 to 2009. This study was performed in accordance with the institutional review board at Cincinnati Children's Hospital Medical Center (IRB# 2011-1926).

The 2000, 2003, 2006, and 2009 KID data were used. The KID, developed by HCUP, which was sponsored by the Agency for Healthcare Research and Quality, was designed to analyze inpatient visits for children aged ≤20 years in the United States and has been used

Results

There were 16,923 admissions for patients with HLHS for the 2000, 2003, 2006, and 2009 time points of which 5,672 (34%) were admissions for staged surgical palliation (Table 1). From 2000 through 2009, there was an increasing number of total HLHS admissions as well as admissions with associated staged surgical palliations. Comparing surgical admissions by stage type, there was a decrease in the percent of admissions for stage I palliation, from 62% in 2000 to 48% in 2009, with a corresponding

Discussion

This study demonstrated a national trend of decreasing HLHS in-hospital mortality that is concurrent with increases in total admissions, surgical admission, and LOS. These changes occurred temporally with stabilization, if not reduction, in the associated per-patient cost burden. The results offer a broad perspective on the direction of care for HLHS in the United States between 2000 and 2009. Such insights on HLHS care are important and complementary to the knowledge gained through efforts

Disclosures

The authors have no conflicts of interest to disclose.

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Cited by (34)

Echocardiographic assessment of ventricular function: Conventional and advanced technologies and their clinical applications
2020, Progress in Pediatric Cardiology
Citation Excerpt :
Peak exercise capacity is generally decreased (approximately 60% of normal) in Fontan patients [67]. Although the development of the staged surgical palliation has improved life expectancy, patients with single ventricle are still at risk for progressive heart failure and death [68], especially in patients with single RV as RV is morphologically not capable of dealing with chronic exposure to the high afterload of the systemic circulation [69]. Although ventricular systolic function was overall preserved in most of the pediatric patients after Fontan operation [70], many of these patients with functional single RV due to hypoplastic left heart syndrome develop ventricular mechanical dyssynchrony, likely contributing to their RV dysfunction [71].
Echocardiogram is the first-line and most used noninvasive imaging diagnostic modality and is convenient and reliable in its assessment of ventricular function in pediatrics. Ventricular function is an important physiological parameter representing myocardial performance in response to overall hemodynamic status. Here, we introduce basic principles of conventional echocardiography and advanced echocardiographic technology, including tissue Doppler imaging, 3-dimensional echocardiogram, and speckled tracking echocardiography. Then, we overview clinical applications of these methods in assessing ventricular function in pediatrics, including single ventricle after staged surgical palliation, right ventricular dysfunction in pulmonary hypertension, and ventricular-ventricular interactions in various heart disease. With multimodality approaches, the advantages and limitations of echocardiographic assessment of ventricular function are discussed.
Selective Use of Inpatient Interstage Management After Norwood Procedure
2020, Annals of Thoracic Surgery
We report our intermediate-term results after Norwood procedure, including use of an interstage inpatient management strategy for high-risk patients, and seek to create a predictive model for probability of discharge.
A single-site retrospective review was conducted for all patients undergoing Norwood procedure from 2006 to 2016 (N = 177). We compared those discharged home with those who either remained hospitalized until Glenn procedure or died before Norwood procedure discharge. Multivariable logistic regression was used to develop a predictive model for discharge.
During the study period, 120 (68%) patients were discharged home, 45 (25%) remained hospitalized, and 12 (7%) died before Glenn procedure (median age: 71 days). Interstage survival for those discharged after Norwood procedure was 100%. Longitudinal survival for the cohort was 86%, 81%, and 77% at 1, 5, and 10 years, resepectively. Ten-year survival was significantly greater for the discharged group compared with the interstage inpatients (86% vs 56%, P < .001). A reduced predictive model of discharge included lower gestational age (odds ratio [OR]: 0.95), lower median income for ZIP code (OR: 0.4), lower birth-weight-for-age z-score (OR: 0.56), longer cardiopulmonary bypass time (OR: 0.45), and Blalock-Taussig shunt (OR: 0.32).
Survival up to 10 years after Norwood procedure is good using a strategy of inpatient care for a subset of high-risk patients to mitigate home interstage mortality. A probabilistic model used after Norwood procedure was able to predict interstage discharge with good accuracy, but will require external validation to ensure generalizability. Further work is also needed to determine optimal palliative pathways for the high-risk patients because of the notable attrition beyond successful bidirectional Glenn procedure.
Health Care Resource Utilization Among Children With Congenital Heart Disease: A Population-Based Study
2018, Canadian Journal of Cardiology
Citation Excerpt :
In Canada, hospitalizations and associated costs among children with CHD have been increasing over time (although to a lesser extent compared with the adult with CHD), with complex CHD lesions accounting for the majority of inpatient resource utilization.10,16 Therefore, improving survival of children with SV1 is anticipated to further increase HRU in this subgroup. This study provides a better understating of the resource utilization among children with CHD during early childhood in the context of a government-funded, universal health care system.
Data regarding health care resource utilization (HRU) in early childhood among children with congenital heart disease (CHD) are scarce. Therefore, we sought to describe the extent of HRU incurred among children with CHD in the first 5 years of life.
This population-based retrospective cohort study included all children born between January 2005 and March 2014 in Alberta, Canada. We linked inpatient, outpatient, practitioner claims, and drug dispensing databases with vital statistics (birth and death registries).
In the first year of life, the cumulative hospitalization rate per 100 children was 335 (95% confidence interval: 312-360) for single ventricle (SV) children, 200 (194-206) for moderate-complex CHD, and 152 (149-156) for simple CHD vs 109 (108-109) among children without CHD (P < 0.001). The ambulatory-care visit rate per 100 children was 4871 (4780-4963) for SV, 2278 (2258-2299) for moderate-complex, and 1416 (1405-1426) for simple CHD vs 246 (246-247) for children without CHD (P < 0.001). The rates of physician claims and drug dispensing also demonstrated similar patterns. The median total hospitalization length of stay during the first year of life was 54 days (interquartile range: 26-95) in SV, 15 (4-39) in moderate-complex, and 6 (2-26) in simple CHD compared with 2 (1-3) among children without CHD (P < 0.001). These differences remained throughout the first 5 years of life, with children with CHD having consistently higher hospitalization rates and emergency department visit rates in every year of age compared with children without CHD.
Cumulative HRU is high among children with CHD in the first 5 years of life and increases with increasing CHD severity. Improving survival of SV lesions will require increasing resource allocation to this group.
Les données sur l’utilisation des ressources en soins de santé chez les jeunes enfants atteints de cardiopathie congénitale sont rares. Nous avons donc voulu quantifier l’utilisation des ressources en soins de santé au cours des 5 premières années de vie des enfants atteints de cardiopathie congénitale.
Cette étude rétrospective de cohorte fondée sur la population portait sur tous les enfants nés entre janvier 2005 et mars 2014 en Alberta, au Canada. Nous avons couplé les bases de données sur les patients hospitalisés, les patients ambulatoires, les demandes de paiement des professionnels de la santé et les médicaments distribués avec les statistiques vitales (registres des naissances et des décès).
Au cours de la première année de vie, le taux cumulatif d’hospitalisation pour 100 enfants était de 335 (intervalle de confiance à 95 % : 312-360) chez les enfants présentant un ventricule unique), de 200 (194-206) pour les cardiopathies congénitales modérées à complexes et de 152 (149-156) pour les cardiopathies congénitales simples, comparativement à 109 (108-109) chez les enfants exempts de cardiopathie congénitale (p < 0,001). Le taux de visite aux services de soins ambulatoires pour 100 enfants était de 4871 (4780-4963) dans les cas de ventricule unique, de 2278 (2258-2299) pour les cardiopathies congénitales modérées à complexes et de 1416 (1405-1426) pour les cardiopathies congénitales simples, comparativement à 246 (246-247) chez les enfants exempts de cardiopathie congénitale (p < 0,001). Les taux des demandes de paiement des médecins et de distribution de médicaments suivaient les mêmes tendances. La durée médiane totale de l’hospitalisation durant la première année de vie était de 54 jours (écart interquartile : 26-95) dans les cas de ventricule unique, de 15 jours (4-39) pour les cardiopathies congénitales modérées à complexes et 6 jours (2-26) pour les cardiopathies congénitales simples, comparativement à 2 jours (1-3) chez les enfants exempts de cardiopathie congénitale (p < 0,001). Ces différences ont persisté tout au long de chacune des 5 premières années de vie, durant lesquelles les taux d’hospitalisation et de visite à l’urgence des enfants atteints de cardiopathie congénitale étaient systématiquement plus élevés que les enfants qui en étaient exempts.
L’utilisation des ressources en soins de santé cumulative est élevée durant les 5 premières années de vie des enfants atteints de cardiopathie congénitale et augmente en fonction de la gravité de cette dernière. L’amélioration de la survie dans les cas de lésions associées à un ventricule unique nécessitera une affectation accrue de ressources à ce groupe.
Genetics of hypoplastic left heart syndrome
2016, Journal of Pediatrics
Citation Excerpt :
The Single Ventricle Reconstruction Trial sponsored by the Pediatric Heart Network demonstrated that the poor neurodevelopmental outcomes were associated more with innate patient factors than intraoperative management.13 In addition to the surgical interventions, survivors with HLHS face long-term health care burden and comorbid conditions resulting in high health care resource utilization.14-16 It is noteworthy that the delineation of the extended natural history of HLHS has just begun as the current surgical intervention was first implemented in the 1980s.
Resource utilization for noncardiac admissions in pediatric patients with single ventricle disease
2016, American Journal of Cardiology
Citation Excerpt :
Characterization and recognition of how hospital resources are used has become an increasingly important aspect of patient management as health care costs have dramatically increased in recent decades. Even with, or perhaps because of, advancements in surgical techniques, costs of staged SV palliation have continued to increase.17 It is prudent, therefore, to understand the reasons why resources are used so steps can be taken to decrease the factors that lead to increased costs.
Patients with single ventricle (SV) congenital heart disease (CHD) incur high hospital costs during staged surgical palliation. Health care resource utilization for noncardiac admissions in patients with SV has not been reported. This study sought to compare costs and outcomes for common noncardiac hospital admissions between patients with SV and patients without CHD. Hospital discharge data from the University Health System Consortium from January 2011 to December 2013 was queried for patients aged ≤18 years with International Classification of Diseases, Ninth Revision (ICD-9) codes for SV lesions: hypoplastic left heart syndrome (746.7), tricuspid atresia (746.1), or common ventricle (745.3). Primary diagnosis, direct cost, length of stay (LOS), intensive care unit admission rate and mortality data were obtained. The 10 most common noncardiac admission diagnoses were compared between patients with SV and patients without CHD using t test and Fisher's exact test. Total direct cost, LOS, and intensive care unit admission rate were higher for patients with SV for all diagnoses with the exception of LOS for dehydration, which was not different between groups. Hospital mortality was significantly higher for patients with SV admitted for acute kidney injury, esophageal reflux, failure to thrive, respiratory syncytial virus bronchiolitis and pneumonia. In conclusion, our study demonstrates that patients with SV CHD admitted with noncardiac diagnoses have higher health care resource utilization compared to those without CHD. As long-term survival increases, it can be expected that this patient group will use a disproportionate amount of medical dollars. Further characterization of costs will be important so steps can be taken to reduce or prevent hospitalization in these patients.
Variation in hospital costs and resource utilisation after congenital heart surgery
2023, Cardiology in the Young

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Congenital heart diseaseStaged Palliation of Hypoplastic Left Heart Syndrome: Trends in Mortality, Cost, and Length of Stay Using a National Database from 2000 Through 2009

Section snippets

Methods

Results

Discussion

Disclosures

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Am J Cardiol

Heart Rhythm

Am J Cardiol

J Pediatr

In-hospital mortality for children with hypoplastic left heart syndrome after stage I surgical palliation: teaching versus nonteaching hospitals

Pediatrics

Staged surgical palliation in hypoplastic left heart syndrome and its variants

J Card Surg

Mitral stenosis and aortic atresia in hypoplastic left heart syndrome: survival analysis after stage I palliation

Ann Thorac Surg

Right ventricle to pulmonary artery conduit reduces interim mortality after stage 1 Norwood for hypoplastic left heart syndrome

Ann Thorac Surg

Two-year survival and mental and psychomotor outcomes after the Norwood procedure: an analysis of the modified Blalock-Taussig shunt and right ventricle-to-pulmonary artery shunt surgical eras

Circulation

Congenital heart disease
Staged Palliation of Hypoplastic Left Heart Syndrome: Trends in Mortality, Cost, and Length of Stay Using a National Database from 2000 Through 2009