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

Neonatal lymphatic flow disorders: impact of lymphatic imaging and interventions on outcomes.

2020

2020 Sep 02

1476-5543

<p>Neonatal chylothorax (NCTx) and central lymphatic flow disorder (CLFD) are historically challenging neonatal disorders with high morbidity and mortality.</p>

<p><strong>METHODS: </strong>We conducted a retrospective study of 35 neonates with pulmonary lymphatic abnormalities at our institution who underwent lymphatic evaluation between December 2015 and September 2018. Patients with only pulmonary lymphatic perfusion syndrome were classified as NCTx and those with multiple flow abnormalities were classified as CLFD. Demographics, clinical characteristics, and outcomes were compared using t-tests/Wilcoxon rank sum tests and Fisher's exact tests.</p>

<p><strong>RESULTS: </strong>All 35 patients had intranodal MR lymphangiography and 14 (40%) also had conventional fluoroscopic lymphangiography. Fifteen (42.8%) patients were diagnosed with NCTx and 20 (57.1%) were diagnosed with CLFD. Thirty-four (97.1%) patients had pleural effusions. None of the NCTx group had ascites, anasarca, or dermal backflow compared to 17 (85%) (p &lt; 0.001), 8 (42.1%) (p: 0.004), and 20 (100%) (p &lt; 0.001) of the CLFD group, respectively. In the NCTx group, 11 (73.3%) had ethiodized oil embolization and 4 (26.7%) received conservative therapy. Ten (50%) of the CLFD patients had an intervention; of those, two (10%) had ethiodized oil-only embolization. Eight had non-ethiodized oil embolizations (two (25%) had embolization with glue, three (37.5%) underwent surgical lymphovenous anastomosis, two (25%) underwent thoracic duct (TD) externalization, and one (12.5%) had a non-TD lymphatic channel drain placed). Complete resolution of pleural effusions was achieved in all 15 NCTx patients, whereas 9 (45%) of 20 CLFD patients had resolution of chylothorax (p: 0.001).</p>

<p><strong>CONCLUSIONS: </strong>Establishing a diagnosis of NCTx or CLFD is paramount in selecting treatment options and providing prognostic information. Development of lymphatic interventions represents a paradigm shift in our understanding of neonatal lymphatic flow disorders and may be associated with improved survival.</p>

10.1038/s41372-020-00771-3

J Perinatol

32879418

Standardization of the Perioperative Management for Neonates Undergoing the Norwood Operation for Hypoplastic Left Heart Syndrome and Related Heart Defects.

2020

2020 Jul 16

1529-7535

<p><strong>OBJECTIVES: </strong>In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center.</p>

<p><strong>DESIGN: </strong>Quality improvement project.</p>

<p><strong>SETTING: </strong>High volume cardiac center, tertiary care children's hospital.</p>

<p><strong>PATIENTS: </strong>Neonates undergoing Norwood operation.</p>

<p><strong>INTERVENTIONS: </strong>The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients.</p>

<p><strong>MEASUREMENTS AND MAIN RESULTS: </strong>One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02).</p>

<p><strong>CONCLUSIONS: </strong>This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events.</p>

10.1097/PCC.0000000000002478

Pediatr Crit Care Med

32701749

A Comparison of Bidirectional Glenn vs. Hemi-Fontan Procedure: An Analysis of the Single Ventricle Reconstruction Trial Public Use Dataset.

2020

2020 May 29

1432-1971

<p>Patients with single ventricle (SV) heart defects have two primary surgical options for superior cavopulmonary connection (SCPC): bidirectional Glenn (BDG) and hemi-Fontan (HF). Outcomes based on type of SCPC have not been assessed in a multi-center cohort. This retrospective cohort study uses the Single Ventricle Reconstruction (SVR) Trial public use dataset. Infants who survived to SCPC were evaluated through 1&nbsp;year of age, based on type of SCPC. The primary outcome was transplant-free survival at 1&nbsp;year. The cohort included 343 patients undergoing SCPC across 15 centers in North America; 250 (73%) underwent the BDG. There was no difference between the groups in pre-SCPC clinical characteristics. Cardiopulmonary bypass times were longer [99&nbsp;min (IQR 76, 126) vs 81&nbsp;min (IQR 59, 116), p &lt; 0.001] and use of deep hypothermic circulatory arrest (DHCA) more prevalent (51% vs 19%, p &lt; 0.001) with HF. Patients who underwent HF had a higher likelihood of experiencing more than one post-operative complication (54% vs 41%, p = 0.05). There were no other differences including the rate of post-operative interventional cardiac catheterizations, length of stay, or survival at discharge, and there was no difference in transplant-free survival out to 1&nbsp;year of age. Mortality after SCPC is low and there is no difference in mortality at 1&nbsp;year of age based on type of SCPC. Differences in support time and post-operative complications support the preferential use of the BDG, but additional longitudinal follow-up is necessary to understand whether these differences have implications for long-term outcomes.</p>

10.1007/s00246-020-02371-6

Pediatr Cardiol

32472151

Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

2020

e015318

2020 Apr 07

2047-9980

<p><strong>Background</strong> Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined.</p>

<p><strong>Methods and Results</strong> This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. The primary outcome was ≤6&nbsp;months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage &gt;14&nbsp;days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2-weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty-eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications (=0.001).</p>

<p><strong>Conclusions </strong>There is substantial morbidity in the early post-Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.</p>

10.1161/JAHA.119.015318

J Am Heart Assoc

32223393

Protein Losing Enteropathy After Fontan Operation: Glimpses of Clarity Through the Lifting Fog.

2020

92-96

2020 Jan

2150-136X

<p>Fontan-associated protein losing enteropathy is a challenge to treat and is a major contributor to morbidity and mortality in patients with palliated single ventricle. Numerous strategies for management have been proposed, with confusion as to how best to stratify and implement care among the many treatments available. Medical management can be helpful in some, while novel lymphatic interventions hold potential for remission with good results. We review our institutional approach to protein losing enteropathy after Fontan operation and provide a suggested algorithm and pathway for effective care.</p>

10.1177/2150135119890555

World J Pediatr Congenit Heart Surg

31835975

Evaluating the utility of routine screening catheterisation before interstage discharge of infants with single-ventricle physiology.

2019

1-7

2019 May 30

1467-1107

<p><strong>INTRODUCTION: </strong>Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes.</p>

<p><strong>METHODS: </strong>Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves.</p>

<p><strong>RESULTS: </strong>There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p &lt; 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64.</p>

<p><strong>CONCLUSION: </strong>Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.</p>

10.1017/S1047951119000696

Cardiol Young

31142394

Imaging of central lymphatic abnormalities in Noonan syndrome.

2019

2019 Jan 06

1432-1998

<p><strong>BACKGROUND: </strong>Children with Noonan syndrome are known to have increased risk for lymphatic disorders, the extent and nature of which are poorly understood.</p>

<p><strong>OBJECTIVE: </strong>Our objective was to describe the imaging findings of the central lymphatic abnormalities in children with Noonan syndrome who underwent central lymphatic imaging.</p>

<p><strong>MATERIALS AND METHODS: </strong>We conducted a single-center retrospective review of all children with a confirmed history of Noonan syndrome who presented for lymphatic imaging over a 5-year period. Imaging evaluation was performed on unenhanced T2-weighted (T2-W) imaging, dynamic-contrast MR lymphangiography or conventional lymphangiography. Two readers evaluated the imaging in consensus for the distribution of fluid on T2-W imaging and for lymphatic flow of intranodal contrast agent and thoracic duct abnormalities on dynamic-contrast MR lymphangiography and conventional lymphangiography. We performed a chart review for clinical history and outcomes.</p>

<p><strong>RESULTS: </strong>We identified a total of 10 children, all but one of whom had congenital heart disease. Presenting symptoms included chylothorax (n=9) and ascites (n=1). Nine had T2-W imaging, seven had dynamic-contrast MR lymphangiography, and seven had conventional lymphangiography. All with T2-W imaging had pleural effusions. On both dynamic-contrast MR lymphangiography and conventional lymphangiography, perfusion to the lung was seen (n=6), with intercostal flow also seen on dynamic-contrast MR lymphangiography (n=6). The thoracic duct was not present in three children and the central thoracic duct was not present in three. A double thoracic duct was seen in two children.</p>

<p><strong>CONCLUSION: </strong>Children with Noonan syndrome and clinical evidence of lymphatic dysfunction have central lymphatic abnormalities characterized by retrograde intercostal flow, pulmonary lymphatic perfusion, and thoracic duct abnormalities.</p>

10.1007/s00247-018-04337-6

Pediatr Radiol

30613845

Emergency Department Visits by Children With Congenital Heart Disease.

2018

1817-1825

2018 Oct 09

1558-3597

<p><strong>BACKGROUND: </strong>Data related to the epidemiology and resource utilization of congenital heart disease (CHD)-related emergency department (ED) visits in the pediatric population is limited.</p>

<p><strong>OBJECTIVES: </strong>The purpose of this analysis was to describe national estimates of pediatric CHD-related ED visits and evaluate medical complexity, admissions, resource utilization, and mortality.</p>

<p><strong>METHODS: </strong>This was an epidemiological analysis of ED visit-level data from the 2006 to 2014 Nationwide Emergency Department Sample. Patients age&nbsp;&lt;18 years with CHD were identified using International Classification of Diseases-9th Revision-Clinical Modification codes. We evaluated time trends using weighted regression and tested the hypothesis that medical complexity, resource utilization, and mortality are higher in CHD patients.</p>

<p><strong>RESULTS: </strong>A total of 420,452 CHD-related ED visits (95% confidence interval [CI]: 416,897 to 422,443 visits) were identified, accounting for 0.17% of all pediatric ED visits. Those with CHD were more likely to be&nbsp;&lt;1 year of age (43% vs. 13%), and to have&nbsp;≥1 complex chronic condition (35% vs. 2%). CHD-related ED visits had higher rates of inpatient admission (46% vs. 4%; adjusted odds ratio: 1.89; 95% CI: 1.85 to 1.93), higher median ED charges ($1,266 [interquartile range (IQR): $701 to $2,093] vs. $741 [IQR: $401 to $1,332]), and a higher mortality rate (1% vs. 0.04%; adjusted odds ratio: 1.25; 95% CI: 1.07 to 1.45). Adjusted median charges for CHD-related ED visits increased from $1,219 (IQR: $673 to $2,138) to $1,630 (IQR: $901 to $2,799), while the mortality rate decreased from 1.13% (95% CI: 0.71% to 1.52%) to 0.75% (95% CI: 0.41% to 1.09%) over the 9 years studied.</p>

<p><strong>CONCLUSIONS: </strong>Children with CHD presenting to the ED represent a medically complex population at increased risk for morbidity, mortality, and resource utilization compared with those without CHD. Over 9 years, charges increased, but the mortality rate improved.</p>

10.1016/j.jacc.2018.07.055

J. Am. Coll. Cardiol.

30286926

Accuracy of transesophageal echocardiography in the identification of postoperative intramural ventricular septal defects.

2016

688-95

2016 09

1097-685X

<p><strong>BACKGROUND: </strong>Intramural ventricular septal defects (VSDs), residual interventricular communications occurring after repair of conotruncal defects, are associated with poor postoperative outcomes. The ability of intraoperative transesophageal echocardiography (TEE) to identify intramural VSDs has not yet been evaluated.</p>

<p><strong>METHODS: </strong>Intraoperative TEE and postoperative transthoracic echocardiography (TTE) data in all patients undergoing all biventricular repair of conotruncal anomalies in our hospital between January 1, 2006, and June 30, 2013, were reviewed. The ability of TEE to accurately identify residual defects was assessed using postoperative TTE as the reference imaging modality.</p>

<p><strong>RESULTS: </strong>Intramural VSDs occurred in 34 of 337 patients evaluated; 19 were identified by both TTE and TEE, and 15 were identified by TTE only. Sensitivity was 56% and specificity was 100% for TEE to identify intramural VSDs. Peripatch VSDs were identified in 90 patients by both TTE and TEE, in 53 by TTE only, and in 15 by TEE only, yielding a sensitivity of 63% and specificity of 92%. Of the VSDs requiring catheterization or surgical reintervention, 6 of 7 intramural VSDs and all 5 peripatch VSDs were identified by intraoperative TEE. TEE guided the intraoperative decision to return to cardiopulmonary bypass (CPB) in an attempt to close residual defects in 12 patients with intramural VSDs and in 4 patients with peripatch VSDs seen after initial CPB; of these, 10 intramural VSDs and all 4 peripatch VSDs resolved or became smaller on final intraoperative TEE.</p>

<p><strong>CONCLUSIONS: </strong>TEE has modest sensitivity but high specificity for identifying intramural VSDs and can detect most defects requiring reintervention. Repeat attempts at closure in the index operation may successfully correct intramural VSDs identified by TEE.</p>

10.1016/j.jtcvs.2016.04.026

J. Thorac. Cardiovasc. Surg.

27183884