Intervention Codesign in the Pediatric Cardiac Intensive Care Unit to Improve Family Meetings.

2022

2022 Mar 23

1873-6513

<p><strong>CONTEXT: </strong>Family meetings are encouraged in the pediatric cardiac intensive care unit (CICU) with the expectation of supporting parental shared decision-making (SDM). However, they often fall short of this goal. Additionally, interprofessional team and family meetings are dominated by input from physicians, under-utilizing the skillset of the full clinical team.</p>

<p><strong>OBJECTIVES: </strong>1) To determine feasibility of a codesign process to optimize the preparation of the interprofessional team and parents for conducting SDM-oriented family meetings in the CICU, and 2) to describe the resulting elements of the intervention including new support documents for the team and family to prepare for the meeting, team member roles in the meeting, and optimization of communication skills.</p>

<p><strong>METHODS: </strong>Experience-based codesign was used with CICU clinicians and parents of children hospitalized in the CICU to develop an intervention at a single institution. Sessions were audio recorded and transcribed and analyzed using modified grounded theory. Participants were surveyed about their engagement in the codesign process to assess feasibility.</p>

<p><strong>RESULTS: </strong>Fifteen professionals and 6 parents enrolled in the codesign and endorsed engagement in the process and importance of the intervention elements. Participants identified the benefit of complementary parent and team preparation for family meetings noting 5 distinct types of meetings that occurred frequently. Documents, processes, and skills training were developed to improve interprofessional teamwork regarding shared decision making and support of parents in family meetings.</p>

<p><strong>CONCLUSION: </strong>A codesign of an intervention with clinicians and parents in the CICU is a feasible and resulted in an intervention with broad support among clinicians in the CICU.</p>

10.1016/j.jpainsymman.2022.03.010

J Pain Symptom Manage

35339610

Association Between Children With Life-Threatening Conditions and Their Parents' and Siblings' Mental and Physical Health.

2021

e2137250

2021 Dec 01

2574-3805

<p><strong>Importance: </strong>Despite concerns regarding the potential deleterious physical and mental health outcomes among family members of a child with a life-threatening condition (LTC), few studies have examined empirical measures of health outcomes among these family members.</p>

<p><strong>Objectives: </strong>To examine whether mothers, fathers, sisters, and brothers of children with 1 of 4 types of pediatric LTCs have higher rates of health care encounters, diagnoses, and prescriptions compared with families of children without these conditions.</p>

<p><strong>Design, Setting, and Participants: </strong>This retrospective cohort study included US families with commercial insurance coverage from a single carrier. Children who had 1 of 4 LTCs (substantial prematurity, critical congenital heart disease, cancer, or a condition resulting in severe neurologic impairment) were identified by a diagnosis in their insurance claim data between July 1, 2015, and June 30, 2016. Each case child and their family was matched with up to 4 control children and their families based on the age of the case and control children. Data were analyzed between August 2020 and March 2021.</p>

<p><strong>Exposures: </strong>Having a child or sibling with substantial prematurity, critical congenital heart disease, cancer, or a condition resulting in severe and progressive neurologic impairment.</p>

<p><strong>Main Outcomes: </strong>Rates of occurrence of health care encounters, physical and mental health diagnoses, and physical and mental health medication prescriptions, identified from insurance claims data, were compared between case and control families using a multivariable negative binomial regression model. The statistical analysis adjusted for observed differences between case and control families and accounted for clustering at the family level.</p>

<p><strong>Results: </strong>The study included 25 528 children (6909 case children [27.1%] and 18 619 control children [72.9%]; median age, 6.0 years [IQR, 1-13 years]; 13 294 [52.1%] male), 43 357 parents (11 586 case parents [26.7%] and 31 771 control parents [73.3%]; mean [SD] age, 40.4 [8.1] years; 22 318 [51.5%] female), and 25 706 siblings (7664 case siblings [29.8%] and 18 042 control siblings [70.2%]; mean [SD] age, 12.1 [6.5] years; 13 114 [51.0%] male). Overall, case mothers had higher rates of the composite outcome of health care encounters, diagnoses, and prescriptions compared with control mothers (incident rate ratio [IRR], 1.61; 95% CI, 1.54-1.68), as did case fathers compared with control fathers (IRR, 1.55; 95% CI, 1.46-1.64). Sisters of children with LTCs had higher rates of the composite outcome compared with sisters of children without LTCs (IRR, 1.68; 95% CI, 1.55-1.82), as did brothers of children with LTCs compared with brothers of children without LTCs (IRR, 1.70; 95% CI, 1.56-1.85).</p>

<p><strong>Conclusions and Relevance: </strong>In this cohort study, mothers, fathers, sisters, and brothers who had a child or sibling with 1 of 4 types of LTCs had higher rates of health care encounters, diagnoses, and medication prescriptions compared with families who did not have a child with that condition. The findings suggest that family members of children with LTCs may experience poorer mental and physical health outcomes. Interventions for parents and siblings of children with LTCs that aim to safeguard their mental and physical well-being appear to be warranted.</p>

10.1001/jamanetworkopen.2021.37250

JAMA Netw Open

34928360

Post-operative Chylothorax in Patients with Repaired Transposition of the Great Arteries.

2021

2021 Nov 29

1432-1971

<p>Patients with dextro-transposition of the great arteries (d-TGA) require surgical repair as neonates. These patients are at risk for post-operative chylothorax. We sought to describe the presentation, imaging, and outcomes after intervention for patients with d-TGA with post-operative chylothorax. A retrospective chart review was performed in patients with repaired d-TGA who were referred from 1/1/2013 to 4/1/2020 for evaluation of chylothorax. Patient history, lymphatic imaging, and interventional data were collected. Impact of intervention on lymphatic drainage was evaluated with a student's t-test. Eight patients met inclusion criteria for this study. Five patients had a history of central venous thrombus leading to thoracic duct outlet occlusion. Five patients underwent intervention, two were managed conservatively, and one was not a candidate for intervention. Chylothorax resolved in six patients. There was a significant difference in output from 7&nbsp;days prior to first intervention (114&nbsp;mL/kg/day) compared to 28&nbsp;days following final intervention (27&nbsp;mL/kg/day, p = 0.034). There were no procedural complications. Chylothorax in patients with repaired transposition of the great arteries is often amenable to intervention. Early surveillance and management of central venous thrombosis may reduce the burden of lymphatic disease in these patients.</p>

10.1007/s00246-021-02774-z

Pediatr Cardiol

34841467

Intrahepatic Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography: Potential Imaging Signature for Protein-Losing Enteropathy in Congenital Heart Disease.

2021

e021542

2021 Sep 25

2047-9980

<p>Background Protein-losing enteropathy (PLE) is a significant cause of morbidity and mortality in congenital heart disease patients with single ventricle physiology. Intrahepatic dynamic contrast-enhanced magnetic resonance lymphangiography (IH-DCMRL) is a novel diagnostic technique that may be useful in characterizing pathologic abdominal lymphatic flow in the congenital heart disease population and in diagnosing PLE. The objective of this study was to characterize differences in IH-DCMRL findings in patients with single ventricle congenital heart disease with and without PLE. Methods and Results This was a single-center retrospective study of IH-DCMRL findings and clinical data in 41 consecutive patients, 20 with PLE and 21 without PLE, with single ventricle physiology referred for lymphatic evaluation. There were 3 distinct duodenal imaging patterns by IH-DCMRL: (1) enhancement of the duodenal wall with leakage into the lumen, (2) enhancement of the duodenal wall without leakage into the lumen, and (3) no duodenal involvement. Patients with PLE were more likely to have duodenal involvement on IH-DCMRL than patients without PLE (&lt;0.001). Conclusions IH-DCMRL findings of lymphatic enhancement of the duodenal wall and leakage of lymph into the duodenal lumen are associated with PLE. IH-DCMRL is a useful new modality for characterizing pathologic abdominal lymphatic flow in PLE and might be useful as a risk-assessment tool for PLE in at-risk patients.</p>

10.1161/JAHA.121.021542

J Am Heart Assoc

34569246

Liver lymphatic anatomy and role in systemic lymphatic disease.

2021

2021 Jun 24

1432-1084

<p><strong>OBJECTIVES: </strong>To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention.</p>

<p><strong>METHODS: </strong>In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed.</p>

<p><strong>RESULTS: </strong>During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p &lt; .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p &lt; .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6&nbsp;months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics.</p>

<p><strong>CONCLUSION: </strong>We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal.</p>

<p><strong>KEY POINTS: </strong>• We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.</p>

10.1007/s00330-021-08098-z

Eur Radiol

34165621

Use of Contrast-Enhanced Ultrasound to Determine Thoracic Duct Patency.

2020

2020 Sep 09

1535-7732

<p>The aim of this study was to determine the feasibility of using contrast-enhanced ultrasound (CEUS) evaluation to determine thoracic duct (TD) outlet patency. Nine patients referred for lymphatic imaging and intervention underwent percutaneous intranodal ultrasound contrast injection and conventional lymphangiography (CL). Eight of 9 patients had a patent TD by CEUS and CL. One patient did not have a patent TD. There was 100% agreement between CEUS and CL. These results suggest that CEUS is an imaging modality that might be as accurate as CL in determining TD patency.</p>

10.1016/j.jvir.2020.05.022

J Vasc Interv Radiol

32919824

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

Predicting and Surviving Prolonged Critical Illness After Congenital Heart Surgery.

2020

e557-e564

2020 Jul

1530-0293

<p><strong>OBJECTIVES: </strong>Prolonged critical illness after congenital heart surgery disproportionately harms patients and the healthcare system, yet much remains unknown. We aimed to define prolonged critical illness, delineate between nonmodifiable and potentially preventable predictors of prolonged critical illness and prolonged critical illness mortality, and understand the interhospital variation in prolonged critical illness.</p>

<p><strong>DESIGN: </strong>Observational analysis.</p>

<p><strong>SETTING: </strong>Pediatric Cardiac Critical Care Consortium clinical registry.</p>

<p><strong>PATIENTS: </strong>All patients, stratified into neonates (≤28 d) and nonneonates (29 d to 18 yr), admitted to the pediatric cardiac ICU after congenital heart surgery at Pediatric Cardiac Critical Care Consortium hospitals.</p>

<p><strong>INTERVENTIONS: </strong>None.</p>

<p><strong>MEASUREMENTS AND MAIN RESULTS: </strong>There were 2,419 neonates and 10,687 nonneonates from 22 hospitals. The prolonged critical illness cutoff (90th percentile length of stay) was greater than or equal to 35 and greater than or equal to 10 days for neonates and nonneonates, respectively. Cardiac ICU prolonged critical illness mortality was 24% in neonates and 8% in nonneonates (vs 5% and 0.4%, respectively, in nonprolonged critical illness patients). Multivariable logistic regression identified 10 neonatal and 19 nonneonatal prolonged critical illness predictors within strata and eight predictors of mortality. Only mechanical ventilation days and acute renal failure requiring renal replacement therapy predicted prolonged critical illness and prolonged critical illness mortality in both strata. Approximately 40% of the prolonged critical illness predictors were nonmodifiable (preoperative/patient and operative factors), whereas only one of eight prolonged critical illness mortality predictors was nonmodifiable. The remainders were potentially preventable (postoperative critical care delivery variables and complications). Case-mix-adjusted prolonged critical illness rates were compared across hospitals; six hospitals each had lower- and higher-than-expected prolonged critical illness frequency.</p>

<p><strong>CONCLUSIONS: </strong>Although many prolonged critical illness predictors are nonmodifiable, we identified several predictors to target for improvement. Furthermore, we observed that complications and prolonged critical care therapy drive prolonged critical illness mortality. Wide variation of prolonged critical illness frequency suggests that identifying practices at hospitals with lower-than-expected prolonged critical illness could lead to broader quality improvement initiatives.</p>

10.1097/CCM.0000000000004354

Crit. Care Med.

32574468

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