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

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

Interprofessional Team Member Communication Patterns, Teamwork, and Collaboration in Pre-Family Meeting Huddles in a Pediatric Cardiac Intensive Care Unit.

2019

2019 Apr 17

1873-6513

<p><strong>CONTEXT: </strong>Interprofessional teams often develop a care plan prior to engaging in a family meeting in the pediatric cardiac intensive care unit (CICU)-a process that can affect the course of the family meeting and alter team dynamics, but that has not been studied.</p>

<p><strong>OBJECTIVES: </strong>To characterize the types of interactions that interprofessional team members have in pre-family meetings huddles in the pediatric CICU by 1) evaluating the amount of time each team member speaks; 2) assessing team communication and teamwork using standardized instruments; and 3) measuring team members' perceptions of collaboration and satisfaction with decision-making.</p>

<p><strong>METHODS: </strong>We conducted a prospective observational study in a pediatric CICU. Subjects were members of the interprofessional team attending preparation meetings prior to care meetings with families of patients admitted to the CICU for longer than two weeks. We quantitatively coded the amount each team member spoke. We assessed team performance of communication and teamwork using the PACT-Novice tool, and we measured perception of collaboration and satisfaction with decision-making using the CSACD questionnaire.</p>

<p><strong>RESULTS: </strong>Physicians spoke for an average of 83.9% of each meeting's duration (SD 7.5%); non-physicians averaged 9.9% (SD 5.2%). Teamwork behaviors were present and adequately performed as judged by trained observers. Significant differences in physician and non-physician perceptions of collaboration were found in 3 out of 10 observed meetings.</p>

<p><strong>CONCLUSIONS: </strong>Interprofessional team members' interactions in team meetings provide important information about team dynamics, revealing potential opportunities for improved collaboration and communication in team meetings and subsequent family meetings.</p>

10.1016/j.jpainsymman.2019.04.009

J Pain Symptom Manage

31004773

MRI Evaluation of Lymphatic Abnormalities in the Neck and Thorax after Fontan Surgery: Relationship with Outcome.

2019

180877

2019 Apr 02

1527-1315

<p>Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. Purpose To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. Materials and Methods Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. Results A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P &lt; .01). Conclusion Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.</p>

10.1148/radiol.2019180877

Radiology

30938628

Interprofessional Teamwork During Family Meetings in the Pediatric Cardiac Intensive Care Unit.

2019

2019 Mar 12

1873-6513

<p><strong>BACKGROUND: </strong>Parents of children in the pediatric cardiac intensive care unit (CICU) report inadequate communication and a lack of empathy during conversations with their clinicians.</p>

<p><strong>OBJECTIVE: </strong>To assess quantitatively and qualitatively the contributions made by team members of different professions in communicating with parents during family meetings.</p>

<p><strong>DESIGN: </strong>Prospective observational study.</p>

<p><strong>SETTING/SUBJECTS: </strong>The pediatric CICU at the Children's Hospital of Philadelphia. Subjects were members of the interprofessional team attending family meetings for patients admitted to the CICU longer than two weeks.</p>

<p><strong>MEASUREMENTS: </strong>We used quantitative conversation attribution and coding to compare durations of attendee contributions and contribution type by professional role. The SCOPE codebook and other quantitative codes drawn from best practices in family meetings were used to measure communication behaviors. A qualitative analysis of nurses' and social workers' contributions was used to identify themes not otherwise captured.</p>

<p><strong>RESULTS: </strong>Across 10 meetings, physicians spoke for an average of 78.1% (SD 10.7%) of each meeting, non-physicians 9.6% (SD 7.8%), and parents 17.4% (SD 12.2%). Parental understanding was assessed an average of 0.2 (SD 0.4) times per meeting. Parents expressed emotion an average of 4.2 times per meeting (SD 7.1) and the clinical team responded empathetically 2.2 times per meeting (SD 4.3). All clinician empathic responses were a minority of their overall contributions. Conversation was almost exclusively between physicians and families until physicians indicated other team members could contribute.</p>

<p><strong>CONCLUSIONS: </strong>Coordination of team members' roles in the meetings may improve parental engagement necessary for decision-making and empathic responses that are often missed.</p>

10.1016/j.jpainsymman.2019.03.002

J Pain Symptom Manage

30876957