Multidisciplinary approach to patients with lymphatic conduction disorders.

Patients with lymphatic disorders are remarkably complex and require a wide variety of medical and surgical services. Establishing a multidisciplinary program improves the efficiency of the patients' hospital experience minimizing the compartmentalization of their care. Offering a clear intake process guarantees that patients will be seen promptly by all the required teams. Additionally, having regular multidisciplinary meetings allows all participating teams to learn from each other and gain experience in the care of a population that is extraordinarily heterogeneous. Additionally, establishing a solid program allows for long-term data collection, research and education.

A Pediatric Interprofessional Cardiac Intensive Care Unit Intervention: CICU Teams and Loved Ones Communicating (CICU TALC) is Feasible, Acceptable, and Improves Clinician Communication Behaviors in Family Meetings.

Parents of children in the pediatric cardiac intensive care unit (CICU) are often unprepared for family meetings (FM). Clinicians often do not follow best practices for communicating with families, adding to distress. An interprofessional team intervention for FM is feasible, acceptable, and positively impacts family preparation and conduct of FM in the CICU. We implemented a family- and team-support intervention for conducting FM and conducted a pretest-posttest study with parents of patients selected for a FM and clinicians. We measured feasibility, fidelity to intervention protocol, and parent acceptability via questionnaire and semi-structured interviews. Clinician behavior in meetings was assessed through semantic content analyses of meeting transcripts tracking elicitation of parental concerns, questions asked of parents, and responses to parental empathic opportunities. Logistic and ordinal logistic regression assessed intervention impact on clinician communication behaviors in meetings comparing pre- and post-intervention data. Sixty parents (95% of approached) were enrolled, with collection of 97% FM and 98% questionnaire data. We accomplished > 85% fidelity to intervention protocol. Most parents (80%) said the preparation worksheet had the right amount of information and felt positive about families receiving this worksheet. Clinicians were more likely to elicit parental concerns (adjusted odds ratio = 3.42; 95%CI [1.13, 11.0]) in post-intervention FM. There were no significant differences in remaining measures. Implementing an interprofessional team intervention to improve family preparation and conduct of FM is locally feasible, acceptable, and changes clinician behaviors. Future research should assess broader impact of training on clinicians, patients, and families.

Critical care management of patients with lymphatic conduction disorders.

Lymphatic dysfunction in critical illness is complex. Primary complex lymphatic anomalies can lead to profound organ dysfunction, particularly respiratory failure and shock. Critical illness, the complications of critical illness, and the procedures and therapies used to treat critical illness, can lead to secondary lymphatic dysfunction. This is most often seen with congenital and acquired cardiovascular disease and respiratory disease. The critical care management of these patients requires an expert multidisciplinary team.

Mechanical Ventilation and Outcomes of Children Who Undergo Ventricular Assist Device Placement: 2014-2020 Linked Analysis From the Advanced Cardiac Therapies Improving Outcomes Network and Pediatric Cardiac Critical Care Consortium Registries.

OBJECTIVES: Placement of a ventricular assist device (VAD) improves outcomes in children with advanced heart failure, but adverse events remain important consequences. Preoperative mechanical ventilation (MV) increases mortality, but it is unknown what impact prolonged postoperative MV has. DESIGN: Advanced Cardiac Therapies Improving Outcomes Network (ACTION) and Pediatric Cardiac Critical Care Consortium (PC4) registries were used to identify and link children with initial VAD placement admitted to the cardiac ICU (CICU) from August 2014 to July 2020. Demographics, cardiac diagnosis, preoperative and postoperative CICU courses, and outcomes were compiled. Univariable and multivariable statistics assessed association of patient factors with prolonged postoperative MV. Multivariable logistic regression sought independent associations with outcomes. SETTING: Thirty-five pediatric CICUs across the United States and Canada. PATIENTS: Children on VADs included in both registries. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two hundred forty-eight ACTION subjects were linked to a matching patient in PC4. Median (interquartile) age 7.7 years (1.5-15.5 yr), weight 21.3 kg (9.1-58 kg), and 56% male. Primary diagnosis was congenital heart disease (CHD) in 35%. Pre-VAD explanatory variables independently associated with prolonged postoperative MV included: age (incidence rate ratio [IRR], 0.95; 95% CI, 0.93-0.96; p < 0.01); preoperative MV within 48 hours (IRR, 2.76; 95% CI, 1.59-4.79; p < 0.01), 2-7 days (IRR, 1.82; 95% CI, 1.15-2.89; p = 0.011), and greater than 7 days before VAD implant (IRR, 2.35; 95% CI, 1.62-3.4; p < 0.01); and CHD (IRR, 1.96; 95% CI, 1.48-2.59; p < 0.01). Each additional day of postoperative MV was associated with greater odds of mortality (odds ratio [OR], 1.09 per day; p < 0.01) in the full cohort. We identified an associated greater odds of mortality in the 102 patients with intracorporeal devices (OR, 1.24; 95% CI, 1.04-1.48; p = 0.014), but not paracorporeal devices (77 patients; OR, 1.04; 95% CI, 0.99-1.09; p = 0.115). CONCLUSIONS: Prolonged MV after VAD placement is associated with greater odds of mortality in intracorporeal devices, which may indicate inadequacy of cardiopulmonary support in this group. This linkage provides a platform for future analyses in this population.

Programmatic Palliative Care Consultations in Pediatric Heart Transplant Evaluations.

Guidelines advocate for integrating palliative care into the management of heart failure (HF) and of children with life-limiting disease. The potential impact of palliative care integration into pediatric HF on patient-centered outcomes is poorly understood. The present study sought to assess the association of programmatic implementation of palliative care into the heart transplant evaluation process with hospital-free days (HFD) and end of life (EOL) treatment choices. The study included patients less than 19 years of age who underwent a heart transplant evaluation between February 2012 and April 2020 at a single center. Patients evaluated in the programmatic palliative care (PPC) era (January 2016-April 2020) were compared to patients evaluated in the pre-PPC era (February 2012-December 2015). The study included 188 patients, with 91 (48%) in the PPC era and 97 (52%) in the pre-PCC era. Children < 1 year of age at the time of the evaluation represented 32% of the cohort. 52% of patients had single ventricle physiology. PPC was not significantly associated with increased HFD (IRR 0.94 [95% CI 0.79-1.2]). PPC was however associated with intensity of EOL care with decreased mechanical ventilation (OR 0.12 [95% CI 0.02-0.789], p = 0.03) and decreased use of ionotropic support (OR 0.13 [95% CI 0.02-0.85], p =0.03). PPC in pediatric heart transplant evaluations may be associated with less invasive interventions at EOL.

Parental communication satisfaction with the clinical team in the paediatric cardiac ICU.

INTRODUCTION: Understanding parents' communication preferences and how parental and child characteristics impact satisfaction with communication is vital to mitigate communication challenges in the cardiac ICU. METHODS: This cross-sectional survey was conducted from January 2019 to March 2020 in a paediatric cardiac ICU with parents of patients admitted for at least two weeks. Family satisfaction with communication with the medical team was measured using the Communication Assessment Tool for Team settings. Clinical characteristics were collected via Epic, Pediatric Cardiac Critical Care Consortium local entry and Society for Thoracic Surgeons Congenital Heart Surgery Databases. Associations between communication score and parental mood, stress, perceptions of clinical care, and demographic characteristics along with patient demographic and clinical characteristics were examined. Multivariable ordinal models were conducted with characteristics significant in bivariate analysis. RESULTS: In total, 93 parents of 84 patients (86% of approached) completed surveys. Parents were 63% female and 70% White. Seventy per cent of patients were <6 months old at admission, 25% had an extracardiac abnormality, and 80% had a cardiac surgery this admission. Parents of children with higher pre-surgical risk of mortality scores (OR 2.875; 95%CI 1.076-7.678), presence of surgical complications (72 [63.0, 75.0] vs. 64 [95%CI 54.6, 73] (p = 0.0247)), and greater satisfaction with care in the ICU (r = 0.93922; p < 0.0001) had significantly higher communication scores. CONCLUSION: These findings can prepare providers for scenarios with higher risk for communication challenges and demonstrate the need for further investigation into interventions that reduce parental anxiety and improve communication for patients with unexpected clinical trajectories.

Current practices for refractory chylothorax following congenital heart surgery.

INTRODUCTION: Chylothorax following paediatric cardiac surgery is associated with significant morbidity, particularly those that are refractory to conservative therapy. It is our impression that there is important variability in the medical, surgical, and interventional therapies used to manage refractory chylothorax between congenital heart programmes. We therefore conducted a survey study of current practices for managing refractory chylothorax. METHODS: The Chylothorax Work Group, formed with the support of the Pediatric Cardiac Critical Care Consortium, designed this multi-centre survey study with a focus on the timing and indication for utilising known therapies for refractory chylothorax. The survey was sent to one chylothorax expert from each Work Group centre, and results were summarised and reported as the frequency of given responses. RESULTS: Of the 20 centres invited to participate, 17 (85%) submitted complete responses. Octreotide (13/17, 76%) and sildenafil (8/17, 47%) were the most utilised medications. Presently, 9 (53%) centres perform pleurodesis, 15 (88%) perform surgical thoracic duct ligation, 8 (47%) perform percutaneous lymphatic interventions, 6 (35%) utilise thoracic duct decompression procedures, and 3 (18%) perform pleuroperitoneal shunts. Diagnostic lymphatic imaging is performed prior to surgical thoracic duct ligation in only 7 of the 15 (47%) centres that perform the procedure. Respondents identified barriers to referring and transporting patients to centres with expertise in lymphatic interventions. CONCLUSIONS: There is variability in the treatment of refractory post-operative chylothorax across a large group of academic heart centres. Few surveyed heart centres have replaced surgical thoracic duct ligation or pleurodesis with image-guided selective lymphatic interventions.

Hereditary haemorrhagic telangiectasia and SMAD4 mutation in a patient with complex single ventricle heart disease.

We report a case of hypoplastic left heart syndrome and with subsequent aortopathy and then found to have hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome due to a germline SMAD4 pathologic variant. The patient's staged palliation was complicated by the development of neoaortic aneurysms, arteriovenous malformations, and gastrointestinal bleeding thought to be secondary to Fontan circulation, but workup revealed a SMAD4 variant consistent with hereditary haemorrhagic telangiectasia/juvenile polyposis syndrome. This case underscores the importance of genetic modifiers in CHD, especially those with Fontan physiology.

Anesthesia and Sedation Exposure and Neurodevelopmental Outcomes in Infants Undergoing Congenital Cardiac Surgery: A Retrospective Cohort Study.

BACKGROUND: Children undergoing complex cardiac surgery are exposed to substantial cumulative doses of sedative medications and volatile anesthetics and are more frequently anesthetized with ketamine, compared with healthy children. This study hypothesized that greater exposure to sedation and anesthesia in this population is associated with lower neurodevelopmental scores at 18 months of age. METHODS: A secondary analysis was conducted of infants with congenital heart disease who participated in a prospective observational study of environmental exposures and neurodevelopmental outcomes to assess the impact of cumulative volatile anesthetic agents and sedative medications. Cumulative minimum alveolar concentration hours of exposure to volatile anesthetic agents and all operating room and intensive care unit exposures to sedative and anesthesia medications were collected before administration of Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley III), at 18 months of age. RESULTS: The study cohort included 41 (37%) single-ventricle and 69 (63%) two-ventricle patients. Exposures to volatile anesthetic agents, opioids, benzodiazepines, and dexmedetomidine were not associated with abnormal Bayley III scores. At 18-month follow-up, after adjusting for confounders, each mg/kg increase in ketamine exposure was associated with a 0.34 (95% CI, -0.64 to -0.05) point decrease in Bayley III motor scores (P = 0.024). CONCLUSIONS: Total cumulative exposures to volatile anesthetic agents were not associated with neurodevelopmental impairment in infants with congenital heart disease undergoing various imaging studies and procedures, whereas higher ketamine doses were associated with poorer motor performance.

Stratifying Severity of Acute Respiratory Failure Severity in Cyanotic Congenital Heart Disease.

Hypoxemia is used to stratify severity in acute respiratory failure (ARF) but is less useful in cyanotic congenital heart disease (CCHD) due to an inability to differentiate hypoxemia from lung injury versus cardiac shunting. Therefore, we aimed to determine whether variables related to respiratory mechanics were associated with outcomes to assist in stratifying ARF severity in pediatric CCHD. We performed a retrospective cohort study from a single cardiac intensive care unit enrolling children with CCHD with ARF requiring mechanical ventilation between 2011 and 2019. Time-averaged ventilator settings and oxygenation data in the first 24 h of ARF were screened for association with the primary outcome of 28-day mortality. Of 344 eligible patients, peak inspiratory pressure (PIP) and driving pressure (ΔP) were selected as candidate variables to stratify ARF severity. PIP (OR 1.10, 95% CI 1.02-1.19) and ΔP (1.11, 95% CI 1.01-1.24) were associated with higher mortality and fewer ventilator-free days (VFDs) at 28 days after adjusting for age, severity of cardiac history, and FiO2. A three-level (mild, moderate, severe) severity stratification was established for both PIP (≤ 20, 21-29, ≥ 30) and ΔP (≤ 16, 17-24, ≥ 25), showing increasing mortality (both P < 0.01), decreasing VFDs and increasing ventilator days in survivors (all P < 0.05) across increasing pressures. Overall, we found that higher PIP and ΔP were associated with mortality and duration of ventilation across a three-level severity stratification system in pediatric CCHD with ARF, providing a practical method to prognosticate in subjects with multifactorial etiologies for hypoxemia.

Occlusion Pressure of the Thoracic Duct in Fontan Patients With Lymphatic Failure: Does Dilatation Challenge Contractility?

BACKGROUND: The Fontan circulation challenges the lymphatic system. Increasing production of lymphatic fluid and impeding lymphatic return, increased venous pressure may cause lymphatic dilatation and decrease lymphatic contractility. In-vitro studies have reported a lymphatic diameter-tension curve, with increasing passive stretch affecting the intrinsic contractile properties of each thoracic duct segment. We aimed to describe thoracic duct occlusion pressure and asses if thoracic duct dilation impairs contractility in individuals with a Fontan circulation and lymphatic failure. METHODS: Central venous pressure and thoracic duct measurements were retrospectively collected from 31 individuals with a Fontan circulation. Thoracic duct occlusion pressure was assessed during a period of external manual compression and used as an indicator of lymphatic vessel contractility. Measurements of pressure were correlated with measurements of the thoracic duct diameter in images obtained by dynamic contrast-enhanced MR lymphangiography. RESULTS: The average central venous pressure and average pressure of the thoracic duct were 17 mm Hg. During manual occlusion, the thoracic duct pressure significantly increased to 32 mm Hg. The average thoracic duct diameter was 3.3 mm. Thoracic duct diameter correlated closely with the central venous pressure. The rise in pressure following manual occlusion showed an inverse correlation with the diameter of the thoracic duct. CONCLUSION: Higher central venous pressures are associated with increasing diameters of the thoracic duct. When challenged by manual occlusion, dilated thoracic ducts display a decreased ability to increase pressure. Dilatation and a resulting decreased contractility may partly explain the challenged lymphatic system in individuals with a Fontan circulation.

Development of consensus recommendations for the management of post-operative chylothorax in paediatric CHD.

OBJECTIVE: A standardised multi-site approach to manage paediatric post-operative chylothorax does not exist and leads to unnecessary practice variation. The Chylothorax Work Group utilised the Pediatric Critical Care Consortium infrastructure to address this gap. METHODS: Over 60 multi-disciplinary providers representing 22 centres convened virtually as a quality initiative to develop an algorithm to manage paediatric post-operative chylothorax. Agreement was objectively quantified for each recommendation in the algorithm by utilising an anonymous survey. "Consensus" was defined as ≥ 80% of responses as "agree" or "strongly agree" to a recommendation. In order to determine if the algorithm recommendations would be correctly interpreted in the clinical environment, we developed ex vivo simulations and surveyed patients who developed the algorithm and patients who did not. RESULTS: The algorithm is intended for all children (<18 years of age) within 30 days of cardiac surgery. It contains rationale for 11 central chylothorax management recommendations; diagnostic criteria and evaluation, trial of fat-modified diet, stratification by volume of daily output, timing of first-line medical therapy for "low" and "high" volume patients, and timing and duration of fat-modified diet. All recommendations achieved "consensus" (agreement >80%) by the workgroup (range 81-100%). Ex vivo simulations demonstrated good understanding by developers (range 94-100%) and non-developers (73%-100%). CONCLUSIONS: The quality improvement effort represents the first multi-site algorithm for the management of paediatric post-operative chylothorax. The algorithm includes transparent and objective measures of agreement and understanding. Agreement to the algorithm recommendations was >80%, and overall understanding was 94%.

Real-Time Ultrasound Guidance for Umbilical Venous Cannulation in Neonates With Congenital Heart Disease.

OBJECTIVES: Umbilical venous cannulation is the favored approach to perinatal central access worldwide but has a failure rate of 25-50% and the insertion technique has not evolved in decades. Improving the success of this procedure would have broad implications, particularly where peripherally inserted central catheters are not easily obtained and in neonates with congenital heart disease, in whom umbilical access facilitates administration of inotropes and blood products while sparing vessels essential for later cardiac interventions. We sought to use real-time, point-of-care ultrasound to achieve central umbilical venous access in patients for whom conventional, blind placement techniques had failed. DESIGN: Multicenter case series, March 2019-May 2021. SETTING: Cardiac and neonatal ICUs at three tertiary care children's hospitals. PATIENTS: We identified 32 neonates with congenital heart disease, who had failed umbilical venous cannulation using traditional, blind techniques. INTERVENTIONS: Real-time ultrasound guidance and liver pressure were used to replace malpositioned catheters and achieve successful placement at the inferior cavoatrial junction. MEASUREMENTS AND MAIN RESULTS: In 32 patients with failed prior umbilical venous catheter placement, real-time ultrasound guidance was used to successfully "rescue" the line and achieve central position in 23 (72%). Twenty of 25 attempts (80%) performed in the first 48 hours of life were successful, and three of seven attempts (43%) performed later. Twenty-four patients (75%) were on prostaglandin infusion at the time of the procedure. We did not identify an association between patient weight or gestational age and successful placement. CONCLUSIONS: Ultrasound guidance has become standard of care for percutaneous central venous access but is a new and emerging technique for umbilical vessel catheterization. In this early experience, we report that point-of-care ultrasound, together with liver pressure, can be used to markedly improve success of placement. This represents a significant advance in this core neonatal procedure.

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

CONTEXT: 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. OBJECTIVES: 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. METHODS: 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. RESULTS: Fifteen professionals and six 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 five 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. CONCLUSION: 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.

Establishing Entrustable Professional Activities in Pediatric Cardiac Critical Care.

OBJECTIVES: Define a set of entrustable professional activities for pediatric cardiac critical care that are recognized as the core activities of the subspecialty by a diverse group of pediatric cardiac critical care physicians and that can be broadly and consistently applied irrespective of training pathway. DESIGN: Mixed methods study with sequential integration of qualitative and quantitative data. SETTING: Structured telephone interviews of pediatric cardiac critical care medical directors at Pediatric Cardiac Critical Care Consortium centers followed by an electronic survey of pediatric cardiac critical care physician members of the Pediatric Cardiac Intensive Care Society from across the United States and internationally. SUBJECTS: Pediatric cardiac intensive care physicians. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty-four of 26 eligible Pediatric Cardiac Critical Care Consortium medical directors participated in the interviews. Based on qualitative analyses of interview data, we identified an initial set of nine entrustable professional activities. Fifty-eight of 185 eligible physicians completed a subsequent survey asking them to rate their agreement with the entrustable professional activities. It showed consensus (> 80% agreement) with the entire initial set of entrustable professional activities, with greater than 96% agreement in most cases. The feedback from free-text survey responses was incorporated to generate a final set of entrustable professional activities. CONCLUSIONS: We generated a set of nine entrustable professional activities, which we believe can be broadly applied to any physician training in pediatric cardiac critical care, irrespective of individual training pathway. Next steps include incorporation of these entrustable professional activities into curriculum design and trainee assessment tools.

Liver lymphatic anatomy and role in systemic lymphatic disease.

OBJECTIVES: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. METHODS: 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. RESULTS: 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 < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .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 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. CONCLUSION: 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. KEY POINTS: • 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.

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

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 days prior to first intervention (114 mL/kg/day) compared to 28 days following final intervention (27 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.

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

Importance: 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. Objectives: 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. Design, Setting, and Participants: 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. Exposures: Having a child or sibling with substantial prematurity, critical congenital heart disease, cancer, or a condition resulting in severe and progressive neurologic impairment. Main Outcomes: 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. Results: 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). Conclusions and Relevance: 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.

A Novel Culprit in a Patient with Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon illness that is characterized by obstruction of hepatic venous outflow. Patients typically present with nausea, vomiting, and abdominal pain, which can further progress into signs associated with liver failure, including jaundice, encephalopathy, and coagulopathy. The most common causes of BCS include pathologies that induce portal vein thrombosis, such as myeloproliferative disorders, malignancy, and acquired hypercoagulable states. In this case report, a patient who presented with abdominal pain and distention is diagnosed with BCS caused by an unusual etiology. He was found to have significant eosinophilia, prompting additional evaluation for parasitic infections. Using stool diagnostics/studies, he was found to have Dientamoeba fragilis trophozoites. The patient was treated with enoxaparin, warfarin, and metronidazole with a resolution of his symptoms. This case outlines a novel cause of BCS as well as the proposed mechanism of Dientamoeba fragilis induction of BCS.