A single institution anesthetic experience with catheterization of pediatric pulmonary hypertension patients.

Cardiac catheterization remains the gold standard for the diagnosis and management of pediatric pulmonary hypertension (PH). There is lack of consensus regarding optimal anesthetic and airway regimen. This retrospective study describes the anesthetic/airway experience of our single center cohort of pediatric PH patients undergoing catheterization, in which obtaining hemodynamic data during spontaneous breathing is preferential. A total of 448 catheterizations were performed in 232 patients. Of the 379 cases that began with a natural airway, 274 (72%) completed the procedure without an invasive airway, 90 (24%) received a planned invasive airway, and 15 (4%) required an unplanned invasive airway. Median age was 3.4 years (interquartile range [IQR] 0.7-9.7); the majority were either Nice Classification Group 1 (48%) or Group 3 (42%). Vasoactive medications and cardiopulmonary resuscitation were required in 14 (3.7%) and eight (2.1%) cases, respectively; there was one death. Characteristics associated with use of an invasive airway included age <1 year, Group 3, congenital heart disease, trisomy 21, prematurity, bronchopulmonary dysplasia, WHO functional class III/IV, no PH therapy at time of case, preoperative respiratory support, and having had an intervention (p < 0.05). A composite predictor of age <1 year, Group 3, prematurity, and any preoperative respiratory support was significantly associated with unplanned airway escalation (26.7% vs. 6.9%, odds ratio: 4.9, confidence interval: 1.4-17.0). This approach appears safe, with serious adverse event rates similar to previous reports despite the predominant use of natural airways. However, research is needed to further investigate the optimal anesthetic regimen and respiratory support for pediatric PH patients undergoing cardiac catheterization.

Palliative Care Across the Life Span for Children With Heart Disease: A Scientific Statement From the American Heart Association.

AIM: This summary from the American Heart Association provides guidance for the provision of primary and subspecialty palliative care in pediatric congenital and acquired heart disease. METHODS: A comprehensive literature search was conducted from January 2010 to December 2021. Seminal articles published before January 2010 were also included in the review. Human subject studies and systematic reviews published in English in PubMed, ClinicalTrials.gov, and the Cochrane Collaboration were included. Structure: Although survival for pediatric congenital and acquired heart disease has tremendously improved in recent decades, morbidity and mortality risks remain for a subset of young people with heart disease, necessitating a role for palliative care. This scientific statement provides an evidence-based approach to the provision of primary and specialty palliative care for children with heart disease. Primary and specialty palliative care specific to pediatric heart disease is defined, and triggers for palliative care are outlined. Palliative care training in pediatric cardiology; diversity, equity, and inclusion considerations; and future research directions are discussed.

Lymphoscintigraphy Using Tilmanocept Detects Multiple Sentinel Lymph Nodes in Melanoma Patients.

BACKGROUND: Technetium-99m-labeled Tilmanocept, a multivalent mannose, is readily internalized by the CD206 surface receptor on macrophages and dendritic cells which are abundantly present in lymph nodes. We want to examine the drainage patterns of Technetium-99m-labeled Tilmanocept to sentinel lymph nodes (SLNs) in melanoma patients following the 10% rule. METHODS: Multi-center retrospective review of patients with cutaneous melanoma undergoing SLN biopsy using Technetium-99m-labeled Tilmanocept between 2008 and 2014 was conducted. Statistical methods were used for data analyses. RESULTS: Of the 564 patients (mean age of 60.3 and 62% male) with preoperative lymphoscintigraphy showing at least one SLN, several primary tumor sites were included: 27% head/neck, 33% trunk, 21% upper extremity and 19% lower extremity. For the head/neck primary site, 36.5% of patients had multiple draining basins; for the trunk site, 36.4% of patients; for the upper extremity site, 13% of patients; and for the lower extremity, 27.4% of patients. A median of 3 (range 1-18) SLNs were identified and resected. Overall, 78% of patients had >1 SLN identified by Technetium-99m-labeled Tilmanocept. In a multivariate model, patients with >1 SLN were significantly associated with age, Breslow depth, tumor location and higher AJCC tumor stage. A total of 17.7% of patients (100/564) had a positive SLN identified. A total of 145 positive SLNs were identified out of 1,812 SLNs with a positive SLN rate of 8%. Positive SLN status was significantly associated with younger age, greater Breslow depth, mitosis rate, higher AJCC tumor stage, presence of ulceration and angiolymphatic invasion. CONCLUSIONS: Using the 10% rule, Technetium-99m-labeled Tilmanocept detects multiple SLNs in most melanoma patients.

Parent-Provider Communication in Hospitalized Children with Advanced Heart Disease.

Communication between parents and providers of children with cardiac disease is essential to parental decision-making. This study explored how parents of hospitalized children with advanced heart disease perceived communication with their child's providers. We performed a prospective survey study of parents and physicians of children with advanced heart disease age 30 days to 19 years admitted to the hospital for > 7 days over a 1-year period at a single institution (n = 160 parent-provider pairs). Descriptive statistics were primarily used and Fisher exact tests and kappa statistics were used to assess agreement. All parents rated communication with their child's care team as excellent, very good, or good, but 56% of parents reported having received conflicting information. Parental perception of "too many" people giving them information was associated with overall poorer communication and less preparedness for decision-making. One-third (32%) of parents felt unprepared for decision-making, despite 88% feeling supported. Parents and physicians showed poor agreement with respect to overall adequacy of communication, receipt of conflicting information, and evaluation of the most effective way for parents to receive information. Interventions involving physician communication training and proactive assessment of parent communication preferences may be beneficial.

The Surprise Question as a Trigger for Primary Palliative Care Interventions for Children with Advanced Heart Disease.

There is significant uncertainty in describing prognosis and a lack of reliable entry criteria for palliative care studies in children with advanced heart disease (AHD). This study evaluates the utility of the surprise question-"Would you be surprised if this child died within the next year?"-to predict one-year mortality in children with AHD and assess its utility as entry criteria for future trials. This is a prospective cohort study of physicians and nurses caring for children (1 month-19 years) with AHD hospitalized ≥ 7 days. AHD was defined as single ventricle physiology, pulmonary vein stenosis or pulmonary hypertension, or any cardiac diagnosis with signs of advanced disease. Primary physicians were asked the surprise question and medical record review was performed. Forty-nine physicians responded to the surprise question for 152 patients. Physicians responded "No, I would not be surprised if this patient died" for 54 (36%) patients, 20 (37%) of whom died within 1 year, predicting one-year mortality with 77% sensitivity, 73% specificity, 37% positive predictive value, and 94% negative predictive value. Patients who received a "No" response had an increased 1-year risk of death (hazard ratio 7.25, p < 0.001). Physician years of experience, subspecialty, and self-rated competency were not associated with the accuracy of the surprise question. The surprise question offers promise as a bedside screening tool to identify children with AHD at high risk for mortality and help physicians identify patients who may benefit from palliative care and advance care planning discussions.

A "Good Death" for Children with Cardiac Disease.

Children with heart disease often experience symptoms and medically intense end-of-life care. Our study explored bereaved parents' perceptions of a "good death" via a mail survey to 128 parents of children with heart disease who died in two centers. Parental perceptions of end-of-life circumstances were assessed by closed-ended questions including level of agreement with the question: "would you say your child experienced a good death?" and open-ended comments were contributed. Medical therapies at end-of-life and mode of death were retrieved through chart review. Of 50 responding parents, 44 (response rate: 34%) responded to the "good death" question; 16 (36%) agreed strongly, 15 (34%) agreed somewhat, and 30% disagreed (somewhat: 7, 16%; strongly: 6, 14%). Half the children were on mechanical support and 84% intubated at death. Of children with cardiopulmonary resuscitation (CPR) at end-of-life, 71% of parents disagreed with the "good death" question compared with 22% of parents whose child died following discontinuation of life-sustaining therapy or comfort measures (OR 9.1, 95% CI 1.3, 48.9, p < 0.01). Parent-reported circumstances associated with disagreement with the "good death" question included cure-oriented goals-of-care (OR 16.6, 95% CI 3.0, 87.8, p < 0.001), lack of advance care planning (ACP) (OR 12.4 95% CI 2.1, 65.3 p < 0.002), surprise regarding timing of death (OR 11.7, 95% CI 2.6, 53.4 p < 0.002), and experience of pain (OR 42.1, 95% CI 2.3, 773.7 p < 0.02). Despite high medical intensity, many bereaved parents of children with cardiac disease agree a "good death" was experienced. A "good death" was associated with greater preparedness, ACP, non-cure-oriented goals-of-care, pain control, and CPR avoidance.

Parent-Reported Symptoms and Perceived Effectiveness of Treatment in Children Hospitalized with Advanced Heart Disease.

OBJECTIVE: To characterize parent-reported symptom burden and effectiveness of symptom management in children hospitalized with advanced heart disease. STUDY DESIGN: Prospective survey study of 161 parents whose child was admitted to a single institution with advanced heart disease between March 2018 and February 2019 using the Survey about Caring for Children with Heart Disease. RESULTS: Of the 161 patients, 54% were under 2 years old with a diagnosis of single ventricle physiology (39%), pulmonary hypertension (12%), and other congenital heart disease (28%). Over one-half (56%) of parents reported that their child was experiencing a high degree ("a great deal"/"a lot") of symptoms. The most frequently reported symptoms were pain (68%), fatigue (63%), and breathing difficulties (60%). Of the symptoms that were treated, parents perceived successful treatment to be least likely for their child's sleep disturbance (24%), depression (29%), and fatigue (35%). Parents who reported their child's functional status as New York Heart Association class III/IV were more likely to report that their child was experiencing "a great deal" of symptoms, compared with those who reported class I/II (51% vs 19%, P < .001). Parents who reported their child was experiencing a high degree of suffering from fatigue were also more likely to report a high symptom burden (P < .001). CONCLUSIONS: Parents of children with advanced heart disease reported high symptom burden with a broad spectrum of symptoms. Parents reported fatigue and psychiatric symptoms frequently and rarely reported treatment as successful. Parents' view of their child's symptom burden was concordant with their perception of their child's functional status.

Mortality from Pulmonary Hypertension in the Pediatric Cardiac ICU.

Rationale: Patients with pulmonary hypertension (PH) admitted to pediatric cardiac ICUs are at high risk of mortality. Objectives: To identify factors associated with mortality in cardiac critical care admissions with PH. Methods: We evaluated medical admissions with PH to Pediatric Cardiac Critical Care Consortium institutions over 5 years. PH was standardly defined in the clinical registry by diagnosis and/or receipt of intensive care-level pulmonary vasodilator therapy. Multivariable logistic regression identified independent associations with mortality. Measurements and Main Results: We analyzed 2,602 admissions; mortality was 10% versus 3.9% for all other medical admissions. Covariates most strongly associated with mortality included invasive ventilation (adjusted odds ratio, 44.8; 95% confidence interval, 6.2-323), noninvasive ventilation (19.7; 2.8-140), cardiopulmonary resuscitation (8.9; 5.6-14.1), and vasoactive infusions (4.8; 2.6-8.8). Patients receiving both invasive ventilation and vasoactive infusions on admission Days 1 and 2 had an observed mortality rate of 29.2% and 28.6%, respectively, compared with <5% for those not receiving either. Vasoactive infusions emerged as the dominant early risk factor for mortality, increasing the absolute risk of mortality on average by 6.4% when present on admission Day 2. Conclusions: Patients with PH admitted to pediatric cardiac critical care units have high mortality rates. Those receiving invasive ventilation and vasoactive infusions on Day 1 or Day 2 had an observed mortality rate that was more than fivefold greater than that of those who did not. These data highlight the illness severity of patients with PH in this setting and could help inform conversations with families regarding the prognosis.

Palliative Care Referrals in Cardiac Disease.

OBJECTIVES: With evidence of benefits of pediatric palliative care (PPC) integration, we sought to characterize subspecialty PPC referral patterns and end of life (EOL) care in pediatric advanced heart disease (AHD). METHODS: In this retrospective cohort study, we compared inpatient pediatric (<21 years) deaths due to AHD in 2 separate 3-year epochs: 2007-2009 (early) and 2015-2018 (late). Demographics, disease burden, medical interventions, mode of death, and hospital charges were evaluated for temporal changes and PPC influence. RESULTS: Of 3409 early-epoch admissions, there were 110 deaths; the late epoch had 99 deaths in 4032 admissions. In the early epoch, 45 patients (1.3% admissions, 17% deaths) were referred for PPC, compared with 146 late-epoch patients (3.6% admissions, 58% deaths). Most deaths (186 [89%]) occurred in the cardiac ICU after discontinuation of life-sustaining therapy (138 [66%]). Medical therapies included ventilation (189 [90%]), inotropes (184 [88%]), cardiopulmonary resuscitation (68 [33%]), or mechanical circulatory support (67 [32%]), with no temporal difference observed. PPC involvement was associated with decreased mechanical circulatory support, ventilation, inotropes, or cardiopulmonary resuscitation at EOL, and children were more likely to be awake and be receiving enteral feeds. PPC involvement increased advance care planning, with lower hospital charges on day of death and 7 days before (respective differences $5058 [P = .02] and $25 634 [P = .02]). CONCLUSIONS: Pediatric AHD deaths are associated with high medical intensity; however, children with PPC consultation experienced substantially less invasive interventions at EOL. Further study is warranted to explore these findings and how palliative care principles can be better integrated into care.

Parent and Physician Understanding of Prognosis in Hospitalized Children With Advanced Heart Disease.

Background The unpredictable trajectory of pediatric advanced heart disease makes prognostication difficult for physicians and informed decision-making challenging for families. This study evaluated parent and physician understanding of disease burden and prognosis in hospitalized children with advanced heart disease. Methods and Results A longitudinal survey study of parents and physicians caring for patients with advanced heart disease age 30 days to 19 years admitted for ≥7 days was performed over a 1-year period (n=160 pairs). Percentage agreement and weighted kappa statistics were used to assess agreement. Median patient age was 1 year (interquartile range, 1-5), 39% had single-ventricle lesions, and 37% were in the cardiac intensive care unit. Although 92% of parents reported understanding their child's prognosis "extremely well" or "well," 28% of physicians thought parents understood the prognosis only "a little," "somewhat," or "not at all." Better parent-reported prognostic understanding was associated with greater preparedness for their child's medical problems (odds ratio, 4.7; 95% CI, 1.4-21.7, P=0.02). There was poor parent-physician agreement in assessing functional class, symptom burden, and likelihood of limitations in physical activity and learning/behavior; on average, parents were more optimistic. Many parents (47%) but few physicians (6%) expected the child to have normal life expectancy. Conclusions Parents and physicians caring for children with advanced heart disease differed in their perspectives regarding prognosis and disease burden. Physicians tended to underestimate the degree of parent-reported symptom burden. Parents were less likely to expect limitations in physical activity, learning/behavior, and life expectancy. Combined interventions involving patient-reported outcomes, parent education, and physician communication tools may be beneficial.

Congenital Cardiothoracic Surgeons and Palliative Care: A National Survey Study.

BACKGROUND: The majority of children with advanced heart disease in the inpatient setting die in an intensive care unit under 1 year of age following multiple interventions. While pediatric cardiology and palliative care provider attitudes have been described, little is known about pediatric cardiothoracic surgeon attitudes toward palliative care in children with advanced heart disease. OBJECTIVE: To describe perspectives of pediatric cardiothoracic surgeons regarding palliative care in pediatric heart disease. DESIGN: Cross-sectional web-based national survey. RESULTS: Of the 220 surgeons who were e-mailed the survey, 36 opened the survey and 5 did not meet inclusion criteria (n = 31). Median years of practice was 23.5 (range: 12-41 years), and 87.1% were male. Almost all (90%) reported that they had experience consulting palliative care. While 68% felt palliative care consultation was initiated at the appropriate time, 29% felt it occurred too late. When asked the appropriate timing for palliative care consultation in hypoplastic left heart syndrome, 45% selected "at time of prenatal diagnosis" and 30% selected "when surgical and transcatheter options have been exhausted." Common barriers to palliative care involvement included the perception of "giving up" (40%) and concern for undermining parental hope (36%). CONCLUSIONS: While a majority of pediatric cardiothoracic surgeons are familiar with palliative care, there is variation in perception of appropriate timing of consultation. Significant barriers to consultation still exist, including concern that parents will think they are "giving" up, undermining parental hope, and influence of palliative care on the medical care team's approach.

Extracorporeal Membrane Oxygenation in Pediatric Pulmonary Hypertension.

OBJECTIVE: To describe the epidemiology, critical care interventions, and mortality of children with pulmonary hypertension receiving extracorporeal membrane oxygenation. DESIGN: Retrospective analysis of prospectively collected multicenter data. SETTING: Data entered into the Extracorporeal Life Support Organization database between January 2007 and November 2018. PATIENTS: Pediatric patients between 28 days and 18 years old with a diagnosis of pulmonary hypertension. MEASUREMENTS AND MAIN RESULTS: Six hundred thirty-four extracorporeal membrane oxygenation runs were identified (605 patients). Extracorporeal membrane oxygenation support type was pulmonary (43.1%), cardiac (40.2%), and extracorporeal cardiopulmonary resuscitation (16.7%). The majority of cannulations were venoarterial (80.4%), and 30% had a pre-extracorporeal membrane oxygenation cardiac arrest. Mortality in patients with pulmonary hypertension was 51.3% compared with 44.8% (p = 0.001) in those without pulmonary hypertension. In univariate analyses, significant predictors of mortality included age less than 6 months and greater than 5 years; pre-extracorporeal membrane oxygenation cardiac arrest; pre-extracorporeal membrane oxygenation blood gas with pH less than 7.12, PaCO2 greater than 75, PaO2 less than 35, and arterial oxygen saturation less than 60%; extracorporeal membrane oxygenation duration greater than 280 hours; extracorporeal cardiopulmonary resuscitation; and extracorporeal membrane oxygenation complications including cardiopulmonary resuscitation, inotropic support, myocardial stun, tamponade, pulmonary hemorrhage, intracranial hemorrhage, seizures, other hemorrhage, disseminated intravascular coagulation, renal replacement therapy, mechanical/circuit problem, and metabolic acidosis. A co-diagnosis of pneumonia was associated with significantly lower odds of mortality (odds ratio, 0.5; 95% CI, 0.3-0.8). Prediction models were developed using three sets of variables: 1) pre-extracorporeal membrane oxygenation (age, absence of pneumonia, and pH < 7.12; area under the curve, 0.62); 2) extracorporeal membrane oxygenation related (extracorporeal cardiopulmonary resuscitation, any neurologic complication, pulmonary hemorrhage, renal replacement therapy, and metabolic acidosis; area under the curve, 0.72); and 3) all variables combined (area under the curve, 0.75) (p < 0.001). CONCLUSIONS: Children with pulmonary hypertension who require extracorporeal membrane oxygenation support have a significantly greater odds of mortality compared with those without pulmonary hypertension. Risk factors for mortality include age, absence of pneumonia, pre-extracorporeal membrane oxygenation acidosis, extracorporeal cardiopulmonary resuscitation, pulmonary hemorrhage, neurologic complications, renal replacement therapy, and acidosis while on extracorporeal membrane oxygenation. Identification of those pulmonary hypertension patients requiring extracorporeal membrane oxygenation who are at even higher risk for mortality may inform clinical decision-making and improve prognostic awareness.

Palliative care and paediatric cardiology: current evidence and future directions.

Although outcomes for children with heart disease have improved substantially over the past several decades, heart disease remains one of the leading causes of paediatric mortality. For children who progress to advanced heart disease, disease morbidity is high, with many children requiring multiple surgical interventions and long-term intensive care hospitalisations. Care for children with advanced heart disease requires a multidisciplinary approach, and opportunities for earlier integration of palliative care are being explored. This Viewpoint summarises the relevant literature over the past decade. We also identify gaps in parent and provider understanding of prognosis and communication, propose indications for palliative care consultation in paediatric advanced heart disease, and summarise attitudes and perceived barriers to palliative care consultation. Areas for additional research that we identify include paediatric cardiologist education, parental distress, socioeconomic disparities, and patient-reported outcomes. Interdisciplinary clinical and research efforts are required to further advance the field and improve integration of palliative care in the care of children with heart disease.

Patterns of care at end of life in children with advanced heart disease.

OBJECTIVE: To describe patterns of care for pediatric patients with advanced heart disease who experience in-hospital death. DESIGN: Retrospective single-institution medical record review. SETTING: A tertiary care pediatric hospital. PARTICIPANTS: All patients younger than 21 years who died in the inpatient setting between January 1, 2007, and December 31, 2009, with primary cardiac diagnoses or who had ever received a cardiology consult (N=468). After excluding patients with significant noncardiac primary diagnoses, 111 children formed the analytic sample. MAIN OUTCOME MEASURE: In-hospital deaths of children with heart disease during a 3-year period. RESULTS: Median age at death was 4.8 months (age range,1 day to 20.5 years), with 84 deaths (75.7%) occurring before age 1 year. Median length of terminal hospital stay was 22 days (range, 1-199 days). Diagnoses included 84 patients (75.7%) with congenital heart disease, 10 (9.0%)with cardiomyopathy/myocarditis, 9 (8.1%) with pulmonary hypertension, and 8 (7.2%) with heart transplants.Sixty-two patients (55.9%) had received cardio-pulmonary resuscitation during their last hospital admission. At the end of life, 21 children (18.9%) had gastrostomy tubes and 26 (23.4%) had peritoneal drains.Most patients (91.9%) received ventilation, with half also receiving mechanical circulatory support. Eighty-three patients (74.8%) experienced additional end-organ failure. Classified by mode of death, 76 patients (68.5%) had disease-directed support withdrawn, 28 (25.2%) died during resuscitation, and 7 (6.3%) died while receiving comfort care after birth. Eighty-three percent of parents were present at the time of death. CONCLUSION: Infants and children who die of advanced heart disease frequently succumb in the intensive care setting with multisystem organ failure and exposure to highly technical care.

Methicillin-resistant Staphylococcus aureus: a pervasive pathogen highlights the need for new antimicrobial development.

Staphylococcus aureus (S. aureus) has entered the spotlight as a globally pervasive drug-resistant pathogen. While historically associated exclusively with hospital-acquired infections in immunocompromised hosts, the methicillin-resistant form of S. aureus has been spreading throughout communities since the 1990s. Indeed, it has now become a common household term: MRSA. S. aureus has developed numerous mechanisms of virulence and strategies to evade the human immune system, including a host of surface proteins, secreted enzymes, and toxins. In hospital intensive care units, the proportion of MRSA-related S. aureus infections has increased strikingly from just 2 percent in 1974 to 64 percent in 2004. Its presence in the community has been rising similarly, posing a significant public health burden. The growing incidence of MRSA unfortunately has been met with dwindling efforts to develop new, more effective antibiotics. The continued emergence of resistant strains of bacteria such as MRSA demands an urgent revival of the search for new antibiotics.