Is a Chest Radiograph Required After Removal of Chest Tubes in Children?

Our objective was to determine the clinical value of obtaining a chest radiograph after removal of a chest tube. We conducted a retrospective chart review of pediatric general surgical patients with a chest tube in place after a thoracic procedure over a 3-year time period. Postremoval films were considered to be of value if they led to a change in clinical management. Of 468 patients who had a thoracic procedure, 281 patients had a chest tube and a postremoval film. In 263 patients (93.6%) there was no change in the postremoval film result compared with baseline. Only two patients (0.7%) required an intervention based on symptoms, not based on the postremoval film. Eliminating routine postremoval radiographs after chest tube removal in pediatric patients will lessen radiation exposure and provide cost savings with no adverse impact on outcome.

Regorafenib treatment for advanced, refractory gastrointestinal stromal tumor: a report of the UK managed access program.

BACKGROUND: Tyrosine kinase inhibitors (TKI) have revolutionized the treatment of gastrointestinal stromal tumors (GIST) although most patients develop resistance to first and second-line therapies. Regorafenib, an oral multi-targeted TKI, has demonstrated benefit in previously treated GIST patients. METHODS: We assessed safety and activity of regorafenib in patients treated within the Managed Access Program (MAP). All consecutive patients with advanced GIST who had progressed on or were intolerant to imatinib and sunitinib were recruited from the Royal Marsden and University College Hospitals. We retrospectively reviewed the data for response, toxicity, treatment duration and survival. Response was assessed by RECIST and Choi criteria. Toxicity was graded according to CTCAE v4.0 criteria. RESULTS: 20 patients were included in the MAP in the UK between 3/2013 and 9/2013. Median age was 68 (range 45-87), 65% of patients were male. Performance Status was 0-1 for 18 patients (90%), 2 for 2 patients (10%). The median treatment duration was 9.25 months (range 0.1-15.33). 18 patients were assessable for response and all patients attained a best response of at least stable disease. At a median follow-up of 12.6 months, there were 2 partial responses (11%) by RECIST and 7 partial responses (39%) according to Choi criteria. 7 patients remain on regorafenib. 3 patients discontinued treatment due to unacceptable adverse events; fistulation, myalgia and fatigue. 10 (50%) patients had grade 3 toxicities and 11 (55%) patients required a dose reduction. Median PFS was 9.4 months (95% Cl: 6.2-not calculable) and median OS was 12.2 months (95% Cl: 10.5-not calculable). Notably, prolonged stable disease was seen in 1 patient with exon 9 mutation and 1 patient with PDGFR D842V mutation. CONCLUSIONS: These data demonstrate encouraging activity and tolerability of regorafenib in routine clinical practice. The documented adverse events are in line with previous trial data.

The teachable moment captured: a framework for nurse-led smoking cessation interventions for parents of hospitalized children.

This article explores the topic of smoking cessation counseling for parents in the context of pediatric hospitalization. Teachable moments, a widely used concept in the literature, uses three key concepts including perception of risk, emotional response, and self-concept to precipitate change (McBride, Health Education Research, 18 [McBride, 2003], 156-170). The interweaving of these concepts with institutional systems; clinically trained personnel; parental smoking considerations; parent presence; and external supports, or collectively the novel idea of the "capturable moment", may allow for an increased rate of parental smoking cessation. Using these concepts, the authors constructed a hospital model for pediatric nursing efforts in parental smoking cessation. The pilot study built on this framework in February 2010 began enrolling parents of hospitalized pediatric patients into two intervention groups to motivate smoking cessation. Starting in September 2010, new electronic medical record-based systems of identifying parents who smoke were implemented in the hopes of enhancing enrollment numbers and streamlining recruitment. It is hoped that by introducing this process and framework, there will be increased national dialogue related to secondhand smoke (SHS) exposure, routine screening for SHS exposure, and nursing recognition of teachable moments.

The relationship between inflammatory activation and clinical outcome after infant cardiopulmonary bypass.

BACKGROUND: Cardiopulmonary bypass (CPB) induces a systemic inflammatory response. The magnitude and consequences in infants remain unclear. We assessed the relationship between inflammatory state and clinical outcomes in infants undergoing CPB. METHODS: Plasma concentrations of interleukin (IL)-6, IL-8, IL-10, tumor necrosis factor α, IL-1ß, and C-reactive protein (CRP) were measured pre-CPB and immediately post-CPB, and at 6, 12, and 24 hours post-CPB in infants ≤9 months old. Perioperative clinical data were collected prospectively. RESULTS: Diagnoses of 93 patients included transposition of the great arteries (40), tetralogy of Fallot (28), ventricular septal defect (21), truncus arteriosus (2), and complete atrioventricular canal (2). The median age was 37 days (range = 2 to 264). Pre-CPB IL-6 and CRP were higher in younger infants but were not associated with postoperative inflammatory mediator concentrations or measured clinical outcomes. IL-6 increased post-CPB (median 3.2 pg/mL pre-CPB, 24.2 post-CPB, 95.4 at 6 hours, and 90.3 at 24 hours; all P < 0.001). CRP increased post-CPB, peaking at 24 hours (median 27.5 at 24 hours, 0.3 pre-CPB; P < 0.001). IL-10 and IL-8 increased immediately post-CPB. After adjusting for age and diagnosis, postoperative IL-6 and IL-8 correlated with intensive care unit length of stay and postoperative blood product administration and, for IL-8, 24-hour lactate. CONCLUSIONS: Greater preoperative cytokine and CRP production in younger infants did not correlate with postoperative outcomes; correlation between postoperative inflammatory mediator production and clinical course was statistically significant but clinically modest. We conclude that in infants undergoing low-to-moderate-complexity cardiac surgery in a single high-volume center, the contribution of inflammatory mediator production to postoperative morbidity is relatively limited.

Brain volume and metabolism in fetuses with congenital heart disease: evaluation with quantitative magnetic resonance imaging and spectroscopy.

BACKGROUND: Adverse neurodevelopmental outcome is an important source of morbidity in children with congenital heart disease (CHD). A significant proportion of newborns with complex CHD have abnormalities of brain size, structure, or function, which suggests that antenatal factors may contribute to childhood neurodevelopmental morbidity. METHODS AND RESULTS: Brain volume and metabolism were compared prospectively between 55 fetuses with CHD and 50 normal fetuses with the use of 3-dimensinal volumetric magnetic resonance imaging and proton magnetic resonance spectroscopy. Fetal intracranial cavity volume, cerebrospinal fluid volume, and total brain volume were measured by manual segmentation. Proton magnetic resonance spectroscopy was used to measure the cerebral N-acetyl aspartate: choline ratio (NAA:choline) and identify cerebral lactate. Complete fetal echocardiograms were performed. Gestational age at magnetic resonance imaging ranged from 25 1/7 to 37 1/7 weeks (median, 30 weeks). During the third trimester, there were progressive and significant declines in gestational age-adjusted total brain volume and intracranial cavity volume in CHD fetuses relative to controls. NAA:choline increased progressively over the third trimester in normal fetuses, but the rate of rise was significantly slower (P<0.001) in CHD fetuses. On multivariable analysis adjusted for gestational age and weight percentile, cardiac diagnosis and percentage of combined ventricular output through the aortic valve were independently associated with total brain volume. Independent predictors of lower NAA:choline included diagnosis, absence of antegrade aortic arch flow, and evidence of cerebral lactate (P<0.001). CONCLUSIONS: Third-trimester fetuses with some forms of CHD have smaller gestational age- and weight-adjusted total brain volumes than normal fetuses and evidence of impaired neuroaxonal development and metabolism. Hemodynamic factors may play an important role in this abnormal development.

Parent- versus child-reported functional health status after the Fontan procedure.

OBJECTIVE: We sought to compare perceptions of functional health status between children who had undergone a Fontan procedure and their parents. METHODS: Fontan procedure survivors 10 to 18 years of age were included in the study if the child completed the Child Health Questionnaire (CHQ) and the parent completed the parent form to assess the child's functional health status. Comparisons were made between raw domain scores for the parent- and child-completed CHQs. RESULTS: Between March 2003 and April 2004, 1078 Fontan survivors were screened. Of the 546 eligible and consented patients, 354 were 10-18 years of age and 328 parent/child pairs completed the CHQs. Parents reported significantly lower scores (worse functioning) for their children than the children reported for themselves in the domains of physical functioning (P < .01), impact on school or activities from emotional and behavioral problems (P < .01), impact on school or activities from physical health issues (P < .01), general behavior (P < .01), mental health (P < .01), self-esteem (P < .01), and general health perceptions (P < .01). No significant differences were noted for the domains of bodily pain, family cohesiveness, or family activities. For the physical functioning domain, factors contributing to lower scores for parent versus child reports included pulmonary artery anomalies and fenestration at the time of the Fontan operation. Lower parent-reported scores also were associated with more noncardiac health problems in the child. CONCLUSIONS: Parents' perceptions of the functional health status of their children after the Fontan procedure were worse than the children's perceptions.

Cerebral oximetry during infant cardiac surgery: evaluation and relationship to early postoperative outcome.

BACKGROUND: We examined changes in cerebral oxygen saturation during infant heart surgery and its relationship to anatomic diagnosis and early outcome. METHODS: Regional cerebral oxygen saturation (rSO(2)) was measured by near-infrared spectroscopy in 104 infants undergoing biventricular repair without aortic arch obstruction as part of a randomized trial of hemodilution to a hematocrit of 25% vs 35%. RESULTS: Before cardiopulmonary bypass (CPB), infants with tetralogy of Fallot had higher rSO(2) values compared to those with D-transposition of the great arteries (D-TGA) or ventricular septal defect (P < 0.001). During CPB cooling, low flow, and at the termination of CPB, D-TGA subjects had the highest rSO(2) values (P < 0.001). There were no significant associations between intraoperative rSO(2) and early postoperative outcomes after adjustment for diagnosis. In 39 D-TGA subjects with > or =5 min of deep hypothermic circulatory arrest (DHCA), there was no correlation between the rSO(2) (91% +/- 6%) or hematocrit (29.2% +/- 5.5%) at the onset of arrest and the rate of decline in rSO(2) during arrest. CONCLUSIONS: Intraoperative rSO(2) varies according to anatomic diagnosis but accounts for very little of the variance in early outcome. As measured by frontal near-infrared spectroscopy, higher levels of hematocrit and current perfusion techniques appear to provide an adequate oxygen reservoir prior to relatively short periods of DHCA.

Randomized trial of hematocrit 25% versus 35% during hypothermic cardiopulmonary bypass in infant heart surgery.

OBJECTIVES: We previously reported that postoperative hemodynamics and developmental outcomes were better among infants randomized to a higher hematocrit value during hypothermic cardiopulmonary bypass. However, worse outcomes were concentrated in patients with hematocrit values of 20% or below, and the benefits of hematocrit values higher than 25% were uncertain. METHODS: We compared perioperative hemodynamics and, at 1 year, developmental outcome and brain magnetic resonance imaging in a single-center, randomized trial of hemodilution to a hematocrit value of 25% versus 35% during hypothermic radiopulmonary bypass for reparative heart surgery in infants undergoing 2-ventricle repairs without aortic arch obstruction. RESULTS: Among 124 subjects, 56 were assigned to the lower-hematocrit strategy (24.8% +/- 3.1%, mean +/- SD) and 68 to the higher-hematocrit strategy (32.6% +/- 3.5%). Infants randomized to the 25% strategy, compared with the 35% strategy, had a more positive intraoperative fluid balance (P = .007) and lower regional cerebral oxygen saturation at 10 minutes after cooling (P = .04) and onset of low flow (P = .03). Infants with dextro-transposition of the great arteries in the 25% group had significantly longer hospital stay. Other postoperative outcomes, blood product usage, and adverse events were similar in the treatment groups. At age 1 year (n = 106), the treatment groups had similar scores on the Psychomotor and Mental Development Indexes of the Bayley Scales; both groups scored significantly worse than population norms. CONCLUSIONS: Hemodilution to hematocrit levels of 35% compared with those of 25% had no major benefits or risks overall among infants undergoing 2-ventricle repair. Developmental outcomes at age 1 year in both randomized groups were below those in the normative population.

An evaluation of bilateral monitoring of cerebral oxygen saturation during pediatric cardiac surgery.

Cerebral oximetry is a technique that enables monitoring of regional cerebral oxygenation during cardiac surgery. In this study, we evaluated differences in bi-hemispheric measurement of cerebral oxygen saturation using near-infrared spectroscopy in 62 infants undergoing biventricular repair without aortic arch reconstruction. Left and right regional cerebral oxygen saturation index (rSO2i) were recorded continuously after the induction of anesthesia, and data were analyzed at 12 time points. Baseline rSO2i measurements were left 65 +/- 13 and right 66 +/- 13 (P = 0.17). Mean left and right rSO2i measurements were similar (< or =2 percentage points/absolute scale units) before, during, and after cardiopulmonary bypass, irrespective of the use of deep hypothermic circulatory arrest. Further longitudinal neurological outcome studies are required to determine whether uni- or bi-hemispheric monitoring is required in this patient population.

A randomized, double-blind, placebo-controlled pilot trial of triiodothyronine in neonatal heart surgery.

OBJECTIVE: This study was undertaken to evaluate the effect of triiodothyronine replacement on the early postoperative course of neonates undergoing aortic arch reconstruction. METHODS: We performed a randomized, double-blind, placebo-controlled trial of triiodothyronine supplementation in neonates undergoing either a Norwood procedure or two-ventricle repair of interrupted aortic arch and ventricular septal defect. Patients were assigned to receive a continuous infusion of triiodothyronine (0.05 micro/kg/h) or placebo for 72 hours after cardiopulmonary bypass. Primary end points were a composite clinical outcome score and cardiac index at 48 postoperative hours. RESULTS: We enrolled 42 patients (triiodothyronine n = 22, placebo n = 20). Baseline characteristics were similar in the treatment groups. Study drug was discontinued prematurely because of hypertension (n = 1) and ectopic atrial tachycardia (n = 1), both cases in the triiodothyronine group. Free and total triiodothyronine levels were higher in the triiodothyronine group than in the placebo group at 24, 48, and 72 postoperative hours (P < .001). The median clinical outcome scores were 2.0 (range 0-4) with triiodothyronine and 2.0 (range 0-7) with placebo (P = .046). Compared with those in the placebo group, neonates assigned to triiodothyronine had shorter median time to negative fluid balance (2.0 vs 2.5 days, P = .027). Cardiac index values were 2.11 +/- 0.64 L/min x m2 with triiodothyronine and 2.05 +/- 0.72 L/min x m2 with placebo (P = .81). Heart rate and diastolic blood pressure were not influenced by triiodothyronine supplementation, but systolic blood pressure was higher in the triiodothyronine group (P < .001). No serious adverse events were attributed to triiodothyronine administration. CONCLUSION: Triiodothyronine supplementation was safe and resulted in more rapid achievement of negative fluid balance after aortic arch reconstruction. Cardiac index at 48 hours was not significantly improved.

Prediction of IQ and achievement at age 8 years from neurodevelopmental status at age 1 year in children with D-transposition of the great arteries.

OBJECTIVE: Studies of developmental outcomes in children with congenital heart disease (CHD) frequently use assessments conducted in infancy as primary endpoints. Whether test scores of CHD patients in infancy are predictive of status at school age has not been evaluated, however. METHODS: In the Boston Circulatory Arrest Study, 135 children with D-transposition of the great arteries repaired by arterial switch operation were administered the Bayley Scales of Infant Development and the Fagan Test of Infant Intelligence at 1 year of age and the Wechsler Intelligence Scale for Children, Third Edition and the Wechsler Individual Achievement Test at 8 years. RESULTS: Although most 1-year test scores were significantly associated with 8-year test scores, the amounts of shared variance were modest (<10%). All 1-year test scores had poor sensitivity (16%-32%) and poor positive predictive value (35%-42%) but good specificity (80%-93%) and negative predictive value (78%-79%). More than half of the children with low scores at 8 years (< or =85) had had scores >84 at 1 year. CONCLUSION: This pattern suggests that although test scores at 1 year are modestly associated with test scores at 8 years, many children who are at risk for poor late outcomes will not be identified on the basis of 1-year test scores. Long-term follow-up of children with CHD is necessary to draw inferences about the developmental sequelae of preoperative, intraoperative, and postoperative factors.

Recombinant human growth hormone treatment for dilated cardiomyopathy in children.

OBJECTIVE: Dilated cardiomyopathy (DCM) is one of the most common causes of heart failure among children and is often progressive despite maximal medical therapy. Heart failure is characterized by a number of neurohormonal abnormalities, including derangements in the growth hormone (GH)/insulin-like growth factor-1 (IGF-1) signaling axis. Decreased serum levels of GH, which acts on cardiac myocytes primarily through IGF-1, are associated with impaired myocardial growth and function, which can be improved with restoration of GH/IGF-1 homeostasis. In animal models and among human adults with heart failure attributable to DCM, treatment with GH results in acquisition of left ventricular (LV) mass and improved LV function, through a combination of mechanisms. We undertook this study to determine the effects of recombinant human GH on LV function and mass among children with stable LV dysfunction attributable to DCM. METHODS: We performed a prospective, single-center, randomized, partially blinded, crossover trial among children 1 to 19 years of age with DCM and cardiac dysfunction of > or =6-month duration. After enrollment, patients were randomly assigned to receive treatment for 6 months with either conventional therapy (determined by the patient's primary cardiologist) plus recombinant human GH (0.025-0.04 mg/kg per day), administered as daily subcutaneous injections, or conventional therapy alone. Patients were then crossed over to the other treatment strategy for 6 months. The primary outcome measure was change in LV shortening fraction (SF). Other echocardiographic indices of LV function, somatic growth, and somatotropic/thyroid hormone levels were also monitored. RESULTS: Only 8 of an intended 15 patients were enrolled, because of a combination of factors. Two patients withdrew during the study as a result of declining LV function requiring transplantation. LV SF did not change significantly during GH treatment, although both LV SF and LV SF z score were higher 6 months after cessation of GH treatment than at baseline. LV ejection fraction increased during GH therapy to a degree that approached significance. Height and weight percentiles for age increased significantly during GH therapy and remained higher 6 months after treatment. Annualized height velocity during GH treatment (13.7 +/- 3.3 cm/year, >97th percentile for all patients) was significantly higher than that after GH discontinuation (3.2 +/- 3.5 cm/year). Serum levels of IGF-1 and IGF-binding protein-3 were significantly higher after 6 months of GH treatment and 6 months after discontinuation of GH treatment than at baseline. There were no adverse events related to GH treatment. DISCUSSION: In this prospective, single-center, randomized, partially blinded, crossover trial, recombinant human GH was administered to 8 pediatric patients with stable chronic heart failure secondary to DCM. Because of unanticipated difficulty enrolling eligible patients, the study was underpowered to detect changes in our primary outcome measure of the magnitude we projected. Nevertheless, we did observe several notable cardiovascular effects of GH treatment, including a trend toward improved LV ejection fraction during the course of GH treatment and significantly improved LV SF, SF z score, and LV end systolic stress z score 6 months after discontinuation of GH treatment (relative to baseline values). Given the fact that levels of IGF-1, the primary myocardial effector of GH signaling, remained significantly higher 6 months after GH treatment than at baseline, the improvement in LV functional indices 6 months after discontinuation of therapy may represent progression or perpetuation of a GH treatment effect. In addition to its cardiovascular effects, GH therapy was associated with significant acceleration of somatic growth. The benefits of GH were not associated with significant attributable side effects, although 2 patients developed progressive LV dysfunction during the study and underwent cardiac transplantation.

The influence of hemodilution on outcome after hypothermic cardiopulmonary bypass: results of a randomized trial in infants.

BACKGROUND: We hypothesized that cognitive impairment and hemodynamic instability after infant cardiac surgery with cardiopulmonary bypass might be exacerbated by hemodilution. METHODS: In a single-center randomized trial with blinded assessment of outcomes, we compared use of 2 hemodilution protocols during hypothermic cardiopulmonary bypass with infant cardiac surgery. The primary perioperative end point was lowest cardiac index in the first 24 hours postoperatively, and primary end points at age 1 year were scores on the Psychomotor Development Index and Mental Developmental Index of the Bayley Scales. RESULTS: Among 147 subjects, 74 were assigned to the lower-hematocrit strategy (21.5% +/- 2.9%, mean +/- SD at onset of low-flow bypass) and 73 to the higher-hematocrit strategy (27.8% +/- 3.2%). In intent-to-treat analyses the lower-hematocrit group had lower nadirs of cardiac index (P =.02), higher serum lactate levels 60 minutes after cardiopulmonary bypass (P =.03), and a greater percentage increase in total body water on the first postoperative day (P =.006). Blood product use and adverse events were similar in the 2 groups. At age 1 year (113 children), the lower-hematocrit group had worse scores on the Psychomotor Development Index (81.9 +/- 15.7 vs 89.7 +/- 14.7, P =.008), as well as more Psychomotor Development Index scores at least 2 SDs below the population mean (16/56 [29%] vs 5/53 [9%], P =.01). The groups had similar Mental Developmental Index scores and findings on neurologic examination. Inferences using hematocrit as a continuous variable were similar to those based on intent-to-treat analyses. CONCLUSIONS: Hemodilution to a hematocrit level in wide use for cardiopulmonary bypass and thought to be safe is associated with adverse perioperative and developmental outcomes in infants.