Purpose Prospective validation of vasoactive-inotropic score (VIS) and inotrope score (IS) in infants after cardiovascular surgery Methods Prospective observational study of 70 infants (≤90 days of age) undergoing cardiothoracic surgery. VIS and IS were assessed at 24 (VIS24, IS24), 48 (VIS48, IS48), and 72 (VIS72, IS72) hours after surgery. Maximum VIS and IS scores in the first 48 hours were also calculated (VIS48max and IS48max). The primary outcome was length of intubation. Additional outcomes included length of intensive care (ICU) stay and hospitalization, cardiac arrest, mortality, time to negative fluid balance, peak lactate, and change in creatinine. Results Based on Receiver Operating Characteristic (ROC) analysis, area under the curve (AUC) was highest for VIS48 to identify prolonged intubation time. AUC for the primary outcome was higher for VIS than IS at all time points assessed. On multivariate analysis VIS48 was independently associated with prolonged intubation (OR 22.3, p=0.002), prolonged ICU stay (OR 8.1, p=0.017), and prolonged hospitalization (OR 11.3, p=0.011). VIS48max, IS48max, and IS48 were also associated with prolonged intubation, but not prolonged ICU or hospital stay. None of the scores were associated with time to negative fluid balance, peak lactate, or change in creatinine. Conclusion In neonates and infants, a higher VIS at 48 hours after cardiothoracic surgery is strongly associated with increased length of ventilation, and prolonged ICU and total hospital stay. At all time points assessed, VIS is more predictive of poor short term outcome than IS. VIS may be useful as an independent predictor of outcomes.

IntroductionLimited evidence suggests that serum alkaline phosphatase activity may decrease after cardiac surgery in adults and children. The importance of this finding is not known. Recent studies, however, have identified a potential role for alkaline phosphatase as modulator of inflammation in multiple settings, including during adult cardiopulmonary bypass. We sought to describe the change in alkaline phosphatase activity after cardiothoracic surgery in infants and to assess for a correlation with intensity and duration of post-operative support, markers of inflammation, and short-term clinical outcomes.MethodsSub-analysis of a prospective observational study on the kinetics of procalcitonin in 70 infants (≤90 days old) undergoing cardiothoracic surgery. Subjects were grouped based on the use of cardiopulmonary bypass and delayed sternal closure. Alkaline phosphatase, procalcitonin, and C-reactive protein (CRP) levels were obtained pre-operation and on post-operative day 1. Mean change in alkaline phosphatase activity was determined in each surgical group. Generalized linear modeling and logistic regression were employed to assess for associations between post-operative alkaline phosphatase activity and post-operative support, inflammation, and short term outcomes. Primary endpoints were vasoactive-inotropic score at 24 hours and length of intubation. Secondary endpoints included procalcitonin/CRP levels on post-operative day 1, length of hospital stay, and cardiac arrest or death.ResultsMean decrease in alkaline phosphatase was 30 U/L (p = 0.01) in the non-bypass group, 114 U/L (p<0.0001) in the bypass group, and 94 U/L (p<0.0001) in the delayed sternal closure group. On multivariate analysis, each 10 U/L decrease in alkaline phosphatase activity on post-operative day 1 was independently associated with an increase in vasoactive-inotropic score by 0.7 (p<0.0001), intubation time by 6% (p<0.05), hospital stay by 5% (p<0.05), and procalcitonin by 14% (P<0.01), with a trend towards increased odds of cardiac arrest or death (OR 1.3; p = 0.06). Post-operative alkaline phosphatase activity was not associated with CRP (p = 0.7).ConclusionsAlkaline phosphatase activity decreases after cardiothoracic surgery in infants. Low post-operative alkaline phosphatase activity is independently associated with increased procalcitonin, increased vasoactive/inotropic support, prolonged intubation time, and prolonged hospital stay. Alkaline phosphatase may serve as a biomarker and potential modulator of post-operative support and inflammation following cardiothoracic surgery in infants.

In recent decades, survival of children with complex congenital heart disease has improved considerably. Specifically, children with a variety of congenital heart defects resulting in ‘single ventricle’ physiology can now undergo palliative surgery that allows survival beyond the neonatal period, and in many cases into adulthood, despite having a single functional ventricular pumping chamber supplying both the pulmonary and systemic circulation. Our growing understanding of the functionally univentricular heart has resulted in freedom from Fontan failure of >50% at 25 years post-Fontan. Yet there is still a fair amount of knowledge to be gained, specifically as it relates to the pulmonary circulation in this group of patients. Knowledge gaps relate not only to the pulmonary circulation after Fontan operation, but also at each stage of the single ventricle surgical palliation, including the native physiology prior to any intervention. The pulmonary circulation is affected by multiple issues related to the single ventricle, including specific details of the anatomy unique to each patient, any intervention(s) undertaken, and potential complications such as aortopulmonary collaterals, protein losing enteropathy, plastic bronchitis, venovenous collaterals, pulmonary arteriovenous fistulae, ventricular dysfunction, pulmonary venous stenosis, and more. This chapter will review the current knowledge with regard to the pulmonary circulation in the single ventricle patient, primarily after the Fontan operation. Additionally, it is our hope to help the practitioner assess the pulmonary circulation in the single ventricle patient; we will also discuss the evidence behind and approach to treatment strategies in order to optimize the pulmonary circulation in this complex group of patients.

Background. Cardiovascular complications contribute to the high morbidity and mortality rate among children with anorexia nervosa (AN). Advances in cardiac imaging permit a more comprehensive assessment of myocardial performance in children that could not be previously obtained with conventional imaging. Myocardial strain analysis is an emerging quantitative echocardiographic technique to characterize global and regional ventricular function in children. Objective. To assess global and regional left ventricular (LV0 function in children newly diagnosed with AN with conventional and quantitative 2-dimensional speckle tracking echocardiographic (2DSTE)–derived strain imaging. Materials. In a cross-sectional study of 30 patients with AN (DSM-5) and 14 age-, sex-, and race-matched healthy children, markers of cardiovascular risk, conventional and 2DSTE measures of LV function, and structure were evaluated and compared. The AN cohort was further stratified by behavioral patterns (restrict, exercise, or purge). Results. Conventional measures and LV global strain were similar between controls and children with AN. A subgroup of AN children with purging behavior had LV remodeling characterized by significantly decreased LV mass index. Regional ventricular function at the apex, as measured by strain, was also decreased in all AN patients. Percent change from ideal body weight, body mass index Z-score, electrolyte profiles, heart rate, and blood pressure were similar. Conclusions. Subclinical regional ventricular dysfunction is present in children with AN. Ventricular remodeling exists in a subgroup of children with AN in association with purging behavior. Future studies may utilize strain imaging to identify those AN patients who are at an increased risk for developing significant cardiac dysfunction.

BackgroundThe utility of procalcitonin and C-reactive protein (CRP) as infectious biomarkers following infant cardiothoracic surgery is not well defined.MethodsWe designed a prospective cohort study to evaluate procalcitonin and CRP after infant cardiothoracic surgery. Procalcitonin and CRP were drawn pre-operatively and 24/72 hours post-operation or daily in delayed sternal closure patients. Presence of infection within 10 days of surgery, vasoactive-inotropic scores at 24 and 72 hours, and length of intubation, intensive care unit stay, and hospital stay were documented.ResultsProcalcitonin and CRP were elevated at 24 hours. Procalcitonin then decreased while CRP increased in patients undergoing delayed sternal closure or cardiopulmonary bypass. In the delayed sternal closure group, procalcitonin was significantly higher on post-operative days 2–5 in patients who ultimately developed infection. Higher procalcitonin was independently associated with increased vasoactive-inotropic score at 72 hours. CRP did not correlate with infection or post-operative support.ConclusionsProcalcitonin rises after cardiothoracic surgery in infants but decreases by 72 hours while CRP remains elevated. Sternal closure may affect CRP but not procalcitonin. Procalcitonin is independently associated with circulatory support requirements at 72 hours post-operation and development of infection. Procalcitonin may have greater utility as a biomarker in this population.

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Background: Bicuspid aortic valve (BAV) is the most prevalent congenital heart defect affecting 1% to 2% of the population. It is associated with ascending aorta dilatation. Valve morphology, aortic stenosis (AS), and aortic insufficiency (AI) have been proposed as potential risk factors; however, evaluating their role is difficult, as these factors are inherently related. The aim of this study was to determine whether BAV morphology and dysfunction are independent determinants for ascending aorta dilatation in pediatric patients. Methods: A multicenter, retrospective, cross-sectional study of pediatric BAV patients followed since 2004 was performed. Imaging data were assessed for BAV morphology, severity of AS and AI, history of coarctation, and aortic dimensions. Associations were determined using multivariable regression analysis. A subset of patients undergoing aortic interventions (balloon dilation or Ross) were assessed longitudinally. Results: Data were obtained from 2122 patients (68% male; median age 10.2 years). Fifty percent of patients had ascending aorta dilatation. Right and noncoronary cusp fusion, increasing AS and AI, and older age were independently associated with ascending aorta dilatation. A history of coarctation was associated with less ascending aorta dilatation. In patients with neither AS nor AI, 37% had ascending aorta dilatation (4% severe). No complications related to aortic dilatation occurred in this cohort. Aortic Z scores were determined, and a Z -score calculator was created for this population. Conclusions: In this large pediatric cohort of patients with BAV, valve morphology, AS, and AI are independently associated with ascending aorta dilatation, suggesting that hemodynamic factors influence aortopathy. However, even in BAVs with no AS or AI, there is significant ascending aorta dilatation independent of valve morphology. Interventions that led to changes in degree of AI and AS did not seem to influence change in aortic dimensions. The current BAV cohort can be used as a reference group for expected changes in aortic dimensions during childhood.

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