Risk factors for death or mechanical ventilation time after bidirectional cavopulmonary anastomosis in a developing country.

BACKGROUND: Glenn procedure is performed for patients with cyanotic CHD and univentricular physiology and has a survival rate above 90%. AIM: To evaluate the risk factors associated with a poor outcome after Glenn procedure. METHODS: The data for this retrospective analysis were collected from a regional Brazilian registry of congenital heart surgeries (ASSIST initiative) from 2014 to 2019. Data from 97 patients who underwent the Glenn procedure were considered. The primary outcomes were prolonged mechanical ventilation (>24 hours post-operatively) or in-hospital death, alone and combined. RESULTS: The overall in-hospital mortality was 13.4% (n = 13). Prolonged mechanical ventilation occurred in 52% (n = 51) of the patients. Our analysis found that risk factors for death or prolonged post-operative mechanical ventilation were cardiopulmonary bypass duration and post-operative lactate, whereas weight-for-age z-score and age at surgery were risk factors for prolonged mechanical ventilation. Cardiopulmonary bypass duration increased (adjusted odds ratio: 1.02; 95 % CI: 1.01, 1.03) and age at surgery decreased (adjusted odds ratio: 0.96; 95 % CI: 0.93, 0.99) the odds of the combined outcome. CONCLUSIONS: Age at surgery, post-operative lactate, and low-weight-for-age z-score are associated with prolonged mechanical ventilation and death following the Glenn procedure.

Prevalence and Outcomes of Congenital Heart Disease in Very Low Birth Weight Preterm Infants: An Observational Study From the Brazilian Neonatal Network Database.

OBJECTIVES: To evaluate the prevalence of congenital heart disease and their outcomes in a Brazilian cohort of very low birth weight preterm infants. DESIGN: Post hoc analysis of data from the Brazilian Neonatal Network database, complemented by retrospective data from medical charts and a cross-sectional survey. SETTING: Twenty public tertiary-care university hospitals. PATIENTS: A total of 13,955 newborns weighing from 401 to 1,499 g and between 22 and 36 weeks of gestational age, born from 2010 to 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The prevalence of congenital heart disease was 2.45% (95% CI, 2.20-2.72%). In a multivariate regression analysis, risk factors associated with congenital heart disease were maternal diabetes (relative risk, 1.55; 95% CI, 1.11-2.20) and maternal age above 35 years (relative risk, 2.09; 95% CI, 1.73-2.51), whereas the protection factors were maternal hypertension (relative risk, 0.54; 95% CI, 0.43-0.69), congenital infection (relative risk, 0.45; 95% CI, 0.21-0.94), and multiple gestation (relative risk, 0.73; 95% CI, 0.55-0.97). The pooled standardized mortality ratio in patients with congenital heart disease was 2.48 (95% CI, 2.22-2.80), which was significantly higher than in patients without congenital heart disease (2.08; 95% CI, 2.03-2.13). However, in multiple log-binomial regression analyses, only the presence of major congenital anomaly, gestational age (< 29 wk; relative risk, 2.32; 95% CI, 2.13-2.52), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20; relative risk, 3.76; 95% CI, 3.41-4.14) were independently associated with death, whereas the effect of congenital heart disease was spotted only when a conditional inference tree approach was used. CONCLUSIONS: The overall prevalence of congenital heart disease in this cohort of very low birth weight infants was higher and with higher mortality than in the general population of live births. The occurrence of a major congenital anomaly, gestational age (< 29 wk), and Score for Neonatal Acute Physiology and Perinatal Extension II (> 20) were significantly and independently associated with death, whereas the association of congenital heart disease and death was only evident when a major congenital anomaly was present.

Increased Atrial ß-Adrenergic Receptors and GRK-2 Gene Expression Can Play a Fundamental Role in Heart Failure After Repair of Congenital Heart Disease with Cardiopulmonary Bypass.

Surgeries to correct congenital heart diseases are increasing in Brazil and worldwide. However, even with the advances in surgical techniques and perfusion, some cases, especially the more complex ones, can develop heart failure and death. A retrospective study of patients who underwent surgery for correction of congenital heart diseases with cardiopulmonary bypass (CPB) in a university tertiary-care hospital that died, showed infarction in different stages of evolution and scattered microcalcifications in the myocardium, even without coronary obstruction. CPB is a process routinely used during cardiac surgery for congenital heart disease. However, CPB has been related to increased endogenous catecholamines that can lead to major injuries in cardiomyocytes. The mechanisms involved are not completely understood. The aim of this study was to evaluate the alterations induced in the ß-adrenergic receptors and GRK-2 present in atrial cardiomyocytes of infants with congenital heart disease undergoing surgical repair with CPB and correlate the alterations with functional and biochemical markers of ischemia/myocardial injury. The study consisted of right atrial biopsies of infants undergoing surgical correction in HC-FMRPUSP. Thirty-three cases were selected. Atrial biopsies were obtained at the beginning of CPB (group G1) and at the end of CPB (group G2). Real-time PCR, Western blotting, and immunofluorescence analysis were conducted to evaluate the expression of ß1, ß2-adrenergic receptors, and GRK-2 in atrial myocardium. Cardiac function was evaluated by echocardiography and biochemical analysis (N-terminal pro-brain natriuretic peptide (NT-ProBNP), lactate, and cardiac troponin I). We observed an increase in serum lactate, NT-proBNP, and troponin I at the end of CPB indicating tissue hypoxia/ischemia. Even without major clinical consequences in cardiac function, these alterations were followed by a significant increase in gene expression of ß1 and ß2 receptors and GRK-2, suggesting that this is one of the mechanisms responsible for the exacerbated response of cardiomyocytes to circulating catecholamines. These alterations could explain the irreversible myocardial damage and lipid peroxidation of membranes classically attributed to catecholamine excess, observed in some infants who develop heart failure and postoperative death. Although other factors may be involved, this study confirms that CPB acts as a potent inducer of increased gene expression of ß- adrenergic receptors and GRK-2, making the myocardium of these infants more susceptible to the effects of circulating endogenous catecholamines, which may contribute to the development of irreversible myocardial damage and death.

Discrepancies Between Clinical Diagnoses and Autopsy Findings in Critically Ill Children: A Prospective Study.

OBJECTIVES: To evaluate the discrepancies between clinical and autopsy diagnoses in patients who died in the pediatric intensive care units (PICUs) of a tertiary care university hospital. METHODS: A prospective study of all consecutive autopsies discussed at monthly mortality conferences over 5 years. Discrepancies between premortem and autopsy diagnoses were classified according to modified Goldman et al criteria. RESULTS: From January 1, 2011, to December 31, 2015, a total of 2,679 children were admitted to the two PICUs of our hospital; 257 (9.6%) died, 150 (58.4%) underwent autopsy, and 123 were included. Complete concordance between clinical and postmortem diagnoses was observed in 86 (69.9%) patients; 20 (16.3%) had a class I discrepancy, and eight (6.5%) had a class II discrepancy. Comparing 2011 and 2015, the rate of major discrepancies decreased from 31.6% to 15%. CONCLUSIONS: Our results emphasize the importance of autopsy to clarify the cause of death and its potential contribution to improvement of team performance and quality of care.

Outcomes of Patients After Arterial Switch Operation: 18 Years of Experience in a Single Medium-Volume Center.

The objective of this paper was to describe the outcomes in patients submitted to arterial switch operation and to analyze the predictors of in-hospital mortality and further need of re-operation at a single-center institution. Between September 1995 and January 2014, 128 consecutive arterial switch operations were performed. Surgical mortality during this period was analyzed retrospectively, and a follow-up analysis of the survivors was conducted. Surgical era, cardiopulmonary bypass time (p = 0.001), and diagnosis category (p = 0.025) influenced in-hospital mortality. The estimated overall survival for the 91 hospital survivors was 96.8, 96.4, and 96.2 % at 5, 10, and 15 years, respectively. The median follow-up time was 67 months (range 0.71-222 months). Three patients (5 %) presented severe aortic regurgitation. Right ventricle outflow tract systolic gradient by echocardiography was above 60 mmHg in 2 %. Late re-interventions occurred in 12 (13 %) patients with mean time of 64 ± 34 months after the initial procedure. Actuarial freedom from re-interventions at 5, 10, and 15 years was 96.4, 69.7, and 61.9 %, respectively. Arterial switch operation remains the procedure of choice in patients with transposition of great arteries. It can be performed even in middle-volume institutions, leading to the same middle- and long-term outcomes of high-volume institutions. Early high mortality rate may occur due not only to learning curve, but also to cardiopulmonary bypass time and ventricular septal defect closure.

Use of the pressure wire method for measuring pulmonary arterial pressures in patients with pulmonary atresia.

OBJECTIVE: The objective of the study was to analyse the use of the pressure wire for the acquisition of intravascular pulmonary pressures in the presence of pulmonary atresia and systemic-dependent pulmonary blood flow. METHODS: In this study, we included patients with pulmonary atresia and systemic-dependent pulmonary circulation referred for diagnostic catheterisation for evaluation of pulmonary pressures during the period from April, 2012 to April, 2013. The systemic-pulmonary collateral arteries were selectively catheterised, and in the absence of a critical stenosis angiographically determined; the pressure wire was introduced in these arteries to reach the main pulmonary artery, and/or lobar, and segmental branches. Aortic and pulmonary pressures were simultaneously obtained. We evaluated the feasibility and safety of the method. RESULTS: We studied 10 patients (age 21 days to 11 years). In all of them, the pressures of pulmonary circulation - main artery, and/or lobar, and segmental branches - were successfully measured with the pressure wire. Of eight patients with indication for Rastelli surgery, the pulmonary pressures were considered normal in five, and slightly increased in three. In two patients requiring univentricular correction - total cavopulmonary anastomosis - the diastolic pressure was increased (20 mmHg). All procedures were performed without haemodynamic instability, cardiac arrhythmia, systemic saturation reduction, death, or any other complication. CONCLUSION: Measurement of pulmonary vascular pressures using the pressure wire in small patients with pulmonary atresia is safe and effective. It allows the acquisition of reliable pressure curves, even in the presence of small vessels, bending and tortuosity, without the risk usually associated with the use of conventional diagnostic catheters.

Inflammation, myocardial dysfunction, and mortality in children with septic shock: an observational study.

We aimed to investigate whether nuclear factor kappa-B activation, as evaluated by gene expression of its inhibitor (I-κBα) and cytokine serum levels, was associated with myocardial dysfunction and mortality in children with septic shock. Twenty children with septic shock were prospectively enrolled and grouped according to ejection fraction (EF) <45% (group 1) or EF ≥45% (group 2) on the first day after admission to the pediatric intensive care unit. No interventions were made. In the first day, patients from group 1 (n = 6) exhibited significantly greater tumor necrosis factor-alpha (TNF-α) and interleukin (IL)-10 plasma levels. However, I-κBα gene expression was not different in both groups. Mortality and number of complications were significantly greater in group 1. Patients who died had greater plasma concentrations of TNF-α. In conclusion, TNF-α and IL-10 are involved in myocardial dysfunction accompanying septic shock in children, and TNF-α is associated with mortality.

Oxidative stress markers are not associated with outcomes after pediatric heart surgery.

OBJECTIVES: To investigate whether perioperative serum levels of oxidative stress markers, thiobarbituric acid reactive substances (TBARS), and carbonyl moieties are associated with outcomes in children after heart surgery. BACKGROUND: Oxidative stress markers are increased following heart surgery with cardiopulmonary bypass (CPB) and can play a role in ischemia-reperfusion injury, but its associations with myocardial dysfunction, low cardiac output syndrome (LCOS), and outcomes are not proven. METHODS: In a retrospective secondary analysis of a cohort study comprising 55 children (median age, 109 [2-611] days), we compared pre-, intra- and postoperative serum levels of TBARS and carbonyl moieties among patients with and without postoperative LCOS, cyanotic and acyanotic congenital heart disease (CHD), and survivors and nonsurvivors. We also assessed the independent effect of TBARS and carbonyl moieties peak levels on the mortality-adjusted hospital length of stay (aLOS). RESULTS: Patients who developed postoperative LCOS (n = 36) were significantly younger, more frequently cyanotic, more severely ill, and underwent more complex procedures with longer CPB. However, TBARS and carbonyl moieties serum levels did not change significantly over time. Moreover, they were not significantly different in patients with or without LCOS, cyanotic and acyanotic CHD, or survivors and nonsurvivors. There was a significant correlation between TBARS and tumor necrosis factor alpha (TNF-α) peak serum levels. Neither TBARS nor carbonyl moieties peak serum levels were independently associated with aLOS. CONCLUSIONS: In conclusion, oxidative stress markers TBARS and carbonyl moieties were not associated with the development of LCOS, the aLOS, or mortality in children after heart surgery with CPB.

Catch-up growth in children after repair of Tetralogy of Fallot.

PURPOSE: To evaluate the growth of children after repair of Tetralogy of Fallot, as well as the influence of residual lesions and socio-economic status. METHODS: A total of 17 children, including 10 boys with a median age of 16 months at surgery, were enrolled in a retrospective cohort, in a tertiary care university hospital. Anthropometric (as z-scores), clinical, nutritional, and social data were collected. RESULTS: Weight-for-age and weight-for-height z-scores decreased pre-operatively and recovered post-operatively in almost all patients, most markedly weight for age. Weight-for-height z-scores improved, but were still lower than birth values in the long term. Long-term height-for-age z-scores were higher than those at birth, surgery, and 3 months post-operatively. Most patients showed catch-up growth for height for age (70%), weight for age (82%), and weight for height (70%). Post-operative residual lesions (76%) influenced weight-for-age z-scores. Despite the fact that most patients (70%) were from low-income families, energy intake was above the estimated requirement for age and gender in all but one patient. There was no influence of socio-economic status on pre- and post-operative growth. Bone age was delayed and long-term-predicted height was within mid-parental height limits in 16 children (93%). CONCLUSION: Children submitted to Tetralogy of Fallot repair had pre-operative acute growth restriction and showed post-operative catch-up growth for weight and height. Acute growth restriction could still be present in the long term.

Effect of oral hygiene with 0.12% chlorhexidine gluconate on the incidence of nosocomial pneumonia in children undergoing cardiac surgery.

OBJECTIVE: To evaluate the effect of oral hygiene with 0.12% chlorhexidine gluconate on the incidence of nosocomial pneumonia and ventilator-associated pneumonia (VAP) in children undergoing cardiac surgery. DESIGN: Prospective, randomized, double-blind, placebo-controlled trial. SETTING: Pediatric intensive care unit (PICU) at a tertiary care hospital. PATIENTS: One hundred sixty children undergoing surgery for congenital heart disease, randomized into 2 groups: chlorhexidine (n = 87) and control (n = 73). INTERVENTIONS: Oral hygiene with 0.12% chlorhexidine gluconate or placebo preoperatively and twice a day postoperatively until PICU discharge or death. RESULTS: Patients in experimental and control groups had similar ages (median, 12.2 vs 10.8 months; P = .72) and risk adjustment for congenital heart surgery 1 score distribution (66% in category 1 or 2 in both groups; P = .17). The incidence of nosocomial pneumonia was 29.8% versus 24.6% (P = .46) and the incidence of VAP was 18.3% versus 15% (P = .57) in the chlorhexidine and the control group, respectively. There was no difference in intubation time (P = .34), need for reintubation (P = .37), time interval between hospitalization and nosocomial pneumonia diagnosis (P = .63), time interval between surgery and nosocomial pneumonia diagnosis (P = .10), and time on antibiotics (P = .77) and vasoactive drugs (P = .16) between groups. Median length of PICU stay (3 vs 4 days; P = .53), median length of hospital stay (12 vs 11 days; P = .67), and 28-day mortality (5.7% vs 6.8%; P = .77) were also similar in the chlorhexidine and the control group. CONCLUSIONS: Oral hygiene with 0.12% chlorhexidine gluconate did not reduce the incidence of nosocomial pneumonia and VAP in children undergoing cardiac surgery. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT00829842 .

Risk stratification in neonates and infants submitted to cardiac surgery with cardiopulmonary bypass: a multimarker approach combining inflammatory mediators, N-terminal pro-B-type natriuretic peptide and troponin I.

Low cardiac output syndrome (LCOS) is a common problem following cardiac surgery with cardiopulmonary bypass (CPB) in neonates and infants, and its early recognition remains a challenging task. We aimed to test whether a multimarker approach combining inflammatory and cardiac markers provides complementary information for prediction of LCOS and death in children submitted to cardiac surgery with CPB. Forty-six children younger than 18 months with congenital heart defects were prospectively enrolled. No intervention was made. Blood samples were collected pre-operatively, during CPB and post-operatively (PO) for measurement of interleukin (IL)-6, IL-8, IL-10, tumor necrosis factor (TNF)-alpha, cardiac troponin I (cTnI) and N-terminal pro-B-type natriuretic peptide (NT-proBNP). Clinical data and outcome variables were recorded. Logistic regression was used to identify predictors of LCOS and death. Multivariate logistic regression identified pre-operative NT-proBNP and IL-8 4h PO as independent predictors of LCOS, while cTnI 4h PO and CPB length were independent predictors of death. The use of inflammatory and cardiac markers in combination improved sensitivity, negative predictive value and accuracy of the models. In conclusion, the combined assessment of inflammatory and cardiac biochemical markers can be useful for identifying young children at increased risk for LCOS and death after heart surgery with CPB.

Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction.

We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence--also on echocardiogram--of left coronary artery trunk obstruction.

Compressão extrínseca da artéria coronária esquerda por dilatação aneurismática do tronco pulmonar em adolescente: involução após oclusão cirúrgica de comunicação interatrial seio venoso e plastia redutora do tronco pulmonar / Extrinsic compression of left main coronary artery from aneurysmal dilation of pulmonary trunk in a adolescent: involution after surgery occlusion of sinus venosus atrial septal defect and pulmonary trunk plasty for reduction

Relatamos o caso de um adolescente encaminhado com o diagnóstico de hipertensão pulmonar. A investigação não invasiva detectou comunicação interatrial seio venoso com sinais de hipertensão pulmonar. No estudo hemodinâmico o diagnóstico foi confirmado, sendo também notada compressão esquerda pelo tronco pulmonar. O paciente foi submetido à oclusão cirúrgica da comunicação interatrial e à plastia redutora do tronco pulmonar. Dois anos após o procedimento, o paciente encontra-se bem, com sinais clínicos e ecocardiográficos de hipertensão pulmonar discreta e sem evidências, também pelo ecocardiograma, de obstrução do tronco da artéria coronária esquerda.

We report the case of an adolescent referred with initial diagnosis of pulmonary hypertension. Non-invasive investigation disclosed a sinus venous atrial septal defect with pulmonary hypertension. The hemodynamic study confirmed diagnosis, and also showed extrinsic compression of left main coronary artery by pulmonary trunk. Surgical closure of the defect in addition to pulmonary trunk plasty were undertaken. Two years after the surgery the patient is well, with clinical signs of mild pulmonary hypertension, and showing no evidence - also on echocardiogram - of left coronary artery trunk obstruction.