Explore the vast range of titles available.

•Poor exercise capacity and endothelial dysfunction accelerate Fontan disease.•We report baseline data for pediatric Fontan patients in an exercise intervention.•Single ventricle systolic function (3D-EF and strain) was reduced in this cohort.•Diminished systolic function correlated with decreased endothelial function.•Single ventricle systolic function and strain correlate with decreased exercise capacity. By age 40, roughly half of the individuals with Fontan circulation will have died or undergone heart transplantation. Poor exercise capacity and endothelial dysfunction accelerate disease progression. This study aims to assess the systolic function of the single ventricle (SV) in pediatric Fontan patients entering an exercise intervention (RE-ENERGIZE FONTAN) and how it is associated with exercise capacity and endothelial function. This cohort comes from an ongoing randomized trial in Fontan patients, using live video conferencing for supervised exercise. Participants (ages 8-19) cleared for exercise underwent 2D/3D echocardiograms, cardiopulmonary testing, and endothelial function (RHI). 2D longitudinal strain (LS, right ventricle), 2D global longitudinal strain (GLS, left ventricle), and circumferential strain were measured with TomTec, and 3D ejection fraction (3D-EF) and 3D-GLS were calculated. We have enrolled 114 Fontan patients. Median age was 12.7 years (IQR 10.2, 15.6). The median time from Fontan operation was 8.8 years (IQR 6.2, 12.0). Fifty-seven patients (50%) had a single right ventricle. SV systolic performance measures were: 2D-LS/GLS −15.6% ± 4.19%, circumferential strain was −18.7% ± 6.83%, 3D-EF 49.9% ± 7.26%, and 3D-GLS −16.8% ± 4.37%. Peak VO2 was 1,290 ± 502 mL/min, and percent predicted peak VO2 was 67.8% ± 15.6%. RHI was 1.44 ± 0.576. 2D-LS/GLS and 3D EF correlated with percent predicted peak VO2 (R = −0.28, P = .007 and R = 0.24, P = .019). 3D-EF correlated positively with RHI (R = 0.29, P = .0071). In this cohort of pediatric Fontan patients, SV systolic function was diminished at baseline, and there was a direct correlation between 2D strain and 3D-EF with percent predicted peak VO2, and additionally, 3D-EF with endothelial function. NCT04195451. [Display omitted]

Introduction Acute kidney injury (AKI) is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Renal near-infrared spectroscopy (NIRS) is used to evaluate regional oximetry in a non-invasive continuous real-time fashion, and reflects tissue perfusion. The aim of this study was to evaluate the relationship between renal oximetry and development of AKI in the operative and post-operative setting in infants undergoing cardiopulmonary bypass surgery. Methods In this prospective study, we enrolled 59 infants undergoing cardiopulmonary bypass surgery for congenital heart disease for univentricular ( n  = 26) or biventricular ( n  = 33) repair. Renal NIRS was continuously measured intraoperatively and for at least 24 hours postoperatively and analysed for the intraoperative and first 12 hours, first 24 hours and first 48 hours postoperatively. The renal oximetry values were correlated with the paediatric risk, injury, failure, loss, end (pRIFLE) classification for AKI, renal biomarkers and the postoperative course. Results Twenty-eight (48%) infants developed AKI based on pRIFLE classification. Already during intraoperative renal oximetry and further in the first 12 hours, 24 hours and 48 hours postoperatively, significantly lower renal oximetry values in AKI patients compared with patients with normal renal function were recorded ( P  < 0.05). Of the 28 patients who developed AKI, 3 (11%) needed renal replacement therapy and 2 (7%) died. In the non-AKI group, no deaths occurred. Infants with decreased renal oximetry values developed significantly higher lactate levels 24 hours after surgery. Cystatin C was a late parameter of AKI, and neutrophil gelatinase-associated lipocalin values were not correlated with AKI occurrence. Conclusion Our results suggest that prolonged low renal oximetry values during cardiac surgery correlate with the development of AKI and may be superior to conventional biochemical markers. Renal NIRS might be a promising non-invasive tool of multimodal monitoring of kidney function and developing AKI in infants undergoing cardiac surgery with cardiopulmonary bypass.

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care – structured evaluation, psychosocial/spiritual, and communication support before surgery – or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (−7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.

Background Total cavopulmonary connection (TCPC) is a definitive palliative procedure for functionally univentricular congenital heart disease. The study aims to compare the impact of on-pump cardioplegic arrest and on-pump beating heart cardiopulmonary bypass (CPB) on the prognosis of pediatric patients undergoing extracardiac TCPC. Methods The medical data of patients (< 18 years) who underwent extracardiac TCPC with CPB between January 2008 and December 2020 in the cardiac surgery center were retrospectively analyzed. Depending on CPB strategies, the patients were assigned to the beating-heart (BH) and cardioplegic arrest (CA) groups. Data including baseline characteristics, intra/postoperative variables, and clinical outcomes were collected for analysis with 1:1 propensity score matching and multivariable stepwise logistic regressions. Results Fifty-seven matched patient pairs were obtained. No significant difference existed between the two groups in the in-hospital mortality (3.5% vs. 1.8%, P  = 1) and one-year survival rate (100% vs. 96.4%, P  = 0.484). The BH group had significantly less intraoperative platelet transfusion (10 mL vs. 150 mL, P  = 0.019) and blood loss (100 mL vs. 150 mL, P  = 0.033) than the CA group. The CA group had significantly higher vasoactive-inotropic scores ( P  < 0.05) and longer postoperative ICU stays (2.0 d vs. 3.7 d, P  = 0.017). No significant difference existed between the two groups in the incidence of postoperative adverse events. Conclusion Although both CPB strategies are safe and feasible for extracardiac TCPC, the BH technique would cause less intraoperative platelet transfusion and blood loss, and achieve faster early-term postoperative recovery.

This report describes transplantation of hearts from three infant donors (mean age at donation, 3.7 days) who had died from cardiocirculatory causes. The recipients (mean age, 2.2 months) all survived to 6 months with excellent left ventricular function. This approach to transplantation has been controversial but offers the prospect of expanding the donor pool. This report describes transplantation of hearts from three infant donors who had died from cardiocirculatory causes. The recipients all survived to 6 months with excellent left ventricular function. This controversial approach offers the prospect of expanding the donor pool. Pediatric heart transplantation after the declaration of brain death in donors has been performed for more than 25 years in more than 6000 recipients. 1 The first successful heart-transplant procedure in an infant was reported 20 years ago, and decades-long survival has been reported. 1 , 2 The average survival for children who are alive 1 year after transplantation is more than 15 years and exceeds that of adults. 3 However, the risk of death while awaiting a donor is highest for children awaiting a cardiac transplant. 4 Infant heart-transplant recipients face up to a 25% waiting-list mortality, which is an order of magnitude higher . . .

Background The administration of levosimendan prophylactically to patients undergoing cardiac surgery remains a controversial practice, and few studies have specifically assessed the value of this approach in pediatric patients. This study therefore sought to explore the safety and efficacy of prophylactic levosimendan administration to pediatric patients as a means of preventing low cardiac output syndrome (LCOS) based upon hemodynamic, biomarker, and pharmacokinetic readouts. Methods This was a single-center, double-blind, randomized, placebo-controlled trial. Patients ≤ 48 months old were enrolled between July 2018 and April 2019 and were randomly assigned to groups that received either placebo or levosimendan infusions for 48 h post-surgery, along with all other standard methods of care. LCOS incidence was the primary outcome of this study. Results A total of 187 patients were enrolled, of whom 94 and 93 received levosimendan and placebo, respectively. LCOS incidence did not differ significantly between the levosimendan and placebo groups (10 [10.6%] versus 18 [19.4%] patients, respectively; 95% confidence interval [CI] 0.19–1.13; p  = 0.090) nor did 90-day mortality (3 [3.2%] versus 4 [4.3%] patients, CI 0.14–3.69, p  = 0.693), duration of mechanical ventilation (median, 47.5 h and 39.5 h, respectively; p  = 0.532), ICU stay (median, 114.5 h and 118 h, respectively; p  = 0.442), and hospital stay (median, 20 days and 20 days, respectively; p  = 0.806). The incidence of hypotension and cardiac arrhythmia did not differ significantly between the groups. Levels of levosimendan fell rapidly without any plateau in plasma concentrations during infusion. A multiple logistic regression indicated that randomization to the levosimendan group was a predictor of LCOS. Conclusions Prophylactic levosimendan administration was safe in pediatric patients and had some benefit to postoperative hemodynamic parameters, but failed to provide significant benefit with respect to LCOS or 90-day mortality relative to placebo. Trial registration Name of the registry: Safety evaluation and therapeutic effect of levosimendan on the low cardiac output syndrome in patients after cardiopulmonary bypass. Trial registration number: ChiCTR1800016594. Date of registration: 11 June 2018. URL of trial registry record: http://www.chictr.org.cn/index.aspx

ObjectivesThe aim of the present study was to assess the efficacy of feeding a higher-density formula (HDF) in infant with congenital heart disease (CHD).MethodsIn a parallel randomized trial, infants (6 to 12 months) who underwent CHD corrective surgery received either a standard-density formula (SDF, 67 kcal /100 ml) or an HDF (90 kcal/100 ml) after discharge from the intensive care unit for 8 weeks. In addition to the formula, infants could receive breast milk or complementary food. Anthropometry, biochemistry, and formula intake were collected.ResultSixty-four infants completed the study (n = 32 in each group). All infants gained weight. The mean ± standard deviation (SD) of weight z score at baseline and week-8 were −2.38 ± 10.04 to −1.38 + 0.97 in the SDF group and −2.69 ± 1.19 to −0.89 ± 0.90 in the HDF group (between-group p = 0.0001). Both groups gained length, but showed a decline in length z-score which was significant in the SDF group but not significant in the HDF group. Mid-upper arm circumference and its z score improved in both groups, with more improvement in the HDF group. Serum albumin level was higher in the HDF than the SDF group at week-8, but no significant between-group differences were observed in hemoglobin, serum ferritin, or iron. Symptoms of gastrointestinal intolerance were not reported, but parents of 4 infants in the HDF group complained of their infants’ constipation.ConclusionFeeding infants using a concentrated formula could increase infants’ weight gain and growth, and improve the nutritional status after CHD surgery.

For decades, physicians have administered corticosteroids in the perioperative period to infants undergoing heart surgery with cardiopulmonary bypass (CPB) to reduce the postoperative systemic inflammatory response to CPB. Some question this practice because steroid efficacy has not been conclusively demonstrated and because some studies indicate that steroids could have harmful effects. STRESS is a randomized, placebo-controlled, double-blind, multicenter trial designed to evaluate safety and efficacy of perioperative steroids in infants (age < 1 year) undergoing heart surgery with CPB. Participants (planned enrollment = 1,200) are randomized 1:1 to methylprednisolone (30 mg/kg) administered into the CPB pump prime versus placebo. The trial is nested within the existing infrastructure of the Society of Thoracic Surgeons Congenital Heart Surgery Database. The primary outcome is a global rank score of mortality, major morbidities, and hospital length of stay with components ranked commensurate with their clinical severity. Secondary outcomes include several measures of major postoperative morbidity, postoperative hospital length of stay, and steroid-related safety outcomes including prevalence of hyperglycemia and postoperative infectious complications. STRESS will be one of the largest trials ever conducted in children with heart disease and will answer a decades-old question related to safety and efficacy of perioperative steroids in infants undergoing heart surgery with CPB. The pragmatic “trial within a registry” design may provide a mechanism for conducting low-cost, high-efficiency trials in a heretofore-understudied patient population.

The Fontan operation is a common end point for children born with a single functional ventricle. Fontan patients typically experience physiological deterioration leading to transplant or death in their third or fourth decades of life. This deterioration is partially attributable to progressive increases in pulmonary vascular resistance (PVR) and as such endothelin receptor antagonists, which are known to decrease pulmonary vascular resistance, have been proposed as potentially beneficial in this population. We conducted a single-center, randomized, double-blind, placebo-controlled, crossover study of 12 weeks of ambrisentan therapy (10 mg per day) versus placebo to test the hypothesis that endothelin receptor antagonism will improve cardiopulmonary exercise test parameters and 36-item short form (SF-36) assessed quality of life in adult Fontan patients. Twenty-eight patients entered the trial, 19 patients completed the protocol. Ambrisentan therapy improved peak oxygen consumption by 1.7 ml/kg/min in patients who achieved a respiratory exchange ratio of >0.95 (p = 0.05) and decreased the slope of the ventilatory equivalent ratio for oxygen (−2.8, p = 0.019) in all completers. It did not change SF-36 physical function score compared with placebo (p = 0.28). Ambrisentan therapy resulted in a decrease in (−1.4 g/dl, p <0.001) with no change in liver or renal function. Therapy was generally well tolerated, with no greater rate of side effects than placebo. In conclusion, ambrisentan is well tolerated and improves exercise capacity in adult Fontan patients.

Objective To investigate the safety and effects of a restrictive red blood cell (RBC) transfusion strategy in pediatric cardiac surgery patients. Design Randomized controlled trial. Setting Pediatric ICU in an academic tertiary care center, Leiden University Medical Center, Leiden, The Netherlands. Patients One hundred seven patients with non-cyanotic congenital heart defects between 6 weeks and 6 years of age. One hundred three patients underwent corrective surgery on cardiopulmonary bypass. Interventions Prior to surgery patients were randomly assigned to one of two groups with specific RBC transfusion thresholds: Hb 10.8 g/dl (6.8 mmol/l) and Hb 8.0 g/dl (5.0 mmol/l). Measurements Length of stay in hospital (primary outcome), length of stay in PICU, duration of ventilation (secondary outcome), incidence of adverse events and complications related to randomization (intention to treat analysis). Results In the restrictive transfusion group, mean volume of transfused RBC was 186 (±70) ml per patient and in the liberal transfusion group 258 (±87) ml per patient, (95 % CI 40.6–104.6), p  < 0.001. Length of hospital stay was shorter in patients with a restrictive RBC transfusion strategy: median 8 (IQR 7–11) vs. 9 (IQR 7–14) days, p  = 0.047. All other outcome measures and incidence of adverse effects were equal in both RBC transfusion groups. Cost of blood products for the liberal transfusion group was 438.35 (±203.39) vs. 316.27 (±189.96) euros (95 % CI 46.61–197.51) per patient in the restrictive transfusion group, p  = 0.002. Conclusions For patients with a non-cyanotic congenital heart defect undergoing elective cardiac surgery, a restrictive RBC transfusion policy (threshold of Hb 8.0 g/dl) during the entire perioperative period is safe, leads to a shorter hospital stay and is less expensive.