Pulmonary Atresia with Intact Ventricular Septum, a National Comparison Between Interventional and Surgical Approach, in Combination with a Systemic Literature Review.

This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocardiogram. Comparisons between groups were performed with Mann-Whitney U-test and Fisher´s exact test. A systematic literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34 (11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no definitive golden standard treatment.

High-sensitive troponinT, interleukin-8, and interleukin-6 link with post-surgery risk in infant heart surgery.

BACKGROUND: This study focuses on biomarkers in infants after open heart surgery, and examines the association of high-sensitive troponin T (hs-cTnT), interleukin-6 (IL-6), and interleukin-8 (IL-8) with postoperative acute kidney injury (AKI), ventilatory support time and need of vasoactive drugs. METHODS: Secondary exploratory study from a double-blinded clinical randomized trial (Mile-1) on 70 infants undergoing open heart surgery with cardiopulmonary bypass (CPB). In this sub-study, the entire study population was examined without considering the study drugs. The biomarkers' peak concentration (highest concentration at 2 or 6 h post-CPB) were used for statistical analyses. RESULTS: Peak IL-8, hs-cTnT, and IL-6 occurred at 2 h post-CPB for 96%, 79%, and 63% of the patients, respectively. The odds ratio of developing AKI2-3 for IL-6 > 293 pg/mL was 23.4 (95% CI 5.3;104.0), for IL-8 > 100 pg/mL it was 11.5 (3.0;44.2), and for hs-cTnT >5597 pg/mL it was 6.1 (1.5; 24.5). In more than two third of the patients with the highest peak concentrations of IL-8, IL-6, and hs-cTnT, there was a need for ventilatory support for >24 h and use of vasoactive drugs at 24 h post-CPB, while in less than one third of the patients with the lowest peak concentrations of IL-8 and hs-cTnT such requirements were observed. CONCLUSIONS: The peak biomarker concentrations and CPB-time strongly predicted AKI2-3, with IL-6 and IL-8 emerging as strongest predictors. Furthermore, our findings suggest that measuring hs-cTnT and IL-8 just 2 h post-CPB-weaning may assist in identifying infants suitable for early extubation and highlight those at risk of prolonged ventilation.

Transcatheter Intervention for Coarctation of the Aorta: A Nordic Population-Based Registry With Long-Term Follow-Up.

BACKGROUND: Coarctation of the aorta (CoA), a congenital narrowing of the proximal descending thoracic aorta, is a relatively common form of congenital heart disease. Untreated significant CoA has a major impact on morbidity and mortality. In the past 3 decades, transcatheter intervention (TCI) for CoA has evolved as an alternative to surgery. OBJECTIVES: The authors report on all TCIs for CoA performed from 2000 to 2016 in 4 countries covering 25 million inhabitants, with a mean follow-up duration of 6.9 years. METHODS: During the study period, 683 interventions were performed on 542 patients. RESULTS: The procedural success rate was 88%, with 9% considered partly successful. Complications at the intervention site occurred in 3.5% of interventions and at the access site in 3.5%. There was no in-hospital mortality. During follow-up, TCI for CoA reduced the presence of hypertension significantly from 73% to 34%, but despite this, many patients remained hypertensive and in need of continuous antihypertensive treatment. Moreover, 8% to 9% of patients needed aortic and/or aortic valve surgery during follow-up. CONCLUSIONS: TCI for CoA can be performed with a low risk for complications. Lifetime follow-up after TCI for CoA seems warranted.

Immunological differences between heart- and kidney-transplanted children: a cross-sectional study.

Post-transplantation lymphoproliferative disorder is a potentially mortal complication after heart transplantation in children. As the immune system plays a crucial role in the development of lymphoma, we explored the influence of thymus function in relation to immunosuppressive treatment in organ-transplanted children and healthy control subjects. A prospective case-control study was performed at a single centre, in which 36 children who had undergone heart transplantation were compared to two control groups: 34 kidney-transplanted children and 33 healthy age- and sex-matched children. T- and B-lymphocyte subtypes and monocytes were analysed by flow cytometry, and T-cell receptor excision circles were assessed using quantitative polymerase chain reaction. Heart-transplanted children had a lymphocyte profile characterised by reduced or absent thymic function with low numbers of T-cell receptor excision circles and total and naïve T cells, together with immune activation against the allograft. Despite similar immunosuppressive treatment, the kidney-transplanted group showed an activated T-lymphocyte compartment.

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension.

AIMS: Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5 year period. METHODS AND RESULTS: Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5 years follow-up. Data are presented as median (Q1-Q3), count or per cent. Prior to surgery, 63% in the PEA-group [n = 98, age 64 (51-71) years, 37% female] used PH-specific treatment and 20, 69, and 10% were assessed as low, intermediate or high risk, respectively. After 1 year post-surgery, 34% had no PH-specific treatment or follow-up visit registered despite being alive at 5 years. Of patients with a 5 year visit (n = 23), 46% were at low and 54% at intermediate risk, while 91% used PH-specific treatment. In the non-PEA group [n = 213, age 72 (65-77) years, 56% female], 28% were assessed as low, 61% as intermediate and 11% as high risk. All patients at high risk versus 50% at low risk used PH-specific treatment. The 1 year mortality was 6%, while the risk was unchanged in 57% of the patients; 14% improved from intermediate to low risk, and 1% from high to low risk. At 5 years, 27% had a registered visit and 28% had died. Of patients with a 5 year visit (n = 58), 38% were at low, 59% at intermediate and 1% at high risk, and 86% used PH-specific treatment. CONCLUSIONS: Risk status assessed pre-surgery did not foresee long-term post-PEA risk and pre-surgery PH-specific treatment did not foresee long-term post-PEA treatment. Medically treated CTEPH patients tend to remain at the same risk over time, suggesting a need for improved treatment strategies in this group.

Levosimendan Versus Milrinone and Release of Myocardial Biomarkers After Pediatric Cardiac Surgery: Post Hoc Analysis of Clinical Trial Data.

OBJECTIVES: We compared the effect of two inodilators, levosimendan and milrinone, on the plasma levels of myocardial injury biomarkers, that is, high-sensitivity troponin T and heart-type fatty acid binding protein, and on N-terminal prohormone of brain natriuretic peptide as a biomarker of ventricular function. We hypothesized that levosimendan could attenuate the degree of myocardial injury when compared with milrinone. DESIGN: A post hoc, nonprespecified exploratory secondary analysis of the Milrinone versus Levosimendan-1 trial (ClinicalTrials.gov Identifier: NCT02232399). SETTING: Two pediatric tertiary university hospitals. PATIENTS: Infants 1-12 months old, diagnosed with ventricular septal defect, complete atrioventricular septal defect, or Tetralogy of Fallot undergoing corrective surgery with cardiopulmonary bypass. INTERVENTIONS: Seventy patients received a loading dose of either levosimendan or milrinone at the start of cardiopulmonary bypass followed by an infusion of the respective drug, which continued for 26 hours. MEASUREMENTS AND MAIN RESULTS: Plasma levels of the three cardiac biomarkers were measured prior to the initiation of cardiopulmonary bypass and 2, 6, and 24 hours after weaning from cardiopulmonary bypass. In both groups, the levels of high-sensitivity troponin T and heart-type fatty acid binding protein were highest at 2 hours post cardiopulmonary bypass, whereas the highest level of N-terminal prohormone of brain natriuretic peptide occurred at 24 hours post cardiopulmonary bypass. There was no significant difference in the biomarkers' plasma levels between the study groups over time. Neither was there a significant difference in the postoperative peak plasma levels of the cardiac biomarkers. CONCLUSIONS: In this post hoc analysis of the MiLe-1 trial, there was no demonstrable difference in the postoperative cardiac biomarker profile of myocardial injury and ventricular function when comparing infants managed in the perioperative period with levosimendan versus milrinone.

Preoperative heart failure is not associated with impaired coagulation in paediatric cardiac surgery.

OBJECTIVE: The objectives of the present study were to determine whether there was any association between the grade of heart failure, as expressed by preoperative levels of brain natriuretic peptide and Ross score, and the preoperative coagulation status in patients with non-restrictive ventricular shunts and determine whether there were any postoperative disturbances of the coagulation system in these patients, as measured by thromboelastometry and standard laboratory analyses of coagulation. DESIGN: Perioperative coagulation was analysed with laboratory-based coagulation tests and thromboelastometry before, 8 hours after, and 18 hours after cardiac surgery. In addition, brain natriuretic peptide was analysed before and 18 hours after surgery. PATIENTS: 40 children less than 12 months old with non-restrictive congenital ventricular or atrio-ventricular shunts scheduled for elective repair of their heart defects. RESULTS: All coagulation parameters measured were within normal ranges preoperatively. There was a significant correlation between brain natriuretic peptide and plasma fibrinogen concentration preoperatively. There was no statistically significant correlation between brain natriuretic peptide and INTEM-MCF, FIBTEM-MCF, plasma fibrinogen, activated partial thromboplastin time, prothrombin time, or platelet count at any other time point, either preoperatively or postoperatively. Postoperatively, fibrinogen plasma concentration and FIBTEM-MCF decreased significantly at 8 hours, followed by a large increase at 18 hours to higher levels than preoperatively. CONCLUSIONS: There was no evidence of children with non-restrictive shunts having coagulation abnormalities before cardiac surgery. Brain natriuretic peptide levels or Ross score did not correlate with coagulation parameters in any clinically significant way.

Clinical course and outcome after treatment with ventricular assist devices in paediatric patients: A single-centre experience.

BACKGROUND: Heart failure is a rare condition in the paediatric population, associated with high morbidity and mortality. When medical therapy is no longer sufficient, mechanical circulatory support such as a ventricular assist device can be used to bridge these children to transplant or recovery. Coagulation-related complications such as thrombi, embolism and bleeding events represent the greatest challenge in paediatric patients on mechanical support. We aimed to describe the outcomes and coagulation-related complications in this patient population at our institution. METHODS: A total of 20 patients with either Berlin Heart EXCOR® or HeartWare® implantation were reviewed in this retrospective study. Study endpoints were survival to heart transplant, weaning due to recovery or death. Thrombotic events were defined as thrombus formation in the device or in the patient, or cardioembolic strokes. Bleeding events were defined as events requiring interventional surgery or transfusion of red blood cells. RESULTS: The aetiology of heart failure included cardiomyopathy (n = 12), end-stage congenital heart disease (n = 6) and myocarditis (n = 2). Of the 20 patients, 12 were bridged to transplant, 7 recovered and could be weaned and 1 died. The median duration of mechanical support was 84 days (range: 20-524 days). At least one major or minor bleeding event occurred in 45% of the patients. Thrombotic events occurred 21 times in 10 patients. Four of the patients (20%) had no bleeding or thromboembolic event. CONCLUSION: In all, 95% of the patients were successfully bridged to transplant or recovery. Bleeding events and thrombotic events were common.

Durable circulatory support with a paracorporeal device as an option for pediatric and adult heart failure patients.

OBJECTIVES: Not all patients in need of durable mechanical circulatory support are suitable for a continuous-flow left ventricular assist device. We describe patient populations who were treated with the paracorporeal EXCOR, including children with small body sizes, adolescents with complex congenital heart diseases, and adults with biventricular failure. METHODS: Information on clinical data, echocardiography, invasive hemodynamic measurements, and surgical procedures were collected retrospectively. Differences between various groups were compared. RESULTS: Between 2008 and 2018, a total of 50 patients (21 children and 29 adults) received an EXCOR as bridge to heart transplantation or myocardial recovery. The majority of patients had heart failure compatible with Interagency Registry for Mechanically Assisted Circulatory Support profile 1. At year 5, the overall survival probability for children was 90%, and for adults 75% (P = .3). After we pooled data from children and adults, the survival probability between patients supported by a biventricular assist device was similar to those treated with a left ventricular assist device/ right ventricular assist device (94% vs 75%, respectively, P = .2). Patients with dilated cardiomyopathy had a trend toward better survival than those with other heart failure etiologies (92% vs 70%, P = .05) and a greater survival free from stroke (92% vs 64%, P = .01). Pump house exchange was performed in nine patients due to chamber thrombosis (n = 7) and partial membrane rupture (n = 2). There were 14 cases of stroke in eleven patients. CONCLUSIONS: Despite severe illness, patient survival on EXCOR was high, and the long-term overall survival probability following heart transplantation and recovery was advantageous. Treatment safety was satisfactory, although still hampered by thromboembolism, mechanical problems, and infections.

Levosimendan Versus Milrinone for Inotropic Support in Pediatric Cardiac Surgery: Results From a Randomized Trial.

OBJECTIVE: The present study aimed to determine the differential effects of intraoperative administration of milrinone versus levosimendan on myocardial function after pediatric cardiac surgery. Transthoracic echocardiography was used for myocardial function evaluation using biventricular longitudinal strain with 2-dimensional speckle tracking echocardiography in addition to conventional echocardiographic variables. DESIGN: A secondary analysis of a randomized, prospective, double-blinded clinical drug trial. SETTING: Two pediatric tertiary university hospitals. PARTICIPANTS: Infants between 1 and 12 months old diagnosed with ventricular septal defect, complete atrioventricular septal defect, or tetralogy of Fallot who were scheduled for corrective surgery with cardiopulmonary bypass. INTERVENTIONS: The patients were randomly assigned to receive an infusion of milrinone or levosimendan at the start of cardiopulmonary bypass and for 26 consecutive hours. MEASUREMENTS AND MAIN RESULTS: Biventricular longitudinal strain and conventional echocardiographic variables were measured preoperatively, on the first postoperative morning, and before hospital discharge. The association between perioperative parameters and postoperative myocardial function also was investigated. Images were analyzed for left ventricular (n = 67) and right ventricular (n = 44) function. The day after surgery, left ventricular longitudinal strain deteriorated in both the milrinone and levosimendan groups (33% and 39%, respectively). The difference was not significant. The corresponding deterioration in right ventricular longitudinal strain was 42% and 50% (nonsignificant difference). For both groups, biventricular longitudinal strain approached preoperative values at hospital discharge. Preoperative N-terminal pro-brain natriuretic peptide could predict the left ventricular strain on postoperative day 1 (p = 0.014). CONCLUSIONS: Levosimendan was comparable with milrinone for left and right ventricular inotropic support in pediatric cardiac surgery.

2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT.

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990-2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH.

The Effect of Levosimendan Versus Milrinone on the Occurrence Rate of Acute Kidney Injury Following Congenital Heart Surgery in Infants: A Randomized Clinical Trial.

OBJECTIVES: It has been shown that, in contrast to other inotropic agents, levosimendan improves glomerular filtration rate after adult cardiac surgery. The aim of this study was to investigate the efficacy of levosimendan, compared with milrinone, in preventing acute kidney dysfunction in infants after open-heart surgery with cardiopulmonary bypass. DESIGN: Two-center, double-blinded, prospective, randomized clinical trial. SETTING: The study was performed in two tertiary pediatric centers, one in Sweden (Gothenburg) and one in Finland (Helsinki). PATIENTS: Infants between 1 and 12 months old, diagnosed with Tetralogy of Fallot, complete atrioventricular septal defect or nonrestrictive ventricular septal defect, undergoing total corrective cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: Seventy-two infants were randomized to receive a perioperative infusion of levosimendan (0.1 µg/kg/min) or milrinone (0.4 µg/kg/min). The infusion was initiated at the start of cardiopulmonary bypass and continued for 26 hours. MEASUREMENTS AND MAIN RESULTS: The primary outcome variable was the absolute value of serum creatinine data on postoperative day 1. Secondary outcomes included the following: 1) acute kidney injury according to the serum creatinine criteria of the Kidney Diseases: Improving Global Outcomes; 2) acute kidney injury with serum creatinine corrected for fluid balance; 3) plasma neutrophil gelatinase-associated lipocalin; 4) cystatin C; 5) urea; 6) lactate; 7) hemodynamic variables; 8) use of diuretics in the PICU; 9) need of dialysis; 10) length of ventilator therapy; and 11) length of PICU stays. There was no significant difference in postoperative serum creatinine between the treatment groups over time (p = 0.65). The occurrence rate of acute kidney injury within 48 hours was 46.9% in the levosimendan group and 39.5% in the milrinone group (p = 0.70). There were no significant differences in other secondary outcome variables between the groups. CONCLUSIONS: Levosimendan compared with milrinone did not reduce the occurrence rate of acute kidney injury in infants after total corrective heart surgery for atrioventricular septal defect, ventricular septal defect, or Tetralogy of Fallot.

Post-transplant lymphoproliferative disease is associated with early sternotomy and left ventricular hypoplasia during infancy: a population-based retrospective review.

BACKGROUND: Heart transplantation has been an option for children in Sweden since 1989. As our unit faced an increased rate of post-transplant lymphoproliferative disorder, the objective of the study was to identify possible risk factors. METHODS: This is a retrospective study of all children aged 0-18 years who underwent heart transplantation in Gothenburg from 1989 to 2014. RESULTS: A total of 71 children underwent heart transplantation. The overall incidence of post-transplant lymphoproliferative disorder was 14% (10/71); however, 17% (6/36) of those undergoing transplantation after 2007 developed lymphoma, compared with only 10% (4/35) of transplantation cases before 2007 (p=0.85). The mean age at transplantation was 9 years (0-17). The mean post-transplant follow-up time was 5.5 years (0.5-21.9) in the group that developed post-transplant lymphoproliferative disorder, compared with 10.2 years (0.02-25.2) in those who did not. In our study group, risk factors for post-transplant lymphoproliferative disorder were surgically palliated CHD (p=0.0005), sternotomy during infancy (p⩽0.0001), hypoplastic left ventricle (p=0.0001), number of surgical events (p=0.0022), mismatch concerning Epstein-Barr virus infection - that is, a positive donor-negative recipient (p⩽0.0001) - and immunosuppressive treatment with tacrolimus compared with ciclosporine (p=0.028). Discussion This study has three major findings. First, post-transplant lymphoproliferative disorder only developed in subjects born with CHD. Second, the vast majority (9/10) of the subjects developing the disorder had undergone sternotomy as infants. Third, the number of surgical events correlated with a higher risk for developing post-transplant lymphoproliferative disorder.

Earlier detection of coagulopathy with thromboelastometry during pediatric cardiac surgery: a prospective observational study.

OBJECTIVE: Earlier detection of coagulopathy in pediatric cardiac surgery patients. AIM: To determine whether thromboelastometry (TEM) analysis before weaning from cardiopulmonary bypass (CPB) and hemoconcentration is predictive of post-CPB results and whether analysis of clot firmness already after 10 min yields reliable results. BACKGROUND: Cardiac surgery with CPB induces a coagulopathy that may contribute to postoperative complications. Earlier detection increases the possibility of initiating countermeasures. METHODS/MATERIAL: Fifty-six pediatric cardiac surgery patients were included in a prospective observational study. HEPTEM and FIBTEM clotting time (CT), clot formation time (CFT), and clot firmness after 10 min (A10) and at maximum (MCF) were analyzed during CPB and after CPB and ultrafiltration with modified rotational thromboelastometry (ROTEM). The analyses were compared, and correlations and differences were calculated. RESULTS: Hemoconcentration with modified ultrafiltration increased hematocrit from 28 ± 3 to 37 ± 4% (P < 0.001). Correlation coefficients of the TEM variables during and after CPB ranged from 0.61 to 0.82 (all P < 0.001). HEPTEM-CT and HEPTEM-MCF differed significantly but the differences were marginal. Both HEPTEM and FIBTEM A10 measurements during CPB were significantly less than MCF (P < 0.001 for both), but the correlations were highly significant (HEPTEM: r = 0.95, P < 0.001; FIBTEM: r = 0.96, P < 0.001), and the differences were predictable, with narrow confidence intervals (HEPTEM: -8.2 mm (-8.9 to -7.5); FIBTEM: -0.5 mm (-0.7 to -0.3). CONCLUSION: The results suggest that intraoperative TEM analyses can be accelerated by analyzing HEPTEM/FIBTEM on CPB before hemoconcentration and by analyzing clot firmness already after 10 min.

First two decades of paediatric heart transplantation in Sweden - outcome of listing and post-transplant results.

AIMS: To evaluate outcome in the first generation of children with end-stage heart disease to whom heart transplantation was available. METHODS: Retrospective review of all 135 Swedish children <18 years old listed for heart transplantation 1989-2009, followed to December 31, 2009, including 74 (55%) with cardiomyopathy and 61 (45%) with congenital heart disease; 34 (25%) were infants (<1 year). Cumulative risk of requiring heart transplantation was 1:17,300 (11 patients who improved were omitted from outcome analysis). RESULTS: Waiting-list mortality was 31% (44% in infants). Median waiting time in 82 transplanted patients was 57 days (0-585 days). Post-transplant follow-up time was median 5.9 years (0.03-20.1 years), and actuarial survival was 92% at 1 year, 82% at 5 years, 76% at 10 years and 58% at 15 years. Survival after listing was 64% at 1 year, 58% at 5 years, 52% at 10 years and 40% at 15 years. Post-transplant complications included rejections (34%), malignancies (12%), renal failure (8%), coronary artery vasculopathy (6%) and re-transplantation (5%). Among 64 survivors, 84% were free of complications affecting prognosis. CONCLUSION: High waiting-list mortality and post-transplant attrition precluded 60% of this pioneer population from reaching adulthood. Functional status in survivors is generally good.

Intraoperative thromboelastometry is associated with reduced transfusion prevalence in pediatric cardiac surgery.

BACKGROUND: The majority of pediatric cardiac surgery patients receive blood transfusions. We hypothesized that the routine use of intraoperative thromboelastometry to guide transfusion decisions would reduce the overall proportion of patients receiving transfusions in pediatric cardiac surgery. METHODS: One hundred pediatric cardiac surgery patients were included in the study. Fifty patients (study group) were prospectively included and compared with 50 procedure- and age-matched control patients (control group). In the study group, thromboelastometry, performed during cardiopulmonary bypass, guided intraoperative transfusions. Intraoperative and postoperative transfusions of packed red blood cells, fresh frozen plasma, platelets, and fibrinogen concentrates, and postoperative blood loss and hemoglobin levels were compared between the 2 groups. RESULTS: The proportion of patients receiving any intraoperative or postoperative transfusion of packed red blood cells, fresh frozen plasma, platelets, or fibrinogen concentrates was significantly lower in the study group than in the control group (32 of 50 [64%] vs 46 of 50 [92%], respectively; P < 0.001). Significantly fewer patients in the study group received transfusions of packed red blood cells (58% vs 78%, P = 0.032) and plasma (14% vs 78%, P < 0.001), whereas more patients in the study group received transfusions of platelets (38% vs 12%, P = 0.002) and fibrinogen concentrates (16% vs 2%, P = 0.015). Neither postoperative blood loss nor postoperative hemoglobin levels differed significantly between the study group and the control group. CONCLUSIONS: The results suggest that routine use of intraoperative thromboelastometry in pediatric cardiac surgery to guide transfusions is associated with a reduced proportion of patients receiving transfusions and an altered transfusion pattern.

Increased natriuretic peptide type B level after the second palliative step in children with univentricular hearts with right ventricular morphology but not left ventricular morphology.

We analyzed the natriuretic peptide type B (BNP) and A (ANP) levels in the plasma of 38 children with stable functionally univentricular heart defects, 10 of whom had undergone the first palliative step (Shunt), 13 of whom had undergone the second palliative step (Glenn), and 15 of whom had completed total cavopulmonary connection (TCPC). The levels of BNP and ANP were significantly higher after the first palliative step, 31.6 ng/l (8.3-122) (median and range) and 101.2 ng/l (17.1-203), respectively, than after the Glenn procedure, 6.7 ng/l (0.0-16.0) and 23.6 ng/l (15.7-54.4), respectively, as well as after completed TCPC, 9.0 ng/l (0.0-39.1) and 20.9 ng/l (11.2-28.3), respectively, and, in a control group of children without heart defects, 5.8 ng/l (0.0-38.7) and 32.9 ng/l (11.7-212.1), respectively (p = 0.0003 and p = 0.0003, respectively). After the first palliative step, the BNP and ANP levels were the same in children with right and left ventricular morphology (p = 0.67 and p = 0.52, respectively). After unloading the ventricle (Glenn and TCPC together), BNP levels were higher in children with right ventricular morphology compared with those with left ventricular morphology and controls (p = 0.02). Children with functionally univentricular hearts in stable condition have increased BNP and ANP levels after the first palliative procedure. After the second and third palliative steps, the BNP and ANP levels were low and similar to those of children without heart defects. However, BNP levels in children with a systemic ventricle of right ventricular morphology were higher than those in children with left ventricular morphology.

Brain natriuretic peptide assessed at long-term follow-up before and after maximal exercise in surgically palliated patients with functionally univentricular hearts.

We evaluated the concentrations of brain natriuretic peptide in the plasma as a marker of systolic ventricular function before and after maximal exercise in 15 surgically palliated patients with functionally univentricular hearts, with apparently good ventricular function. Of the patients, 6 with median age of 14.6 years, and a range from 12.5 to 17.9 years, had been palliated by construction of a total cavopulmonary connection, while the other 9 patients, with a median age of 32.1 years, and a range from 15.6 to 54.2 years, had undergone the classical Fontan procedure. We used 8 healthy individuals, with a median age of 13.9 years, and a range from 12.8 to 14.2 years, as a control group for the measurements of brain natriuretic peptide. The values of the peptide were significantly higher in those with the classical Fontan procedure, both before, when the median value was 131.8 nanogram per litre, with a range from 0.5 to 296.4, and after maximal exercise, when the median value was 108.1, with a range from 0.1 to 235.9. The comparable values in those with a total cavopulmonary connection were a median of 12.8, and a range from 0.5 to 39.1 before, and a median of 9.7, with a range from 2.7 to 26.2 after maximal exercise. The median value for the control group was 13.1, with a range from 2.6 to 38.7 before exercise (p = 0.016), and a median of 24.1, with a range from 5.8 to 66.7 after maximal exercise (p = 0.03), respectively. In the control subjects, the level of the peptide increased by a median of 9.7 nanograms per litre, with a range from 1.2 to 28.0 after maximal exercise (p = 0.008). The level was unchanged after maximal exercise in those with classical Fontan procedures and total cavopulmonary connections, with a difference between levels before and after exercise of a median of 5.9 nanogram per litre, and a range from -23.7 to 31.0 (p = 0.96), and a median of -1.0 nanogram per litre, with a range from -12.0 to 3.9 (p > 0.99), respectively. We conclude that maximal exercise did not increase the level of brain natriuretic peptide level in those patients with the classical Fontan procedure, nor those with a total cavopulmonary connection, findings which may indicate that systolic ventricular dysfunction is not the major cause of the decreased working capacity observed in patients with well functioning palliated functionally univentricular hearts.

Increased plasma levels of natriuretic peptide type B and A in children with congenital heart defects with left compared with right ventricular volume overload or pressure overload.

AIM: Natriuretic peptide levels B (BNP) and A (ANP) have been described in children with congenital heart defects (CHD) with pressure and volume overload. However, the impact of ventricular morphology per se on natriuretic peptide levels has not been reported. The aim of the present study was to evaluate plasma BNP and ANP in children with CHD with left or right ventricular volume or pressure overload. METHODS AND RESULTS: Plasma BNP and ANP were analysed in 61 children, median age 3.1 (0.3-16.2) years. Haemodynamic load was evaluated by echo-Doppler and/or catheterization measurements and classified as: pressure overload of the right (RV pressure) or left (LV pressure) ventricle, or volume overload of the right (RV volume) or left (LV volume) ventricle, of a sufficient degree to indicate surgery/catheter intervention. Twenty-three children, with a median age of 1.1 (0.1-8.3) years, without heart disease, served as controls for the natriuretic peptide measurements. Children in the LV volume group had significantly higher BNP and ANP values, 55.4 ng l-1 (10.7-352) and 164 (31.8-346), than children in the RV volume, 15.6 (0.0-105.1) and 57.2 (11.3-234.1), LV pressure, 6.8 (0.7-170) and 40.8 (12.6-210), and RV pressure, 18.0 (5.0-29.1) and 69.3 (8.7-182), groups respectively (P<0.0001). The values in the LV pressure group were close to the values in the Control group, 4.7 (0.0-17.7) and 32.9 (11.7-212.1), respectively (P=0.051 and P=0.378, respectively). CONCLUSIONS: Plasma concentrations of BNP and ANP were higher in children with CHD with left ventricular volume overload compared with right ventricular volume overload or pressure overload.

Increased levels of brain and atrial natriuretic peptides after the first palliative operation, but not after a bidirectional glenn anastomosis, in children with functionally univentricular hearts.

We evaluated the concentrations of the brain and atrial natriuretic peptides in the plasma as markers of ventricular function and volume load in children with functionally univentricular hearts. We studied 7 children aged from 0.5 to 0.7 years with functionally univentricular hearts who had undergone a first palliative operation, and 10 children aged from 1.8 to 3.7 years who had undergone a bidirectional Glenn anastomosis at ages ranging from 0.4 to 1.0 year. As a control group, we studied 14 children without heart defects aged from 0.1 to 4.5 years. Levels of the brain natriuretic peptide were measured at 8.3 to 122 ng/l, with a mean of 52.8 ng/l, after the first palliative operation, compared to 0 to 16 ng/l, with a mean of 7.3 ng/l, after a bidirectional Glenn anastomosis, and 0 to 13.8 ng/l, with a mean of 5.9 ng/l, in the children serving as controls. Corresponding values for atrial natriuretic peptide were 17 to 203 ng/l, with a mean of 103 ng/l, after the first palliative operation, compared to 16 to 54 ng/l, with a mean of 29 ng/l, after the bidirectional Glenn anastomosis, and 12 to 52 ng/l, with a mean of 32 ng/l in the controls. Echocardiography showed that all the children with functionally univentricular hearts had normal ventricular function. Blood presssure, pulmonary arterial pressure, and arterial saturations of oxygen did not differ between the groups. We conclude, that in children with functionally univentricular hearts, the volume overload imposed on the heart after the first palliative operation is associated with increased production of brain and atrial natriuretic peptides, while after ventricular unloading, levels of the natriuretic peptides return to control values.