Pneumonia, wheezing and asthma were more common in children after thymectomy due to open-heart surgery.

AIM: This nationwide study evaluated the clinical impact that an early thymectomy, during congenital heart defect (CHD) surgery, had on the health of children and adolescents. METHODS: The subjects were patients aged 1-15 years who had undergone CHD surgery at the University Children's Hospital, Helsinki, where all CHD surgery in Finland is carried out, from 2006 to 2018. The parents or the cases and population-based controls, matched for sex, age and hospital district, completed electronic questionnaires. We excluded those with low birth weights or a known immunodeficiency. Adjusted odds ratios (aOR) and 95% confidence intervals (CI) were calculated for prespecified outcomes. RESULTS: We received responses relating to 260/450 (58%) cases and 1403/4500 (31%) controls and excluded 73 cases with persistent cardiac or respiratory complaints after surgery. The CHD group reported more recurrent hospitalisations due to infections (aOR 6.3, 95% CI 3.0-13) than the controls and more pneumonia episodes (aOR 3.5, 95% CI 2.1-5.6), asthma (aOR 2.5, 95% CI 1.5-4.1) and wheezing (aOR 2.1, 95% CI 1.5-2.9). CONCLUSION: Hospitalisation due to infections, pneumonia, wheezing and asthma was more common in children after a thymectomy due to open-heart surgery than population-based controls, underlining the importance of immunological follow-ups.

Risk factors for mortality in patients with hypoplastic left heart syndrome after the Norwood procedure.

OBJECTIVES: Several studies have reported mortality risk factors associated with hypoplastic left heart syndrome (HLHS). However, these data are ambiguous and mainly focused on the independent effects of these factors. We examined both the independent and the cumulative effects of preoperative risk factors for poor outcome in patients undergoing the Norwood procedure. Moreover, we studied the risk factors associated with prolonged initial hospital stays in these patients. METHODS: We performed a retrospective national 18-year observational study of preoperative risk factors for 1 year, as well as total follow-up mortality or need for transplant in patients with HLHS (N = 99) born in Finland between 1 January 2004 and 31 December 2021. RESULTS: Overall, one-year survival was 85.6%. In a multivariable analysis, having a major extracardiac anomaly and being small for gestational age were significant predictors of one-year mortality or the need for a transplant. Aortic atresia was a predictor of total follow-up mortality. An analysis of the cumulative effect indicated that the presence of 2 risk factors was associated with higher mortality. CONCLUSIONS: HLHS remains the defect with the highest procedural risks for mortality in paediatric cardiac surgery. From a prognostic point of view, recognition of independent preoperative risk factors as well as the cumulative effect of risk factors for mortality is essential.The results of this study were presented orally at the 55th Annual Meeting of the Association for European Paediatric and Congenital Cardiology, Geneva, Switzerland, 28 May 2022.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Combining Congenital Heart Surgical and Interventional Cardiology Outcome Data in a Single Database: The Development of a Patient-Centered Collaboration of the European Congenital Heart Surgeons Association (ECHSA) and the Association for European Paediatric and Congenital Cardiology (AEPC).

The European Congenital Heart Surgeons Association (ECHSA) Congenital Database (CD) is the second largest clinical pediatric and congenital cardiac surgical database in the world and the largest in Europe, where various smaller national or regional databases exist. Despite the dramatic increase in interventional cardiology procedures over recent years, only scattered national or regional databases of such procedures exist in Europe. Most importantly, no congenital cardiac database exists in the world that seamlessly combines both surgical and interventional cardiology data on an international level; therefore, the outcomes of surgical and interventional procedures performed on the same or similar patients cannot easily be tracked, assessed, and analyzed. In order to fill this important gap in our capability to gather and analyze information on our common patients, ECHSA and The Association for European Paediatric and Congenital Cardiology (AEPC) have embarked on a collaborative effort to expand the ECHSA-CD with a new module designed to capture data about interventional cardiology procedures. The purpose of this manuscript is to describe the concept, the structure, and the function of the new AEPC Interventional Cardiology Part of the ECHSA-CD, as well as the potentially valuable synergies provided by the shared interventional and surgical analyses of outcomes of patients. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow centers to have access to robust surgical and transcatheter outcome data from their own center, as well as robust national and international aggregate outcome data for benchmarking. Each contributing center or department will have access to their own data, as well as aggregate data from the AEPC Interventional Cardiology Part of the ECHSA-CD. The new AEPC Interventional Cardiology Part of the ECHSA-CD will allow cardiology centers to have access to aggregate cardiology data, just as surgical centers already have access to aggregate surgical data. Comparison of surgical and catheter interventional outcomes could potentially strengthen decision processes. A study of the wealth of information collected in the database could potentially also contribute toward improved early and late survival, as well as enhanced quality of life of patients with pediatric and/or congenital heart disease treated with surgery and interventional cardiac catheterization across Europe and the world.

Analysis of thymic generation of shared T-cell receptor α repertoire associated with recognition of tumor antigens shows no preference for neoantigens over wild-type antigens.

BACKGROUND: The number of mutations in cancer cells is an important predictor of a positive response to cancer immunotherapy. It has been suggested that the neoantigens produced by these mutations are more immunogenic than nonmutated tumor antigens, which are likely to be protected by immunological tolerance. However, the mechanisms of tolerance as regards tumor antigens are incompletely understood. METHODS: Here, we have analyzed the impact of thymic negative selection on shared T-cell receptor (TCR) repertoire associated with the recognition of either mutated or nonmutated tumor antigens by comparing previously known TCR-antigen-pairs to TCR repertoires of 21 immunologically healthy individuals. RESULTS: Our results show that TCRα chains associated with either type of tumor antigens are readily generated in the thymus, at a frequency similar to TCRα chains associated with nonself. In the peripheral repertoire, the relative clone size of nonself-associated chains is higher than that of the tumor antigens, but importantly, there is no difference between TCRα chains associated with mutated or nonmutated tumor antigens. CONCLUSION: This suggests that the tolerance mechanisms protecting nonmutated tumor antigens are non-deletional and therefore potentially reversible. As unmutated antigens are, unlike mutations, shared by a large number of patients, they may offer advantages in designing immunological approaches to cancer treatment.

Heart Transplantation for Early-Onset Anthracycline-Induced Cardiomyopathy Within 5 Months of Chemotherapy Completion.

A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after completion of chemotherapy for osteosarcoma. Five months after completion of chemotherapy, at the age of 10 years, heart transplantation was performed. At 29 months since transplantation, the patient remains free of rejection and recurrence of osteosarcoma. (Level of Difficulty: Intermediate.).

Characterization of human T cell receptor repertoire data in eight thymus samples and four related blood samples.

T cell receptor (TCR) is a heterodimer consisting of TCRα and TCRß chains that are generated by somatic recombination of multiple gene segments. Nascent TCR repertoire undergoes thymic selections where non-functional and potentially autoreactive receptors are removed. During the last years, the development of high-throughput sequencing technology has allowed a large scale assessment of TCR repertoire and multiple analysis tools are now also available. In our recent manuscript, Human thymic T cell repertoire is imprinted with strong convergence to shared sequences[1], we show highly overlapping thymic TCR repertoires in unrelated individuals. In the current Data in Brief article, we provide a more detailed characterization of the basic features of these thymic and related peripheral blood TCR repertoires. The thymus samples were collected from eight infants undergoing corrective cardiac surgery, two of whom were monozygous twins [2]. In parallel with the surgery, a small aliquot of peripheral blood was drawn from four of the donors. Genomic DNA was extracted from mechanically released thymocytes and circulating leukocytes. The sequencing of TCRα and TCRß repertoires was performed at ImmunoSEQ platform (Adaptive Biotechnologies). The obtained repertoire data were analysed applying relevant features from immunoSEQ® 3.0 Analyzer (Adaptive Biotechnologies) and a freely available VDJTools software package for programming language R [3]. The current data analysis displays the basic features of the sequenced repertoires including observed TCR diversity, various descriptive TCR diversity measures, and V and J gene usage. In addition, multiple methods to calculate repertoire overlap between two individuals are applied. The raw sequence data provide a large database of reference TCRs in healthy individuals at an early developmental stage. The data can be exploited to improve existing computational models on TCR repertoire behaviour as well as in the generation of new models.

Cryopreserved Venous Allografts in Supra-inguinal Reconstructions: A Single Centre Experience.

OBJECTIVE: This study introduces a novel technique for supra-inguinal arterial reconstructions with cryopreserved femoral vein and caval allografts with a low re-infection rate and an acceptable graft re-intervention rate on early mid term analysis. METHODS: Patients treated from February 2012 to March 2018 with cryopreserved venous allograft reconstructions owing to infection in the supra-inguinal area were reviewed retrospectively. The primary end points were re-infection and the treatment related mortality rate. Secondary end points were 30 and 90 day and overall mortality and graft re-intervention rate. RESULTS: Of the 23 patients treated with cryopreserved venous allografts for infection in aorto-iliac area, 21 (91%) patients underwent reconstruction with cryopreserved femoral veins and two (9%) with vena cava. Indications for treatment were aortic graft infections (n = 12 [52%]), mycotic aneurysms (n = 5 [22%]), femorofemoral prosthetic infections (n = 3 [13%]), anastomotic pseudo-aneurysms (n = 2 [9%]), and aortic thrombosis with intestinal spillage (n = 1 [4%]). In hospital and 90 day mortality were 9% (n = 2); overall treatment related mortality during the median follow up of 15 months was 13% (n = 3). During the follow up, two allografts were re-operated on owing to anastomotic dilatation and one because of re-infection, resulting in a re-intervention rate of 13% (n = 3). None of the grafts was lost and there were no amputations. At the end of follow up 17 patients (74%) were alive. Kaplan-Meier estimation for survival was 76% (95% confidence interval [CI] 57%-95%) at one year and 70% (95% CI 49%-91%) at two years. CONCLUSION: Cryopreserved venous allografts appear to be an infection resistant and reasonably safe reconstruction material in the aorto-iliac axis based upon the early mid term analysis from a single centre experience. Further research is needed to compare their performance with other biological reconstruction material.

Complete Atrioventricular Septal Defect: Evolution of Results in a Single Center During 50 Years.

BACKGROUND: Operative mortality after complete atrioventricular septal defect (cAVSD) repair has improved vastly. Less improvement has been demonstrated regarding late mortality and reoperation rates, however. There is evident lack of comprehensive population-based studies analyzing the history and progress of the ever-changing operative results. METHODS: This is a 5-million population-based retrospective study of consecutive 388 cAVSD patients operated in Finland between 1962 and 2014. Data were collected using Children's Cardiac Surgical Registry of Children's Hospital at the Helsinki University Hospital, Finland. Mortality data and reoperation rates were analyzed on a decade-by-decade basis. RESULTS: During the early era, overall mortality was 17.4%, operative mortality constituting 10.9%. The operative results have improved significantly over the decades, and eventually, the last decade showed no mortality. A total of 23 late deaths occurred; of these, 20 were directly heart-related. Half of the late mortality occurred during the first postoperative year. The only significant risk factor for overall mortality was an earlier decade of operation (p < 0.001). Reoperation rates have not decreased but slightly increased over decades (p = 0.621), and reoperations have been performed mainly during the first year after the primary operation. Actuarial freedom from left side atrioventricular valve reoperation at 15 years was 90.9%. CONCLUSIONS: There has been an outstanding improvement in surgical results through the years even though the general operative approach has remained the same. Rates of reoperation have not been declining, but the reoperations are dated to early childhood years. The improvement in results has been ongoing.

Congenital aortic stenosis: treatment outcomes in a nationwide survey.

OBJECTIVE: To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. DESIGN: Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000-2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR. RESULTS: Sixty-one patients underwent either BAV (n = 54) or surgical valvuloplasty (n = 7) for valvar AS at a median age of 29 days (range 6 hours to 16.9 years). The proportion of patients not requiring reintervention at 1, 5, and 10 years was 61%, 50%, and 29% in neonates and 83%, 73%, and 44% in older patients, respectively (p = .02); without difference between treatment groups. Larger proportion of patients remained free from valve surgery after optimal BAV result than after adequate or inadequate result (p = .01). The reason for the first reintervention was AS in 50%, AR in 36%, and combined aortic valve disease in 16% of cases. Early mortality (before hospital discharge) was 4.9%, and associated with critical AS in neonates. There was no late mortality during the follow-up. CONCLUSIONS: Although majority of congenital AS patients require more than one intervention during childhood, an optimal BAV result improves long-term outcome by increasing the proportion of patients remaining free from valve surgery. High long-term freedom from reintervention is attainable also in the neonatal population.

Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

The effect of continuous wound infusion of ropivacaine on postoperative pain after median sternotomy and mediastinal drain in children.

BACKGROUND: Postoperative pain after median sternotomy is usually treated with i.v. opioids. We hypothesized that continuous wound infusion of ropivacaine decreases postoperative morphine consumption and improves analgesia in children who undergo cardiac surgery. METHODS: This randomized, double-blind study comprised 49 children aged 1-9 years who underwent atrial septal defect (ASD) closure. Patients received continuous local anesthetic wound infiltration either with 0.2% ropivacaine, 0.3-0.4 mg·kg(-1) ·h(-1) (Group R) or with saline (Group C). Rescue morphine consumption, Objective Pain Scale (OPS), time to mobilization, time to enteral food intake, and time to discharge were recorded. RESULTS: There were no statistically significant differences in morphine consumption at 24, 48, and 72 h postsurgery between R and C groups. There was a weak evidence for a difference in the time to the first morphine administration after tracheal extubation to be longer for Group R than Group C (186.2 vs 81.0 min; 95% CI (-236.5, 26.2), P = 0.114). The incidence of nausea and vomiting were comparable between the groups. No signs or symptoms of local anesthetic toxicity were registered. CONCLUSIONS: Contrary to our hypothesis, continuous ropivacaine wound infusion did not reduce morphine consumption, pain score values, or nausea and vomiting in children who underwent ASD closure with median sternotomy and mediastinal drain.

Surgical options after Fontan failure.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0-23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). CONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

Chronic Hypoxemia in Children With Congenital Heart Defect Impairs Airway Epithelial Sodium Transport.

OBJECTIVE: Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-term lung edema accumulation. DESIGN: Prospective, observational study. SETTING: Tertiary care medical center responsible for nationwide pediatric cardiac surgery. PATIENTS: Ninety-nine children with congenital heart defect or acquired heart disease (age range, 6 d to 14.8 yr) were divided into three groups based on their level of preoperative systemic hypoxemia: 1) normoxemic patients (SpO2% ≥ 95%; n = 44), 2) patients with cyanotic congenital heart defect and moderate hypoxemia (SpO2 86-94%; n = 16), and 3) patients with cyanotic congenital heart defect and profound systemic hypoxemia (SpO2 ≤ 85%; n = 39). MEASUREMENTS AND MAIN RESULTS: Nasal transepithelial potential difference served as a surrogate measure for epithelial Na transport of the respiratory tract. Profoundly hypoxemic patients had 29% lower basal nasal transepithelial potential difference (p = 0.02) and 55% lower amiloride-sensitive nasal transepithelial potential difference (p = 0.0003) than normoxemic patients. In profoundly hypoxemic patients, nasal epithelial messenger RNA expressions of two airway Na transporters (amiloride-sensitive epithelial Na channel and ß1- Na-K-ATPase) were not attenuated, but instead α1-Na-K-ATPase messenger RNA levels were higher (p = 0.03) than in the normoxemic patients, indicating that posttranscriptional factors may impair airway Na transport. The chest radiograph lung edema score increased after congenital cardiac surgery in profoundly hypoxemic patients (p = 0.0004) but not in patients with normoxemia or moderate hypoxemia. CONCLUSIONS: The impaired airway epithelial amiloride-sensitive Na transport activity in profoundly hypoxemic children with cyanotic congenital heart defect may hinder defense against lung edema after cardiac surgery.

The Effect of Methylprednisolone on Plasma Concentrations of Neutrophil Gelatinase-Associated Lipocalin in Pediatric Heart Surgery.

OBJECTIVES: Plasma neutrophil gelatinase-associated lipocalin is a kidney injury marker used in pediatric heart surgery. Neutrophil gelatinase-associated lipocalin is also a constituent of specific granules of neutrophils. Corticosteroids are widely used in pediatric heart surgery. Methylprednisolone inhibits degranulation of neutrophil-specific granules. Use of corticosteroids has not been taken into account in studies of neutrophil gelatinase-associated lipocalin in pediatric heart surgery. We studied the influence of systemically administered methylprednisolone on plasma neutrophil gelatinase-associated lipocalin concentrations in pediatric heart surgery. DESIGN: Two separate double-blinded randomized trials. SETTING: PICU at a university-affiliated hospital. PATIENTS: Forty neonates undergoing open-heart surgery and 45 children undergoing ventricular and atrioventricular septal defect correction. INTERVENTIONS: First trial (neonate trial), 40 neonates undergoing open-heart surgery received either 30 mg/kg IV methylprednisolone (n = 20) or placebo (n = 20). Second trial (ventricular septal defect trial), 45 children undergoing ventricular or atrioventricular septal defect correction received one of the following: 30 mg/kg of methylprednisolone IV after anesthesia induction (n = 15), 30 mg/kg methylprednisolone in the cardiopulmonary bypass prime solution (n = 15), or placebo (n = 15). MEASUREMENTS AND MAIN RESULTS: Plasma neutrophil gelatinase-associated lipocalin and creatinine were measured in both series. Lactoferrin levels were measured as a marker of neutrophil-specific granules in the ventricular septal defect trial only. No differences in creatinine levels occurred between the groups of either trial. Preoperative, neutrophil gelatinase-associated lipocalin did not differ between the study groups of either trial. Preoperatively administered methylprednisolone in the neonate trial reduced neutrophil gelatinase-associated lipocalin by 41% at 6 hours postoperatively (p = 0.002). Preoperatively administered methylprednisolone in the ventricular septal defect trial reduced neutrophil gelatinase-associated lipocalin by 47% (p = 0.010) and lactoferrin by 52% (p = 0.013) 6 hours postoperatively. Lactoferrin levels in the ventricular septal defect trial correlated with neutrophil gelatinase-associated lipocalin (R = 0.492; p = 0.001) preoperatively and after weaning from cardiopulmonary bypass (R = 0.471; p = 0.001). CONCLUSIONS: Preoperatively administered methylprednisolone profoundly decreases plasma neutrophil gelatinase-associated lipocalin levels. Neutrophil gelatinase-associated lipocalin seems to originate to a significant extent from activated neutrophils. Preoperative methylprednisolone is a confounding factor when interpreting plasma neutrophil gelatinase-associated lipocalin levels as a kidney injury marker in pediatric heart surgery.

Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970-2007.

OBJECTIVES: Treatment of pulmonary atresia with ventricular septal defect (PA + VSD) has evolved during recent decades, but it still remains challenging. This study evaluated 41-year experience of outcome, survival and treatment of PA + VSD patients. METHODS: Patient records and angiograms of 109 patients with PA + VSD born in Finland between 1970 and 2007, and treated at the Children's Hospital, Helsinki University Central Hospital, were retrospectively analysed in this nationwide study. RESULTS: Of the 109 patients, 66 (61%) had simple PA + VSD without major aortopulmonary collateral arteries (MAPCAs). Although we observed no difference in overall survival between those with or without MAPCAs, the patients without MAPCAs had better probability to achieve repair (64 vs 28%, P < 0.0003). Only 3 patients were treated by compassionate care. Overall survival was affected by the size of true central pulmonary arteries on the first angiogram (P = 0.001) and whether repair was achieved (P < 0.0001). After successful repair, the survival rate was 93% at 1 year, 91% from the second year, and functional capacity as assessed by New York Heart Association (NYHA) I-II remained in 85% of patients alive at the end of follow-up. Palliated patients at 1, 5, 10 and 20 years of age had Kaplan-Meier estimated survival rates of 55, 42, 34 and 20%, respectively. Patients who underwent repair attempts but were left palliated with right ventricle (RV)-pulmonary artery connection and septal fenestration had better survival than the rest of the palliated patients (P = 0.001). Further, the McGoon index improved after implementation of a systemic-pulmonary artery shunt in the overall PA + VSD population (P < 0.0001). CONCLUSIONS: These findings show that achievement of repair and initial size of true central pulmonary arteries affect survival of patients with PA + VSD. Although the overall survival of patients with MAPCAs showed no difference compared with simple PA + VSD patients, they had a higher risk of remaining palliated. However, palliative surgery may have a role in treatment of PA + VSD because the size of pulmonary arteries increased after placement of systemic-pulmonary artery shunt. In addition, subtotal repair by a RV-pulmonary artery connection and septal fenestration improved survival over extracardiac palliation.

Long-term results of the Ross procedure in a population-based follow-up.

OBJECTIVES: The purpose of this study was to evaluate the long-term outcomes of the Ross procedure in a nationwide follow-up. METHODS: This retrospective study involved all children treated with the Ross procedure in Finland between 1994 and 2009. The clinical records were reviewed for demographic and anatomical characteristics, Ross operation data, surgical history and status at the latest follow-up. The median follow-up time was 11.5 (range 2.4-19.2) years. RESULTS: Fifty-one patients underwent either the Ross (n = 37) or the Ross-Konno (n = 14) procedure at a median age of 4.8 (range 0.02-16.3) years, including 13 infants (<1 year of age). The indication for the Ross procedure was aortic valve stenosis, regurgitation or both, which was observed in 29, 24 and 47% of patients, respectively. The early mortality (before hospital discharge) rate was 10% (31% in infants) and the late mortality rate 6% (15% in infants). Higher mortality was discovered in patients treated with the Ross-Konno procedure (P = 0.001). The most common cause for reintervention was pulmonary homograft stenosis. The rate of freedom from right ventricular outflow tract reintervention was 98% at 5 years, 83% at 10 years and 59% at 15 years. The rate of freedom from autograft reintervention was 98% at 5 and 10 years, and 81% at 15 years. At the latest follow-up visit, mild-to-moderate aortic root dilatation was reported in 52% of patients, and 4 patients had undergone autograft-related reinterventions. Trivial autograft valve regurgitation was commonly seen, but only 1 patient developed severe autograft regurgitation requiring mechanical valve replacement 15.9 years after the Ross operation. CONCLUSIONS: The most common reason for reintervention after the Ross procedure in children is homograft stenosis. Aortic root dilatation and autograft valve regurgitation are relatively common but rarely lead to reinterventions before adulthood. Intraoperative complications and complex cardiac anatomy are associated with high mortality in infants undergoing the Ross-Konno procedure. In our centre, the Ross procedure has provided good long-term results in this challenging group of paediatric patients.

Favourable mid-term outcome after heart transplantation for late Fontan failure.

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

Resection of the stenotic segment with individually tailored anastomosis for symptomatic congenital tracheal stenosis in infants.

OBJECTIVES: To analyse retrospectively population-based results of congenital tracheal stenosis (CTS) repair in infants in Finland. METHODS: Data on infants who were operated on for CTS in Helsinki Children's Hospital between August 1988 and May 2013 were analysed retrospectively. Fibreoptic bronchoscopy was performed perioperatively and in follow-up of all the surviving patients. The median follow-up time was 7 (range 1-20) years. RESULTS: Thirteen infants were operated on for CTS. Resection of the stenotic segment with individually tailored anastomosis was used in 12 patients and slide tracheoplasty in 1 patient. The median age at the operation was 2.9 (range 0.2-19) months. Eight (62%) patients had associated cardiovascular defects, which were corrected during the same operation. The median length of stenosis was 35% (range 25-60%) of the total length of the trachea. The median length of time of postoperative mechanical ventilation was 10 (range 5-19) days. The median length of time of intensive care treatment was 15 (range 7-40) days. One patient died from hypoplastic lung tissue and fibrosis, and multiorgan failure. One patient required reoperation, and 3 other patients received balloon bronchodilatations postoperatively. There was no late mortality. All of the 12 survivors had a good outcome. CONCLUSION: Resection with individually tailored anastomosis with up to 55% of the stenotic segment of the trachea presented a good long-term outcome.

Mesoportal bypass using a constructed saphenous vein graft for extrahepatic portal vein obstruction--technique, feasibility, and outcomes.

BACKGROUND: The internal jugular vein is routinely used as a graft for the Rex shunt. We analyzed results of mesoportal bypass using an alternative autologous graft. METHODS: Twenty-one children with extrahepatic portal vein obstruction were treated with a Rex shunt constructed using both greater saphenous veins. Follow-up included ultrasound and blood count screening at 3, 6, and 12 months and annually thereafter. RESULTS: Median age was 8.7 years (range, 3.6-14 years), and follow-up time, 5.3 years (range, 0.6-7.1 years). Occlusion or narrowing occurred in 6 patients after a median of 20 months (range, 2.6-52 months). In 2 cases, patency was restored, giving an overall success rate of 81%. During follow-up, no variceal bleeding occurred while hemoglobin, platelet count, and leukocyte levels increased (P ≤ .02 for all) and spleen size decreased (P = .001). Patients with occlusive shunt complications weighed less (P = .01), had higher preoperative platelet levels (P = .02), and tended to have a smaller spleen preoperatively (P = .06) than patients without shunt complications. Cumulative graft patency at 6 months, 1 year, 3 years, and 5 years was 100%, 89%, 82%, and 74%, respectively. CONCLUSIONS: Rex shunt constructed using the greater saphenous veins is a valuable alternative to the internal jugular vein graft, allowing long-term reversal of portal hypertension, splenomegaly, and hypersplenism. Low patient weight and high platelet count predicted shunt occlusion.